From info at scd-symposium.org Sun Oct 1 00:06:09 2006 From: info at scd-symposium.org (SCD Symposium) Date: Sun, 1 Oct 2006 00:06:09 -0300 Subject: [SCD-FORUM] Opening speech Message-ID: http://www.scd-symposium.org/openingspeech.php To all our colleagues from more than 100 countries who are participating in this World Wide Internet Symposium on Sudden Cardiac Death, we WELCOME YOU! I am pleased and honored to chair this fascinating and challenging topic using the most modern electronic approach to reach a world wide audience. Once again we begin this academic activity within the framework of the Activity of Continuing and Distance Medical Education of ISHNE. We hope that the high level of the presentations given by outstanding world authorities in the field of sudden cardiac death (SCD), as well as by the importance and timeliness of the topics addressed, will meet your expectations. Written lectures, interviews, webcasts, presentation of clinical cases, and the possibility of interacting through discussion Forums, will constitute the essence of this Symposium as we present an update on the most important topics in the area of SCD. As you are aware, SCD remains a major challenge in today?s modern society. Thus, topics related to Epidemiology, Etiology, Clinics, Mechanisms, Risk Factors, Pathology, Genetics, Prevention and Management including pharmacological, devices, ablation, and surgery constitute the topics to be covered in this complex area. Because establishing permanent scientific links within the international community as well as being able to communicate in a more comprehensive fashion, we will offer the opportunity of choosing most activities in five languages ?English, Spanish, Portuguese, Russian, and Chinese. Recognizing the high quality of this Symposium, we offer the option for participants to obtain Continuing Medical Education credits (CME credits). Obtaining such credits is the best way for viewers to achieve a personal evaluation, as well as to evaluate the Symposium in general. Forty-one members of our faculty, along with 20 experts from all over the world, will provide their contributions to the different thematic areas. We express our thanks to all of them. We also want to thank Medtronic as the official sponsor of the meeting and especially the Medtronic managers, Ross Meisner and Hugo Fagioli, because they readily understood the importance of this project and supported it from the beginning. Once again, by organizing this meeting through the Internet, we are expressing our confidence in the immense power of this medium as a tool to transmit knowledge. With these brief introductory comments I now declare that the activities of this World Symposium are open. Now, I invite you go forth to learn! Douglas P. Zipes, Silvia Priori, Sergio Dubner, Edgardo Schapachnik, Wojciech Zareba, Andr?s R. P?rez Riera, Branco Mautner, Li Zhang -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061001/ff277bff/attachment.html From info at scd-symposium.org Sun Oct 1 00:06:28 2006 From: info at scd-symposium.org (SCD Symposium) Date: Sun, 1 Oct 2006 00:06:28 -0300 Subject: [SCD-FORUM] Today's Scientific Activity Message-ID: Dear colleagues, LECTURES As of today, in the Conference Room, the following Presentations are available: ACC/AHA/ESC 2006 guidelines for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death: a report of the American College of Cardiology/American Heart Association Task Force and the European Society of Cardiology Committee for Practice Guidelines (Writing Committee to Develop Guidelines for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death). http://www.scd-symposium.org/lectures.php OPENING SPEECH http://www.scd-symposium.org/openingspeech.php -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061001/8453532d/attachment.html From info at scd-symposium.org Sun Oct 1 00:07:42 2006 From: info at scd-symposium.org (SCD Symposium) Date: Sun, 1 Oct 2006 00:07:42 -0300 Subject: [SCD-FORUM] Passwords Message-ID: <403971B3-A57E-4116-8185-B190280BD0C8@scd-symposium.org> Dear Colleagues: If you don't remember your personal passwords, you can use the following ones: USER ID: scdeath PASSWORD: SCDEATH Best regards Edgardo and Sergio -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee From info at scd-symposium.org Mon Oct 2 00:00:51 2006 From: info at scd-symposium.org (SCD Symposium) Date: Mon, 2 Oct 2006 00:00:51 -0300 Subject: [SCD-FORUM] Today's Scientific Activity Message-ID: Dear colleagues, LECTURES As of today, in the Conference Room, the following Presentations are available: Syncope and Sudden Cardiac Death Stephen Hammill The Role of Public Access Defibrillation in the Chain of Survival from Out-of-Hospital Cardiac Arrest Joseph P. Ornato http://www.scd-symposium.org/lectures.php CLINICAL CASES In the sector corresponding to the Clinical cases, the following one are available: Syncope in a 29 year old male Dr. Martin Borggrefe http://www.scd-symposium.org/clinicalcases.php -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061002/1870ec49/attachment.html From info at scd-symposium.org Tue Oct 3 00:05:13 2006 From: info at scd-symposium.org (SCD Symposium) Date: Tue, 3 Oct 2006 00:05:13 -0300 Subject: [SCD-FORUM] Today's Scientific Activity Message-ID: <3CAE95E8-55D5-4349-B0D8-24DDFBB2D236@scd-symposium.org> Dear colleagues, LECTURES As of today, in the Conference Room, the following Presentations are available: Genetic and Acquired Causes of Sudden Death 1 Jeffrey Towbin The Role of Spatial Dispersion of Repolarization in Sudden Cardiac Death Charles Antzelevitch / Alejandra Guerchicoff / Guido D. Pollevick http://www.scd-symposium.org/lectures.php LPM RADIO - In the sector corresponding to the LPM Radio, you can see the following webcast Cardiac arrest outside hospital Henrick Wellens http://www.scd-symposium.org/lpmradio.php -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061003/0a4f7166/attachment.html From info at scd-symposium.org Wed Oct 4 07:09:47 2006 From: info at scd-symposium.org (SCD Symposium) Date: Wed, 4 Oct 2006 07:09:47 -0300 Subject: [SCD-FORUM] Today's Scientific Activity Message-ID: <40FBE1F0-D8DC-4CBE-B0F1-8F78913ED8AD@scd-symposium.org> Dear colleagues, LECTURES As of today, in the Conference Room, the following Presentations are available: Significance of Inducible Ventricular Flutter - Fibrillation After Myocardial Infarction Beatrice Brembilla-Perrot Reversible Causes of VT/VF: Fact or Fiction? Case presentation and review of the literature George Wyse / G. D. Veenhuyzen Prognosis of effectiveness of antiarrhythmic treatment in patients with malignant tachycardias (only in Russian and Spanish) Lada Nesterenko / Sergei Bakalov / Sergei Golicin http://www.scd-symposium.org/lectures.php LPM RADIO - In the sector corresponding to the LPM Radio, you can listening to the following broadcast Talking on SCD with Arthur Moss Arthur Moss http://www.scd-symposium.org/lpmradio.php CLINICAL CASES In the sector corresponding to the Clinical cases, the following one are available: Risk Stratification and ICD Prophylaxis: A case presentation and commentary on risk stratification for ICD therapy Thomas Bigger http://www.scd-symposium.org/clinicalcases.php -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061004/a41fdb33/attachment.html From info at scd-symposium.org Thu Oct 5 00:10:13 2006 From: info at scd-symposium.org (SCD Symposium) Date: Thu, 5 Oct 2006 00:10:13 -0300 Subject: [SCD-FORUM] Today's Scientific Activity Message-ID: <4D45C80C-C80E-4BFD-930B-5BCD6EB9E8B4@scd-symposium.org> Dear colleagues, OPENING LECTURE BY DR. DOUGLAS P. ZIPES: "Why did he die on Tuesday and not on Monday?" MONDAY, OCTOBER 11th LECTURES As of today, in the Conference Room, the following Presentations are available: Sudden Cardiac Death in Patients with Left Ventricular Dysfunction: Focus on Primary Prevention with ICDs Andrew Epstein Lessons from the DEFINITE Trial Kenneth Ellenbogen / Alan Kadish Community-Wide Analysis of Sudden Cardiac Death: Clinical and Research Implications Sumeet Chugh http://www.scd-symposium.org/lectures.php LPM RADIO - In the sector corresponding to the LPM Radio, you can see the following webcast ICD: Science and Economics in Nonischemic Heart Failure Alan Kadish http://www.scd-symposium.org/lpmradio.php -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061005/c3bc7aa8/attachment.html From info at scd-symposium.org Fri Oct 6 00:03:57 2006 From: info at scd-symposium.org (SCD Symposium) Date: Fri, 6 Oct 2006 00:03:57 -0300 Subject: [SCD-FORUM] Today's Scientific Activity Message-ID: Dear colleagues, OPENING LECTURE BY DR. DOUGLAS P. ZIPES: "Why did he die on Tuesday and not on Monday?" MONDAY, OCTOBER 11th LECTURES As of today, in the Conference Room, the following Presentations are available: Polymorphic ventricular tachycardia in patients with vasospastic angina Masayasu Hiraoka / Mitsuhiro Nishizaki Genetic and Acquired Causes of Sudden Death 2 Jeffrey Towbin PR interval shortening in patients with ventricular tachycardia Leonid Makarov / Tatiana Kyrileva / Vera Komoliatova / Svetlana Chuprova Arrhythmic death and ICD implantation after myocardial infarction Federico Lombardi http://www.scd-symposium.org/lectures.php -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061006/02fe6398/attachment.html From info at scd-symposium.org Fri Oct 6 22:49:52 2006 From: info at scd-symposium.org (SCD Symposium) Date: Fri, 6 Oct 2006 22:49:52 -0300 Subject: [SCD-FORUM] News Message-ID: SOON: LECTURE BY 1985 NOBEL PEACE PRIZE WINNER, DR. EVGENIY CHAZOV -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061006/b5f51973/attachment.html From info at scd-symposium.org Sat Oct 7 00:04:58 2006 From: info at scd-symposium.org (SCD Symposium) Date: Sat, 7 Oct 2006 00:04:58 -0300 Subject: [SCD-FORUM] Today's Scientific Activity Message-ID: <783558BA-1F05-4273-9C4B-34720A620AF5@scd-symposium.org> Dear colleagues, OPENING LECTURE BY DR. DOUGLAS P. ZIPES: "Why did he die on Tuesday and not on Monday?" WEDNESDAY, OCTOBER 11th LECTURES As of today, in the Conference Room, the following Presentations are available: Role of the Signal Ecg in Risk Stratification of Scd. - An overview Nabil El-Sherif Drug-Proarrhythmic Sudden Cardiac Death Michael Lehmann J Wave Syndromes Dongqi Wang / Chongzong Cui / Gan-Xin Yan http://www.scd-symposium.org/lectures.php -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061007/26fbba36/attachment.html From info at scd-symposium.org Sun Oct 8 00:06:42 2006 From: info at scd-symposium.org (SCD Symposium) Date: Sun, 8 Oct 2006 00:06:42 -0300 Subject: [SCD-FORUM] Today's Scientific Activity Message-ID: <4061A828-D4DE-4C9D-AB24-3ED73608C5E2@scd-symposium.org> Dear colleagues, OPENING LECTURE BY DR. DOUGLAS P. ZIPES: "Why did he die on Tuesday and not on Monday?" WEDNESDAY, OCTOBER 11th COMING SOON: LECTURE BY 1985 NOBEL PEACE PRIZE WINNER, DR. EVGENIY CHAZOV LECTURES As of today, in the Conference Room, the following Presentations are available: Usefulness of Epinephrine Test in the Congenital Long QT Syndrome Wataru Shimizu Left cardiac sympathectomy to manage beta-blocker resistant LQT patients Lexin Wang Sudden Death and Management of the Long QT Syndrome - My views Peter J. Schwartz http://www.scd-symposium.org/lectures.php LPM RADIO - In the sector corresponding to the LPM Radio, you can listening to the following broadcast Quinidine Therapy in Brugada Syndrome: an Alternative to ICD in Asymptomatic Patients with Inducible VF Bernard Belhassen http://www.scd-symposium.org/lpmradio.php CLINICAL CASES In the sector corresponding to the Clinical cases, the following one are available: Isolated Noncompaction of Right Ventricular Myocardium and Arrhythmia Yongxin Lu / Qi Run / Zhijian Chen / Zihua Zhou / Yuhua Liao / Mingxin Xie / Heshui Shi http://www.scd-symposium.org/clinicalcases.php -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061008/fa9523c8/attachment.html From info at scd-symposium.org Sun Oct 8 15:31:09 2006 From: info at scd-symposium.org (SCD Symposium) Date: Sun, 8 Oct 2006 15:31:09 -0300 Subject: [SCD-FORUM] Bibliographical update Message-ID: <77AB8EC5-1F2B-491F-9669-1CB1AC8DADDF@scd-symposium.org> Dear colleagues, We include instructions to access the bibliography of this last week. We hope this will be of your interest. To access the bibliography http://www.scd-symposium.org/files/2006_10_07_SCD.html Kind regards, Dr. Edgardo Schapachnik - Dr. Sergio Dubner -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061008/d42dc6e0/attachment.html From info at scd-symposium.org Mon Oct 9 00:02:18 2006 From: info at scd-symposium.org (SCD Symposium) Date: Mon, 9 Oct 2006 00:02:18 -0300 Subject: [SCD-FORUM] Today's Scientific Activity Message-ID: <68A7EDA6-5B3D-42D2-BE59-5B8357C68EF3@scd-symposium.org> Dear colleagues, OPENING LECTURE BY DR. DOUGLAS P. ZIPES: "Why did he die on Tuesday and not on Monday?" WEDNESDAY, OCTOBER 11th COMING SOON: LECTURE BY 1985 NOBEL PEACE PRIZE WINNER, DR. EVGENIY CHAZOV LECTURES As of today, in the Conference Room, the following Presentations are available: Are there low risk patients in Brugada syndrome? Pedro Brugada / Andrea Sarkozy Ankyrin-B Syndrome and Ankyrin-G Brugada Syndrome: Two Arrhythmogenic Fatal Cardiac Arrhythmic Entities Andr?s Ricardo P?rez Riera / Edgardo Schapachnik / Sergio Dubner http://www.scd-symposium.org/lectures.php LPM RADIO - In the sector corresponding to the LPM Radio, you can see the following webcast Genetic of Arrhythmogenic Diseases Silvia Priori http://www.scd-symposium.org/lpmradio.php -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061009/a9368d72/attachment.html From info at scd-symposium.org Tue Oct 10 00:04:05 2006 From: info at scd-symposium.org (SCD Symposium) Date: Tue, 10 Oct 2006 00:04:05 -0300 Subject: [SCD-FORUM] Today's Scientific Activity Message-ID: Dear colleagues, OPENING LECTURE BY DR. DOUGLAS P. ZIPES: "Why did he die on Tuesday and not on Monday?" WEDNESDAY, OCTOBER 11th COMING SOON: LECTURE BY 1985 NOBEL PEACE PRIZE WINNER, DR. EVGENIY CHAZOV LECTURES As of today, in the Conference Room, the following Presentations are available: Arrhythmogenic Right Ventricular Cardiomyopathies and Sudden Death Guy Fontaine / Philippe Charron / Robert Frank http://www.scd-symposium.org/lectures.php LPM RADIO - In the sector corresponding to the LPM Radio, you can listening to the following broadcast Right Ventricular Dysplasia/Cardiomyopathy Frank Marcus http://www.scd-symposium.org/lpmradio.php CLINICAL CASES In the sector corresponding to the Clinical cases, the following one are available: The patient is a 57-year-old previously healthy and very active Caucasian male David Cannom Clinical presentation and management of a patient with catecholamine sensitive polymorphic ventricular tachycardia (CPVT) Cuilan Li / Dayi Hu / Wenling Liu / Chen Tan / Yuntian Li / Lei Li http://www.scd-symposium.org/clinicalcases.php -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061010/24f4ec56/attachment.html From info at scd-symposium.org Wed Oct 11 00:05:38 2006 From: info at scd-symposium.org (SCD Symposium) Date: Wed, 11 Oct 2006 00:05:38 -0300 Subject: [SCD-FORUM] Today's Scientific Activity Message-ID: <189EE208-C8E0-4B95-B0BE-9C6F9CFD1B48@scd-symposium.org> Dear colleagues, OPENING LECTURE BY DR. DOUGLAS P. ZIPES: "Why did he die on Tuesday and not on Monday?" LATER LECTURES As of today, in the Conference Room, the following Presentations are available: Sudden Death Evgeniy Chazov - 1985 NOBEL PEACE PRIZE WINNER Surface ECG and risk of sudden death Bay?s de Luna, A, Bay?s-Gen?s, J. Guindo, X. Vi?olas, J. Cinca Qt dynamicity: clinical and prognostic implications Bay?s de Luna, A http://www.scd-symposium.org/lectures.php -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061011/8e9e8009/attachment.html From info at scd-symposium.org Wed Oct 11 11:16:31 2006 From: info at scd-symposium.org (SCD Symposium) Date: Wed, 11 Oct 2006 11:16:31 -0300 Subject: [SCD-FORUM] Dr. Zipes' Opening Lecture Message-ID: <12C2B773-30A0-40F2-B042-6EEBBD646305@scd-symposium.org> Dear colleagues, We present the Opening Lecture of the Symposium, with the publication of the audiovisual material by Dr. Douglas P. Zipes with the title "Why did he die on Tuesday and not on Monday?" LECTURES As of today, in the Conference Room, Dr. Zipes's Presentation is available: "Why did he die on Tuesday and not on Monday?" Douglas P. Zipes http://www.scd-symposium.org/lectures.php LPM RADIO - In the sector corresponding to the LPM Radio, Dr. Zipes's Presentation is available too: "Why did he die on Tuesday and not on Monday?" Douglas P. Zipes http://www.scd-symposium.org/lpmradio.php Thus, we will start the period of deliberations through the Discussion Forum of this event. We invite all our colleagues to ask all the questions you wish to the lecturers, as well as asking (and answering) all those doubts related with this fascinating topic, and express your opinion freely on the matters that interest you. -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061011/c38868cf/attachment.html From info at scd-symposium.org Wed Oct 11 11:17:42 2006 From: info at scd-symposium.org (SCD Symposium) Date: Wed, 11 Oct 2006 11:17:42 -0300 Subject: [SCD-FORUM] To open the deliberations Message-ID: Dear colleagues and friends, With this message we open the period of deliberations of the Symposium. This Forum will be the channel to which you may send all kinds of questions, opinions, replies, counter-replies, etc. We hope that this new stage of the event will be enriching for all of us. Cordially, Sergio and Edgardo -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061011/7904b6b7/attachment.html From info at scd-symposium.org Wed Oct 11 17:00:41 2006 From: info at scd-symposium.org (SCD Symposium) Date: Wed, 11 Oct 2006 17:00:41 -0300 Subject: [SCD-FORUM] 1E. Dr. Cannom's case. Dr. Li Zhang Message-ID: Dear Dr. Cannom: Thank you so much for providing the ECG*** of your case patient upon my request. *** http://www.scd-symposium.org/files/Cannom.pdf From this ECG I noticed a prominent Osborn (or J) wave in most of 12 leads except V1-2. The ST segment is slightly elevated. The QT interval is normal. Such pattern may be seen in early repolarization syndrome. In this particular case, I wonder whether this Osborn wave is an indication of delayed conduction in some part of left ventricle due to chronic coronary insufficiency, and therefore a substrate to reentrant ventricular tachyarrhythmias. If this assumption were true, he is under a high risk of VT/VF. You are the leading expert in this area, and you know much more about this patient. I look forward to learning from you and from other experts in this SCD-symposium. Sincerely, Li Zhang, MD LDS Hospital, Intermountain Healthcare University of Utah School of Medicine Salt Lake City, UT USA -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061011/17bfcee4/attachment.html From info at scd-symposium.org Wed Oct 11 18:04:33 2006 From: info at scd-symposium.org (SCD Symposium) Date: Wed, 11 Oct 2006 18:04:33 -0300 Subject: [SCD-FORUM] EXPERTS ASK, EXPERTS ANSWER Message-ID: <0A1DE18F-6F3D-41F2-BAE3-C1F3C5DFA181@scd-symposium.org> Dr. Serge Boveda from France asks: - One ICD for each post-MI patient: dream or nightmare? Dr. Beatrice Brembrilla Perrot from France answers - The answer is nightmare To wait heart transplantation without risk in a patient, I agree To delay the death related to heart failure I disagree One other indication : the contraindication to beta blockers !! We still need to collect non invasive and invasive studies of our patients to decrease the indications of ICD, to need more precisely the coronary status of these patients (unknown in MADIT) and I hope for the development a real curative treatment (ex cells injection?) Sincerely yours B?atrice -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061011/6390c63e/attachment.html From info at scd-symposium.org Wed Oct 11 22:30:45 2006 From: info at scd-symposium.org (SCD Symposium) Date: Wed, 11 Oct 2006 22:30:45 -0300 Subject: [SCD-FORUM] 2S. CLBBB. Dr. Ruiz Alais Message-ID: Dear friends, Congratulations on such a novel and scientific symposium on heart pathologies, for I am personally quite surprised with the advanced progresses that help to clarify and understand lots of pathologies still unknown to many colleagues. By the way, I would like to know about the newest advancements about complete left bundle branch block of his bundle; if this may actually be a pathology that may cause sudden death; clarify its etiology, prognosis, proper management, what other complications it may have, and if possible, whether there is some innovative treatment to endure this condition. I would also like to know what is the experience regarding the cardiac ischemia treatment with vastarel (trimetazidine dichlorhydrate). Thank you very much for your consideration to this message. I remain waiting for your kind response, JORGE RUIZ ALAIS . From info at scd-symposium.org Thu Oct 12 11:47:15 2006 From: info at scd-symposium.org (SCD Symposium) Date: Thu, 12 Oct 2006 11:47:15 -0300 Subject: [SCD-FORUM] EXPERTS ASK, EXPERTS ANSWER Message-ID: Dr. Leonid Makarov, from Russia asks - Is in necessary to perform diagnostic studies in family members of a victim of sudden death by noncoronary origin, if this victim is young (up to 45 years old) and had no background of cardiological disease? If there was such need, what 3 first diagnostic studies should be made? Both in nonspecialized external offices, and in a specialized cardiological high-complexity center? Dr. Silvia Priori, from Italy answers - In any case of unexplained sudden cardiac death especially at young age an evaluation of family members is reasonable. First important thing in my view is to perform a careful collection of family history with construction of a family tree. Questioning family members for occurrences of sudden death (especially death associated with stress, exercise and emotion or death during sleep); questioning of familial occurrence of for syncope and arrhythmias. Useful to keep in mind that epilepsy is often a diagnosis in pts with repeated syncope and seizure like manifestation: therefore it may be useful to distinguish between typical epilepsy responsive to therapy versus atypical epilepsy non responsive to therapy. The latter may in fact be a misdiagnosis for an arrhythmogenic syndrome). In term of investigation of family members first step is an ECG/ holter searching for diagnosis such as Brugada, long QT, and ECG signs of cardiomyopathies. An echo is indicated for identification of right ventricular cardiomyopathy, hypertrophic cardiomyopathy and dilated cardiomyopathy. If SCD occurred during physical exercise and/or if any family member experienced syncope during physical activity an exercise stress test may also be performed. To summarize: careful collection of family history plus a full non invasive evaluation of close relatives of the SCD victim (brother and sisters and offspring) is the approach used at our clinic for inherited arrhythmogenic diseases. Based on results of the clinical evaluation above the need/value of genetic analysis will be defined. Silvia G Priori -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061012/11d35016/attachment.html From info at scd-symposium.org Thu Oct 12 13:43:21 2006 From: info at scd-symposium.org (SCD Symposium) Date: Thu, 12 Oct 2006 13:43:21 -0300 Subject: [SCD-FORUM] Passwords Message-ID: <19526348-4B92-4B85-B98A-EF776AC76057@scd-symposium.org> Dear Colleagues: If you don't remember your personal passwords, you can use the following ones: USER ID: scdeath PASSWORD: SCDEATH Best regards Edgardo and Sergio -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee From info at scd-symposium.org Thu Oct 12 13:51:47 2006 From: info at scd-symposium.org (SCD Symposium) Date: Thu, 12 Oct 2006 13:51:47 -0300 Subject: [SCD-FORUM] 3E. A children with LQTS. Dr. Haghjoo Message-ID: <0DEDFEC2-AB49-418F-A3F9-EDF76853B637@scd-symposium.org> Dear Dr. Dubner, Thank you and your colleagues for this nice online symposium. Recently, a 5-year-old children with no evidence of structural heart disease referred to our center for evaluation of syncope. He also had history of congenital deafness. One of the episodes of syncope occurred 48-hrs after betablocker withdrawal and second episodes on 10 mg propranolol tid. Surface ECG revealed a QTc=600 ms and heart rate of 55 bpm. We think that onset in childhood, congenital deafness and QTc=600 would place this patient at higher risk for cardiac events and therefore a candidate for ICD implantation. However, small body size and future risk of inappropriate therapy with its devastating consequences are unfavorable aspects of ICD implantation. Our limitimg factors for increase in dose of betablocker is low baseline hear rate. What is recommendation of scientific committee of SCD symposium regarding our patient? Thank you in advance for your recommendation. Regards, Majid Haghjoo,MD Department of Pacemaker and Electrophysiology Rajaie Cardiovascular Medical and Research Center Tel: +98 21 2392 2931 Fax:+98 21 2204 8174 Tehran, Iran From info at scd-symposium.org Thu Oct 12 17:22:54 2006 From: info at scd-symposium.org (SCD Symposium) Date: Thu, 12 Oct 2006 17:22:54 -0300 Subject: [SCD-FORUM] 4E. Question for Dr. Cannom. Miss Tink Message-ID: Dr. Cannom, Thank you for the case study... You noted: "PVC's...suppressed with exercise"... "but at noon he had a seizure and CPR was initiated by a colleague" Over the course of nearly 8 years, numerous ARVD diagnosed people, group members, have mentioned that their PVCs "go away" when they begin to exercise a little. Others have mentioned that exercise can take them quickly into VT (i.e. hopping on an exercise bicycle, doing a few moves of Tae Kwon Do.) Numerous members have mentioned that when they have had VT and subsequently fainted, that those who were with them said they appeared to be having a "seizure" The questions are: 1. Is it well known that some patients with frequent PVCs often notice that they go away during exercise of some sort, i.e. raise in heart rate? As a note, a number of ARVD diagnosed group members have stabilized (i.e. frequent PVCs, short and/or long runs of VT are alleviated) when their Brady pacing is set to 65 - 85 (dependent on patient.) 2. If it is known that frequent PVCs often disappear in some people during light exercise, is this the reason that some EPs set their patient's brady pacing as mentioned above? 3. Is it well known that patients who go into VT and subsequently pass out often appear to be having a seizure? 4. Had the patient in this case been prone to seizures? Do they have epilepsy? Is it possible that a patient suffering that which is described as seizures and syncope, with no evidence of epilepsy, might be suspected of something known to cause VT? Thanks in advance for your help with these answers. Micheline Tink Long International ARVD Family Network Group -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061012/9c89a6b2/attachment.html From info at scd-symposium.org Thu Oct 12 18:31:34 2006 From: info at scd-symposium.org (SCD Symposium) Date: Thu, 12 Oct 2006 18:31:34 -0300 Subject: [SCD-FORUM] 5S. Brugada syndrome. Dr. Arenzo Message-ID: <2C699C4E-8236-4DE2-81AD-5A70DF8BDB4F@scd-symposium.org> First, I would like to congratulate the organizers of this beautiful Symposium; thanks to them we can exchange ideas with other colleagues. My question is the following: The finding of a typical Brugada pattern in ECG, in asymptomatic patient, without heart disease proven by noninvasive studies (echocardiogram, ergometer and Holter), and no family background of sudden death. In these cases, does the test with ajmaline have a diagnostic/ prognostic value? Is performing electrophysiological evaluation justified? Does the genetic study provide prognostic data? Thank you, and best regards for all the participants. DR. JULIO ARENZO Buenos Aires. ARGENTINA -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061012/28937e66/attachment.html From info at scd-symposium.org Thu Oct 12 20:12:16 2006 From: info at scd-symposium.org (SCD Symposium) Date: Thu, 12 Oct 2006 20:12:16 -0300 Subject: [SCD-FORUM] 6E RE: Brugada syndrome. Dr. Ramon Brugada Message-ID: Dear Dr Arenzo: In the individual with type I ecg, there is no need for an ajamline test, the diagnosis is made. If the ecg is "type II or III" (non-diagnostic), ajmaline test is recommended. You will find all the options available in how to deal with the asymptomatic patient with type I ecg. - follow-up until he develops symptoms. - ICD implantation, especially if there is a remote possibility of sudden cardiac death in the family - EPS as tool for risk stratification. A positive non- aggressive EPS is very indicative of a patient with an increased risk for arrhythmogenecity. A negative EPS in the sporadic individual is indicative of good prognosis. As you well know, the use of EPS to risk stratify asymptomatic patients is very controversial. However, the first two options are not perfect. The first symptom may be sudden death, and ICD is not free of collateral problems and certainly not an easy decision for an asymptomatic 40 year old . Thank you Ramon Brugada MD FACC Associate Professor of Medicine Canadian Research Chair Genetics of Arrhythmias University of Montreal Director Clinical Cardiovascular Genetics Center Montreal Heart Institute 5000 Rue Belanger Montreal, QC H1T 1C8 Canada ramon at brugada.org -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061012/9460f969/attachment.html From info at scd-symposium.org Thu Oct 12 23:12:05 2006 From: info at scd-symposium.org (SCD Symposium) Date: Thu, 12 Oct 2006 23:12:05 -0300 Subject: [SCD-FORUM] 8E A female with burned-out phase of HCM? Dr. Alizadeh Message-ID: Dear Dr Dubner thank you for this excellent educational site A 38 year old femal referred to our center with new onset dyspnea from a few month ago. She had family history of sudden cardia death. She had CHB with wide ventricular escape rhythm. TT.echocardiography revealed sever LV and RV systolic dysfunction, asymetric septal hyperthrophy, pulmonary hypertention (PAP=65-70mmhg) and without LVOT gradient.patient,s dyspnea improved with low dose diuretics , ACE inhibitor and TPM implantation. Do you recommend DDD pacemaker for this patient? or Do you recommend ICD-DR or ICD-CRT? what is your opinion about clinical course of her disease? does she need further workup before device implantation? sincerely yours Hormoz Alizadeh MD Department of Pacemaker and Electrophysiology Rajaie Cardiovascular Medical and Research Center Tehran, Iran -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061012/1f27fdf5/attachment.html From info at scd-symposium.org Fri Oct 13 11:55:23 2006 From: info at scd-symposium.org (SCD Symposium) Date: Fri, 13 Oct 2006 11:55:23 -0300 Subject: [SCD-FORUM] 7R Palpitations and SCD risk. Dr. Andriy Vorotniak Message-ID: <81AB3D7E-A763-4A44-965F-B4F199B1ED2B@scd-symposium.org> Dear colleagues, I would like to ask some questions about the prognostic value of "palpitations" in Brugada and Long QT syndromes: 1. What is the risk of SCD in a young patient with no underlying heart disease or hereditary-familial background for SCD, with Brugada syndrome (or Long QT) ECG pattern, and aborted and isolated episodes of "palpitations" (not recorded with Holter monitoring)? 2. How do we continue the evaluation of this patient, if in the electrophysiological study there is no ventricular arrhythmia triggered? 3. Is there any relationship between the mentioned syndromes and supraventricular tachyarrhythmia incidence? Thank you very much, Dr. Andriy Vorotniak Buenos Aires, Argentina -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061013/d0c43c34/attachment.html From info at scd-symposium.org Fri Oct 13 12:24:50 2006 From: info at scd-symposium.org (SCD Symposium) Date: Fri, 13 Oct 2006 12:24:50 -0300 Subject: [SCD-FORUM] 10E Correction of gender in case report presented by Dr. Hagjoo. Dr. Alizadeh Message-ID: <9C5E5D83-2415-4F10-ACE3-670AE6841AED@scd-symposium.org> Dear Dr Dubner I would like to congratulate you and your colleagues. The patient presented by my colleague was a girl. prognosis of long QT is different in male and femal patient. Best regards Hormoz Alizadeh MD Department of Pacemaker and Electrophysiology Rajaie Cardiovascular Medical and Research Center Tehran, Iran -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061013/2feaa886/attachment.html From info at scd-symposium.org Fri Oct 13 12:40:36 2006 From: info at scd-symposium.org (SCD Symposium) Date: Fri, 13 Oct 2006 12:40:36 -0300 Subject: [SCD-FORUM] EXPERTS ASK, EXPERTS ANSWER Message-ID: Dr. Ariel Szyszko from Argentina asks - In syncope with clear anamnesis of vasovagal cause, is it essential to perform a TILT TEST? Dr. Stephen Hammill from U.S.A. answers - It is not essential to perform a tilt test on a patient with a history that is classic for vasovagal syncope assuming the remaining aspects of their history ore normal (such as no family history of sudden death) and their physical examination and ECG are normal. I may elect to obtain an echocardiogram if any concerns are raised by the history, examination or basic testing. Stephen C. Hammill, MD Professor of Medicine, Mayo Clinic College of Medicine Director, Electrocardiography Laboratory hammill.stephen at mayo.edu -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061013/89278e10/attachment.html From info at scd-symposium.org Fri Oct 13 16:16:31 2006 From: info at scd-symposium.org (SCD Symposium) Date: Fri, 13 Oct 2006 16:16:31 -0300 Subject: [SCD-FORUM] 11E RE: Palpitations and SCD risk. Dr. Haghjoo In-Reply-To: <81AB3D7E-A763-4A44-965F-B4F199B1ED2B@scd-symposium.org> References: <81AB3D7E-A763-4A44-965F-B4F199B1ED2B@scd-symposium.org> Message-ID: Dear Dr. Vorotniak, Thank you for presenting this case. There is no difference in risk of SCD in Brugada patient with palpitation or asymptomatic Brugada syndrome. It is well known that incidence of many supraventricular tachycardias such as AF, AFL, and AVNRT are higher in patients with Brugada syndrome. Thus, medical follow-up is recommended in case of noninducibility of any arrhythmia during electrophysiologic study. Best regards, Majid Haghjoo,MD Department of Pacemaker and Electrophysiology Rajaie Cardiovascular Medical and Research Center Tel: +98 21 2392 2931 Fax:+98 21 2204 8174 Tehran, Iran > > Dear colleagues, > I would like to ask some questions about the prognostic value of > "palpitations" in Brugada and Long QT syndromes: > > 1. What is the risk of SCD in a young patient with no underlying > heart disease or hereditary-familial background for SCD, with > Brugada syndrome (or Long QT) ECG pattern, and aborted and isolated > episodes of "palpitations" (not recorded with Holter monitoring)? > 2. How do we continue the evaluation of this patient, if in the > electrophysiological study there is no ventricular arrhythmia > triggered? > 3. Is there any relationship between the mentioned syndromes and > supraventricular tachyarrhythmia incidence? > > Thank you very much, > Dr. Andriy Vorotniak > Buenos Aires, Argentina > > -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061013/b86e117a/attachment.html From info at scd-symposium.org Fri Oct 13 16:24:41 2006 From: info at scd-symposium.org (SCD Symposium) Date: Fri, 13 Oct 2006 16:24:41 -0300 Subject: [SCD-FORUM] EXPERTS ASK, EXPERTS ANSWER. Dr. Makarov In-Reply-To: References: Message-ID: <2B0A1372-E24E-4835-AA96-C9C5AB32D7E5@scd-symposium.org> Dear dr. Priory, thank you very match for your answer. I am comlectly agree with your mention. But for practictical realisation of it approaches and identification of patients with diseases witn high risk of SCD in young (<45 yo) possiblly important to include family sudden death in 1-st Class of indication for ECG and Holter monitoring (first of all for GP physicians). And according to a next mention, guestion - "Family case of sudden death" is it diagnosis or not for members of victims family? Leonid Makarov -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee > ______________________________________________________________________ > > Dr. Leonid Makarov, from Russia asks > > - Is in necessary to perform diagnostic studies in family members > of a victim of sudden death by noncoronary origin, if this victim > is young (up to 45 years old) and had no background of > cardiological disease? If there was such need, what 3 first > diagnostic studies should be made? Both in nonspecialized external > offices, and in a specialized cardiological high-complexity center? > > Dr. Silvia Priori, from Italy answers > > - In any case of unexplained sudden cardiac death especially at > young age an evaluation of family members is reasonable. > First important thing in my view is to perform a careful collection > of family history with construction of a family tree. Questioning > family members for occurrences of sudden death (especially death > associated with stress, exercise and emotion or death during > sleep); questioning of familial occurrence of for syncope and > arrhythmias. Useful to keep in mind that epilepsy is often a > diagnosis in pts with repeated syncope and seizure like > manifestation: therefore it may be useful to distinguish between > typical epilepsy responsive to therapy versus atypical epilepsy non > responsive to therapy. The latter may in fact be a misdiagnosis for > an arrhythmogenic syndrome). > In term of investigation of family members first step is an ECG/ > holter searching for diagnosis such as Brugada, long QT, and ECG > signs of cardiomyopathies. An echo is indicated for identification > of right ventricular cardiomyopathy, hypertrophic cardiomyopathy > and dilated cardiomyopathy. > If SCD occurred during physical exercise and/or if any family > member experienced syncope during physical activity an exercise > stress test may also be performed. > To summarize: careful collection of family history plus a full non > invasive evaluation of close relatives of the SCD victim (brother > and sisters and offspring) is the approach used at our clinic for > inherited arrhythmogenic diseases. Based on results of the clinical > evaluation above the need/value of genetic analysis will be defined. > Silvia G Priori > _______________________________________________ > Scd-forum mailing list > Scd-forum at scd-symposium.org > http://www.grupoakros.com.ar/mailman/listinfo/scd-forum -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061013/b575dc53/attachment.html From info at scd-symposium.org Fri Oct 13 16:29:38 2006 From: info at scd-symposium.org (SCD Symposium) Date: Fri, 13 Oct 2006 16:29:38 -0300 Subject: [SCD-FORUM] 13E A patient with typical vasovagal syncope based on history and type II Brugada pattern. Dr. Alizadeh Message-ID: I would like to thank the organizers of this symposium My question is about a patient with typical vasovagal syncope based on history and positive HUTT, negative family history for SCD, type II Brugada pattern without drug chalenge test and type I Brugada pattern in ECG after flecainide test. Do you recommend further evaluation by electrophysiologic testing or genetic study? what is the first choice for a patient with typical vasovagal syncope based on history and type II Brugada pattern? flecainide test or HUTT. Best regards Hormoz Alizadeh MD Department of Pacemaker and Electrophysiology Rajaie Cardiovascular Medical and Research Center Tehran, Iran -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061013/7b8a0273/attachment.html From info at scd-symposium.org Fri Oct 13 17:01:16 2006 From: info at scd-symposium.org (SCD Symposium) Date: Fri, 13 Oct 2006 17:01:16 -0300 Subject: [SCD-FORUM] 12E RE: A children with LQTS. Dr. Makarov In-Reply-To: <0DEDFEC2-AB49-418F-A3F9-EDF76853B637@scd-symposium.org> References: <0DEDFEC2-AB49-418F-A3F9-EDF76853B637@scd-symposium.org> Message-ID: <89308DDF-BB85-4982-88D5-3FD93BE034E3@scd-symposium.org> By our experience of the managment and follow up of children with congenital LQTS, beta blockers (Propranolol, Atenolol, Nadolol) therapy in daily dose 1-2 mg/kg not significantly increase of bradycardia in this pts. Sometimes combination of the regular doses of betablockers (1 mg/kg) and Na blockers (we use carbamazepin 10 mg/kg/daily) may be useful. But in this case patient had syncope, very long QTc (600 ms) and heart rate 55 bpm for 5 years old children on rest ECG is very low. By my opinion children need in antyarhythmic device in combination with drug therapy. Dr. Leonid Makarov Moscow Institute pediatry and children surgery Moscow, Russia. Taldomskaya str. 2 leo at oss.ru -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee > > Dear Dr. Dubner, > > Thank you and your colleagues for this nice online symposium. > Recently, a > 5-year-old children with no evidence of structural heart disease > referred to > our center for evaluation of syncope. He also had history of > congenital > deafness. One of the episodes of syncope occurred 48-hrs after > betablocker > withdrawal and second episodes on 10 mg propranolol tid. Surface ECG > revealed a QTc=600 ms and heart rate of 55 bpm. We think that onset in > childhood, congenital deafness and QTc=600 would place this patient at > higher risk for cardiac events and therefore a candidate for ICD > implantation. However, small body size and future risk of > inappropriate > therapy with its devastating consequences are unfavorable aspects > of ICD > implantation. Our limitimg factors for increase in dose of > betablocker is > low baseline hear rate. What is recommendation of scientific > committee of > SCD symposium regarding our patient? > > Thank you in advance for your recommendation. > > Regards, > > Majid Haghjoo,MD > Department of Pacemaker and Electrophysiology > Rajaie Cardiovascular Medical and Research Center > Tel: +98 21 2392 2931 > Fax:+98 21 2204 8174 > Tehran, Iran -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061013/76636065/attachment.html From info at scd-symposium.org Fri Oct 13 17:17:47 2006 From: info at scd-symposium.org (SCD Symposium) Date: Fri, 13 Oct 2006 17:17:47 -0300 Subject: [SCD-FORUM] 9S RE: Palpitations and SD risk Dr. Munoz Message-ID: <64EA7334-BD8F-4A81-A330-7A441ECD4C21@scd-symposium.org> First, my dear colleague, I would like to emphasize that one of the preferential patterns of behavior about pre-excitation syndromes, just as the case of WPW (case of supraventricular tachycardias), is constituted by short PR with delta wave. If there is no anomalous behavior pattern by anomalous reentry conduction (Kent's bundle), we should discuss if there is pharmacological or sympathomimetic background, as well as thyroid evaluation. Possibly, it may also be associated to "ansiogenic" state, as well as possibly researching in more depth, behavioral aspects should be observed, related to possible narcotic drug dependency, considering the age characteristics. Long QT (BrS) is generally characteristic of the female gender with a much greater prevalence over the male gender; on the other hand it would be convenient to perform a stress test (stress ergometer test) to check whether there are symptom alterations, which may leave the patient vulnerable to SDS (sudden death syndrome). An appropriate screening would be very helpful, not forgetting a thorough semiological test. Sincerely, Prof. Marcelo Mu?oz -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061013/427642a9/attachment.html From info at scd-symposium.org Fri Oct 13 20:09:48 2006 From: info at scd-symposium.org (SCD Symposium) Date: Fri, 13 Oct 2006 20:09:48 -0300 Subject: [SCD-FORUM] 16E RE: A children with LQTS. Dr. Towbin In-Reply-To: <89308DDF-BB85-4982-88D5-3FD93BE034E3@scd-symposium.org> References: <0DEDFEC2-AB49-418F-A3F9-EDF76853B637@scd-symposium.org> <89308DDF-BB85-4982-88D5-3FD93BE034E3@scd-symposium.org> Message-ID: <70A6A096-1108-4431-B2BE-E3A0F9FEAEB5@scd-symposium.org> We would place an epicardial lead with a single chambered ICD in this child with probable Jervell-Lange-Nielsen syndrome.We would not be deterred by the age or size of the child. Jeffrey Towbin, MD Professor and Chief Pediatric Cardiology Texas Children's Hospitasl Baylor College of Medicine Houston, Texas USA -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee > > By our experience of the managment and follow up of children with > congenital > LQTS, beta blockers (Propranolol, Atenolol, Nadolol) therapy in > daily dose > 1-2 mg/kg not significantly increase of bradycardia in this pts. > Sometimes > combination of the regular doses of betablockers (1 mg/kg) and Na > blockers > (we use carbamazepin 10 mg/kg/daily) may be useful. But in this > case patient > had syncope, very long QTc (600 ms) and heart rate 55 bpm for 5 > years old > children on rest ECG is very low. By my opinion children need in > antyarhythmic device in combination with drug therapy. > Dr. Leonid Makarov > Moscow Institute pediatry and children surgery > Moscow, Russia. Taldomskaya str. 2 > leo at oss.ru > > -- > Dr. Sergio Dubner > President of Scientific Committee > > Dr. Edgardo Schapachnik > President of Steering Committee > > >> >> Dear Dr. Dubner, >> >> Thank you and your colleagues for this nice online symposium. >> Recently, a >> 5-year-old children with no evidence of structural heart disease >> referred to >> our center for evaluation of syncope. He also had history of >> congenital >> deafness. One of the episodes of syncope occurred 48-hrs after >> betablocker >> withdrawal and second episodes on 10 mg propranolol tid. Surface ECG >> revealed a QTc=600 ms and heart rate of 55 bpm. We think that >> onset in >> childhood, congenital deafness and QTc=600 would place this >> patient at >> higher risk for cardiac events and therefore a candidate for ICD >> implantation. However, small body size and future risk of >> inappropriate >> therapy with its devastating consequences are unfavorable aspects >> of ICD >> implantation. Our limitimg factors for increase in dose of >> betablocker is >> low baseline hear rate. What is recommendation of scientific >> committee of >> SCD symposium regarding our patient? >> >> Thank you in advance for your recommendation. >> >> Regards, >> >> Majid Haghjoo,MD >> Department of Pacemaker and Electrophysiology >> Rajaie Cardiovascular Medical and Research Center >> Tel: +98 21 2392 2931 >> Fax:+98 21 2204 8174 >> Tehran, Iran > > _______________________________________________ > Scd-forum mailing list > Scd-forum at scd-symposium.org > http://www.grupoakros.com.ar/mailman/listinfo/scd-forum -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061013/72b91e46/attachment.html From info at scd-symposium.org Fri Oct 13 20:11:24 2006 From: info at scd-symposium.org (SCD Symposium) Date: Fri, 13 Oct 2006 20:11:24 -0300 Subject: [SCD-FORUM] 15E RE: A female with burned-out phase of HCM? Dr. Kadish In-Reply-To: References: Message-ID: She appears to have a familial cardiomypopathy. Some cohorts of these patients have progressive heart block and LV dysfunciton. In this case HCM may be associated. In other patients with progressive heart block, an ASD may be present. In any case there is a risk of SCD with pacemaker therapy alone. Certainly, in the presence of severe dysfunction and CHB, I would suggest an ICD CRT device with the hope of improving function and protecting aginst the risk of SCD. Alan Kadish > > Dear Dr Dubner > > thank you for this excellent educational site > > A 38 year old femal referred to our center with new onset dyspnea > from a few month ago. She had family history of sudden cardia > death. She had CHB with wide ventricular escape rhythm. > TT.echocardiography revealed sever LV and RV systolic dysfunction, > asymetric septal hyperthrophy, pulmonary hypertention > (PAP=65-70mmhg) and without LVOT gradient.patient,s dyspnea > improved with low dose diuretics , ACE inhibitor and TPM implantation. > Do you recommend DDD pacemaker for this patient? > or > Do you recommend ICD-DR or ICD-CRT? > > what is your opinion about clinical course of her disease? > > does she need further workup before device implantation? > > sincerely yours > > Hormoz Alizadeh MD > Department of Pacemaker and Electrophysiology > Rajaie Cardiovascular Medical and Research Center > Tehran, Iran > -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061013/c3a1e3cb/attachment.html From info at scd-symposium.org Fri Oct 13 22:19:28 2006 From: info at scd-symposium.org (SCD Symposium) Date: Fri, 13 Oct 2006 22:19:28 -0300 Subject: [SCD-FORUM] 15E RE: A female with burned-out phase of HCM? Dr. Kadish In-Reply-To: References: Message-ID: She appears to have a familial cardiomypopathy. Some cohorts of these patients have progressive heart block and LV dysfunciton. In this case HCM may be associated. In other patients with progressive heart block, an ASD may be present. In any case there is a risk of SCD with pacemaker therapy alone. Certainly, in the presence of severe dysfunction and CHB, I would suggest an ICD CRT device with the hope of improving function and protecting aginst the risk of SCD. Alan Kadish > > Dear Dr Dubner > > thank you for this excellent educational site > > A 38 year old femal referred to our center with new onset dyspnea > from a few month ago. She had family history of sudden cardia > death. She had CHB with wide ventricular escape rhythm. > TT.echocardiography revealed sever LV and RV systolic dysfunction, > asymetric septal hyperthrophy, pulmonary hypertention > (PAP=65-70mmhg) and without LVOT gradient.patient,s dyspnea > improved with low dose diuretics , ACE inhibitor and TPM implantation. > Do you recommend DDD pacemaker for this patient? > or > Do you recommend ICD-DR or ICD-CRT? > > what is your opinion about clinical course of her disease? > > does she need further workup before device implantation? > > sincerely yours > > Hormoz Alizadeh MD > Department of Pacemaker and Electrophysiology > Rajaie Cardiovascular Medical and Research Center > Tehran, Iran > -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061013/f4fb0287/attachment.html From info at scd-symposium.org Sat Oct 14 08:44:59 2006 From: info at scd-symposium.org (SCD Symposium) Date: Sat, 14 Oct 2006 08:44:59 -0300 Subject: [SCD-FORUM] 14R RE: Palpitations and SCD risk. Dr. Makarov In-Reply-To: <81AB3D7E-A763-4A44-965F-B4F199B1ED2B@scd-symposium.org> References: <81AB3D7E-A763-4A44-965F-B4F199B1ED2B@scd-symposium.org> Message-ID: <21ED1D1A-144B-40F6-8052-AA7BFEC0F99F@scd-symposium.org> Dear Dr. Vorotniak, I think that you mentioned a very important issue: the evaluation of the prognostic value of clinical symptoms, which may be concomitant to diseases with high risk of SCD. In general, syncopes and "palpitations" that occur in patients with 2nd or 3rd degree AV block, and also in Long QT and Brugada syndromes, have arrhythmogenic origin. For this reason, aggressive antiarrhythmic treatment (implantation of pacemaker, antiarrhythmic drugs, etc.) is indicated. However, it is often observed in patients with AV block, that after pacemaker implantation, syncope episodes continue happening and no arrhythmias are detected during Holter monitoring. The probability of vasovagal syncopes in patients with Brugada and Long QT syndromes has not been well studied yet. As to "palpitations", it is worth mentioning that up to 16% of patients of all nosological groups may present this symptom (Kroenke K et al.The prevalence of symptoms in medical outpatients and the adequacy of therapy. Arch Intern Med . 1990;150). Moreover, "palpitations" do not always correspond to arrhythmias. In year 1836, J.C. Williams mentioned that often times, "palpitations" are mistakenly considered to be an equivalent of structural heart diseases. But it is not so, because the influences of the nervous system have a very important role, both in the normal state and during some diseases. According to different data, in 35% to 85% of the cases, "palpitations" are recorded in a normal ECG. 1. Baratta L, Maffe o N , Tubani L, Paradiso M, Molaioni C , Coppotelli L, Lagana B, Mastrocola C , Cordova C. Arrhythmias in the aged : prevalence and correlation with symptoms]. Recenti Prog Med 1996 Mar;87(3):96?101. 2. Goldberg AD, Raftery EB, Cashman PM. Ambulatory electrocardiographic records in patients with transient cerebral attacks or palpitation. Br Med J 1975 Dec 6;4(5996):569?71. 3. Hashimoto T., Fucatani M., et al. Effects of stending on the of Paroxysmal Supraventricular Tachycardia. JASS. ? 1991. ?Vol. 17. ? N 3. ? p. 650?695. Our data from the pediatric population show that 30% of "palpitations" have a nonarrhythmogenic origin (Makarov et al, Pediatrics (Moscow) 2005; 2:4-8) and they occur due to psychological causes or autonomous nervous system disorders. Therefore, we assume that the "gold standard" to confirm the arrhythmogenic origin of "palpitations" would be to record them during the same episode, or cause these arrhythmias, accompanied by the corresponding symptoms, during ergometer test or transesophageal electrostimulation. As to the clinical value of "palpitations" ?in patients with short QT interval, Brugada syndrome, arrhythmogenic RV dysplasia, or short PR interval- this symptom may indicate the need for a more aggressive antiarrhythmic treatment, mostly if arrhythmias are detected (in Holter monitoring, EPS, ergometer) or if there are some additional risk factors (syncopes, family background of SCD, T wave alternans in patients with long QT syndrome, etc.) Dr. Leonid Macarov Institute of Pediatrics and Child Surgery (Moscow, Russia) leo @oss.ru > > Dear colleagues, > I would like to ask some questions about the prognostic value of > "palpitations" in Brugada and Long QT syndromes: > > 1. What is the risk of SCD in a young patient with no underlying > heart disease or hereditary-familial background for SCD, with > Brugada syndrome (or Long QT) ECG pattern, and aborted and isolated > episodes of "palpitations" (not recorded with Holter monitoring)? > 2. How do we continue the evaluation of this patient, if in the > electrophysiological study there is no ventricular arrhythmia > triggered? > 3. Is there any relationship between the mentioned syndromes and > supraventricular tachyarrhythmia incidence? > > Thank you very much, > Dr. Andriy Vorotniak > Buenos Aires, Argentina > -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061014/40db3b26/attachment.html From info at scd-symposium.org Sat Oct 14 09:09:50 2006 From: info at scd-symposium.org (SCD Symposium) Date: Sat, 14 Oct 2006 09:09:50 -0300 Subject: [SCD-FORUM] 18E Asymptomatic patient with Brugada syndrome. Dr. Roy Message-ID: <014619D0-FBD0-4876-99E6-C95467B9088E@scd-symposium.org> Dear Dr. Ramon Brugada, As you have discussed in the answer to the question for an asymptomatic patient with brugada syndrome, the use of EPS to risk stratify asymptomatic patient is very controversial. The first symptom may be sudden death in such patients. In such a scenario is there any role for microvolt T wave alternans for risk stratifications and to decide the need for ICD? Dr. Sunil Roy. MD, DM Senior Lecturer in Cardiology Medical college, Calicut, Kerala, India Email: sunilroytn at calicutmedicalcollege.ac.in -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061014/e566f9f1/attachment.html From info at scd-symposium.org Sat Oct 14 10:09:49 2006 From: info at scd-symposium.org (SCD Symposium) Date: Sat, 14 Oct 2006 10:09:49 -0300 Subject: [SCD-FORUM] EXPERTS ASK, EXPERTS ANSWER Message-ID: <9713B578-089C-409E-AD37-55B7C0455445@scd-symposium.org> Dr. Jose Luis Merino from Spain asks - How do you envision the future treatment/prevention of sudden death: drugs, catheter ablation or devices? If you see a major role for the devices, what kind of device do you think will be used for sudden death primary prevention i.e.: simple device only able to deliver few electrical high energy shocks, subcutaneous device without endocardial-epicardial leads, single-dual-triple chamber device, etc). Dr. Andrew Epstein from U.S.A. answers - Unfortunately, the future cannot be predicted. We are in need for new drugs, but the market is focused on devices. The new drugs coming out will be for atrial fibrillation--the ventricles are not of interest to the pharmaceutical industry. Ablation will play a minor role in SD prevention, likely for idiopathic VF and to decrease frequent ICD therapy. Finally, a "simple" device woud be nice, but that has not been the way the market has been driven, and I don't see it happening. Recall the failure of Intermedics and the Biotronik Airbag in this regard. A subcutaneous ICD will be of interest, but clinical trial results are awaited, and we'll have to see how physicians react. -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061014/e693637c/attachment.html From info at scd-symposium.org Sat Oct 14 13:27:00 2006 From: info at scd-symposium.org (SCD Symposium) Date: Sat, 14 Oct 2006 13:27:00 -0300 Subject: [SCD-FORUM] 17S RE: A children with LQTS. Dr. Arabia In-Reply-To: <0DEDFEC2-AB49-418F-A3F9-EDF76853B637@scd-symposium.org> References: <0DEDFEC2-AB49-418F-A3F9-EDF76853B637@scd-symposium.org> Message-ID: Dear Dr. Haghjoo, We agree with you in that your patient is in increased risk of sudden death by the factors you point out. The fact that she repeated a syncope event in spite of 30 mg of propranolol in a 5-year-old child (although we ignore her weight), with a heart rate of 55 with clear signs of beta block, suggests at least unsatisfactory response to such medication. In such a case, we would rather implant an ICD, knowing as a pediatric electrophysiologist, what this decision implies. We would attempt the implantation using an endocardial electrode/coil, but implanted epicardially, surrounding the LV epicardium and the inferior side of the RV. The ICD may even be a dual-chamber one, and an atrial epicardial electrode may be implanted in the RA, attempting to shorten the QT with increase of HR, but allowing intrinsic conduction. The aggressiveness in the management is due to the fact that the next event may be the last in this disease. Congratulations to the organizers of this symposium for their excellent work. Sincerely, Luis Arabia MD. C?rdoba Argentina -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee > > Dear Dr. Dubner, > > Thank you and your colleagues for this nice online symposium. > Recently, a > 5-year-old children with no evidence of structural heart disease > referred to > our center for evaluation of syncope. He also had history of > congenital > deafness. One of the episodes of syncope occurred 48-hrs after > betablocker > withdrawal and second episodes on 10 mg propranolol tid. Surface ECG > revealed a QTc=600 ms and heart rate of 55 bpm. We think that onset in > childhood, congenital deafness and QTc=600 would place this patient at > higher risk for cardiac events and therefore a candidate for ICD > implantation. However, small body size and future risk of > inappropriate > therapy with its devastating consequences are unfavorable aspects > of ICD > implantation. Our limitimg factors for increase in dose of > betablocker is > low baseline hear rate. What is recommendation of scientific > committee of > SCD symposium regarding our patient? > > Thank you in advance for your recommendation. > > Regards, > > Majid Haghjoo,MD > Department of Pacemaker and Electrophysiology > Rajaie Cardiovascular Medical and Research Center > Tel: +98 21 2392 2931 > Fax:+98 21 2204 8174 > Tehran, Iran > _______________________________________________ > Scd-forum mailing list > Scd-forum at scd-symposium.org > http://www.grupoakros.com.ar/mailman/listinfo/scd-forum -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061014/0cec7da1/attachment.html From info at scd-symposium.org Sat Oct 14 13:36:56 2006 From: info at scd-symposium.org (SCD Symposium) Date: Sat, 14 Oct 2006 13:36:56 -0300 Subject: [SCD-FORUM] 19E RE: A children with LQTS. Dr. Zaklyazminskya In-Reply-To: <0DEDFEC2-AB49-418F-A3F9-EDF76853B637@scd-symposium.org> References: <0DEDFEC2-AB49-418F-A3F9-EDF76853B637@scd-symposium.org> Message-ID: <156D1C62-F4C9-43EE-A7BA-FE11FCDF82EF@scd-symposium.org> Dear Dr. Hormoz Alizadeh ! I think that you are absolutely right accounting this patient as a candidate for ICD implantation. Most likely this boy suffers from Jervell and Lange-Nielsen syndrome caused both affected alleles of KCNQ1 or KCNE1 genes. In our Research Center we dispose of the information about 15 patients with two independent mutations in ionic channel genes from the families who applied DNA Diagnostics or genetic counselling. Some of them had deafness and QT interval prolongation, but the other had only LQTS or SSS+BrS. Nobody survived the age of 20 years without implanted devices (ICD or PM in the case of SSS). Now, we suppose that the revelation of more than one mutation in ionic channel genes (trans-position) is an independent risk factor of SCD. We recommend genetic counselling and DNA diagnostics for the families. Sincerely, Dr. Elena Zaklyazminskya, MD, PhD Russian Research Center for Medical Genetics dnalab at rol.ru -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee > > Dear Dr. Dubner, > > Thank you and your colleagues for this nice online symposium. > Recently, a > 5-year-old children with no evidence of structural heart disease > referred to > our center for evaluation of syncope. He also had history of > congenital > deafness. One of the episodes of syncope occurred 48-hrs after > betablocker > withdrawal and second episodes on 10 mg propranolol tid. Surface ECG > revealed a QTc=600 ms and heart rate of 55 bpm. We think that onset in > childhood, congenital deafness and QTc=600 would place this patient at > higher risk for cardiac events and therefore a candidate for ICD > implantation. However, small body size and future risk of > inappropriate > therapy with its devastating consequences are unfavorable aspects > of ICD > implantation. Our limitimg factors for increase in dose of > betablocker is > low baseline hear rate. What is recommendation of scientific > committee of > SCD symposium regarding our patient? > > Thank you in advance for your recommendation. > > Regards, > > Majid Haghjoo,MD > Department of Pacemaker and Electrophysiology > Rajaie Cardiovascular Medical and Research Center > Tel: +98 21 2392 2931 > Fax:+98 21 2204 8174 > Tehran, Iran > _______________________________________________ > Scd-forum mailing list > Scd-forum at scd-symposium.org > http://www.grupoakros.com.ar/mailman/listinfo/scd-forum -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061014/d582d6ff/attachment.html From info at scd-symposium.org Sat Oct 14 14:52:26 2006 From: info at scd-symposium.org (SCD Symposium) Date: Sat, 14 Oct 2006 14:52:26 -0300 Subject: [SCD-FORUM] 20E RE: Palpitations and SCD risk. Dr. Perez Riera Message-ID: Dr. Andriy Vorotniak from Buenos Aires, Argentina. Here Andres Ricardo Perez Riera from SP Brazil. THE INCIDENCE OF SUPRAVENTRICULAR ARRHYTHMIAS IN THE BRUGADA SYNDROME Sinus rhythm is the usual; however, Brugada syndrome (BrS) patients exhibit an abnormally high proportion of atrial arrhythmias that are found in 10 to 25% of cases since the arrhythmogenic substrate is not limited to the ventricles. In the original discovery by the Brugada brothers (1992)(1), temporary AF was mentioned, as well as by authors from Brazil (2) and from Japan (3). The latter mentioned that the paroxysmal form of AF is observed in a 30% of cases. A publication by Eckardt L et al (2001) (4), indicates a frequency for supraventricular arrhythmias of 29%. These authors described episodes of AV supraventricular tachycardia with reentry. There are references of Wolff-Parkinson-White syndrome A type associated to BrS (5-6). There is a more advanced disease process in BrS patients with spontaneous atrial arrhytmias and ventricular inducibility was significantly related to a history of atrial arrhythmias.The incidence of atrial arrhythmias in patients with a spontaneous electrocardiogram of BrS was 26% vs 10% in patients with a flecainide-induced ECG.In patients with an indication of ICD, the incidence of atrial arrhythmias reached 27% vs 13% in patients with BrS but without ICD indication; Inappropriate shocks due to atrial arrhythmias episodes were observed in 14% of ICD patient's vs 10.5% of appropriate shocks; The implantation of a single-chamber device is as an independent predictive factor of inappropriate ICD discharges; Careful programming of single-chamber ICD should be recommended to avoid inappropriate discharges in patients with BrS (7). Arrhythmia of atrial origin was the only spontaneous pathologic rhythmic observed in a 46 years old man patient with BrS by Boveda et al (8). Consequently it led to reconsider its prevalence in patients presenting this syndrome both in the literature and according Boveda's time personal experience. A 41-year-old man with BrS and no previous episodes of aborted SCD or syncope referred to local emergency room for an episode of symptomatic AF. Blood chemistry results showed hypokalemia(2.9 mEq/L). The other parameters were within the normal range. After few minutes, an episode of VF treated with biphasic DC shock 150 J occurred. In successive 2 hours, the patient experienced recurrent episodes of VT and VF. Each biphasic DC shock 150 J was effective to restore sinus rhythm. No further episodes occurred after normalization of serum levels of potassium. Before discharge, an ICD was inserted to prevent SCD. Hypokalemia increases the risk of arrhythmic events in BrS (9). Hypokalemia increases the risk of arrhythmic events in BrS(10). Sinus node dysfunction (SND) is not a rare concomitant disorder in BrS and there is a possible genetic connection. SND is associated with AF(11). References 1) Brugada P, Brugada J. Right bundle branch block, persistent ST segment elevation and sudden cardiac death: A distinct clinical and electrocardiographic syndrome. J Am Coll Cardiol 1992, 20: 1391-1396 2) Villacorta H, Faig Torres RA, SimF5es de Castro IR, Lambert H. de Araujo Gonzales Alonso R.: Morte subita em paciente com bloqueio de ramo direito e elevacao persistente do segmento ST. Arq Bras Cardiol. 1996; 66:( N4) 229-231 3) Itoh H, Shimizu M, Ino H, et al. Hokuriku Brugada Study Group. Arrhythmias in-patients with Brugada-type electrocardiograph findings. Jpn Circ J 2001; 65:483-6 4) Eckardt L, Kirchhof P, Loh P, et al. Brugada Syndrome and Supraventricular Tachyarrhythmias: A Novel Association? J Cardiovasc Electrophysiol 2001; 12:680-685 5) Eckardt L, Kirchhof P, Johna R, Haverkamp W, Breithardt G, Borggrefe M. : Wolff-Parkinson-White syndrome associated with Brugada syndrome. Pacing Clin Electrophysiol 2001;24(9 Pt 1):1423-4. 6) Bodegas AI, Arana JI, Vitoria Y, Arriandiaga JR, Barrenetxea JI. Brugada syndrome in a patient with accessory pathway. Europace 2002; 4:87-9 7) Bordachar P, Reuter S, Garrigue S, Cai X, Hocini M, Jais P, Haissaguerre M, Clementy J. Incidence, clinical implications and prognosis of atrial arrhythmias in brugada syndrome.Eur Heart J. 2004;25:879-884. 8) Boveda S, Combes N, Albenque JP, et al. Brugada syndrome and supraventricular arrhythmiasArch Mal Coeur Vaiss. 2004; 97: 688-692. 9) Notarstefano P, Pratola C, Toselli T, et al. Atrial fibrillation and recurrent ventricular fibrillation during hypokalemia in Brugada syndrome. Pacing Clin Electrophysiol. 2005; 28:1350-1353. 10) Notarstefano P, Pratola C, Toselli T, et al Atrial fibrillation and recurrent ventricular fibrillation during hypokalemia in Brugada syndrome. 11) Sumiyoshi M, Nakazato Y, Tokano T, Sinus node dysfunction concomitant with Brugada syndrome. Circ J. 2005; 69:946-950. All the best Andr?s Ricardo P?rez Riera Chief of Electro-Vectocardiology Sector of the Discipline of Cardiology, ABC Faculty of Medicine (FMABC), Foundation of ABC (FUABC) - Santo Andr? - S?o Paulo - Brazil. Rua Sebasti?o Afonso 885 - Zip Code: 044417-100- Jardim Miriam S.P Brazil- -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061014/c99d0f1e/attachment.html From info at scd-symposium.org Sat Oct 14 15:14:39 2006 From: info at scd-symposium.org (SCD Symposium) Date: Sat, 14 Oct 2006 15:14:39 -0300 Subject: [SCD-FORUM] 21E RE: A children with LQTS. Dr. Ray Jordan In-Reply-To: <70A6A096-1108-4431-B2BE-E3A0F9FEAEB5@scd-symposium.org> References: <0DEDFEC2-AB49-418F-A3F9-EDF76853B637@scd-symposium.org> <89308DDF-BB85-4982-88D5-3FD93BE034E3@scd-symposium.org> <70A6A096-1108-4431-B2BE-E3A0F9FEAEB5@scd-symposium.org> Message-ID: Dear Jeff, Do you place the epicardial ICD percutaneously under floroscopy? or echo or go transvenous through the the heart veins in the cath lab? Because of the size and anatomical constraints, we presume the percutaneous approach so, in brief what's TCH, or your, favored approach? Richard-Ray/dba Richard Ray Jordan, M.D. for Semper Fides Medical -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee > > We would place an epicardial lead with a single chambered ICD in > this child with probable Jervell-Lange-Nielsen syndrome.We would > not be deterred by the age or size of the child. > > Jeffrey Towbin, MD > Professor and Chief > Pediatric Cardiology > Texas Children's Hospitasl > Baylor College of Medicine > Houston, Texas USA > > -- > Dr. Sergio Dubner > President of Scientific Committee > > Dr. Edgardo Schapachnik > President of Steering Committee > > >> >> By our experience of the managment and follow up of children with >> congenital >> LQTS, beta blockers (Propranolol, Atenolol, Nadolol) therapy in >> daily dose >> 1-2 mg/kg not significantly increase of bradycardia in this pts. >> Sometimes >> combination of the regular doses of betablockers (1 mg/kg) and Na >> blockers >> (we use carbamazepin 10 mg/kg/daily) may be useful. But in this >> case patient >> had syncope, very long QTc (600 ms) and heart rate 55 bpm for 5 >> years old >> children on rest ECG is very low. By my opinion children need in >> antyarhythmic device in combination with drug therapy. >> Dr. Leonid Makarov >> Moscow Institute pediatry and children surgery >> Moscow, Russia. Taldomskaya str. 2 >> leo at oss.ru >> >> -- >> Dr. Sergio Dubner >> President of Scientific Committee >> >> Dr. Edgardo Schapachnik >> President of Steering Committee >> >> >>> >>> Dear Dr. Dubner, >>> >>> Thank you and your colleagues for this nice online symposium. >>> Recently, a >>> 5-year-old children with no evidence of structural heart disease >>> referred to >>> our center for evaluation of syncope. He also had history of >>> congenital >>> deafness. One of the episodes of syncope occurred 48-hrs after >>> betablocker >>> withdrawal and second episodes on 10 mg propranolol tid. Surface ECG >>> revealed a QTc=600 ms and heart rate of 55 bpm. We think that >>> onset in >>> childhood, congenital deafness and QTc=600 would place this >>> patient at >>> higher risk for cardiac events and therefore a candidate for ICD >>> implantation. However, small body size and future risk of >>> inappropriate >>> therapy with its devastating consequences are unfavorable aspects >>> of ICD >>> implantation. Our limitimg factors for increase in dose of >>> betablocker is >>> low baseline hear rate. What is recommendation of scientific >>> committee of >>> SCD symposium regarding our patient? >>> >>> Thank you in advance for your recommendation. >>> >>> Regards, >>> >>> Majid Haghjoo,MD >>> Department of Pacemaker and Electrophysiology >>> Rajaie Cardiovascular Medical and Research Center >>> Tel: +98 21 2392 2931 >>> Fax:+98 21 2204 8174 >>> Tehran, Iran >> >> _______________________________________________ >> Scd-forum mailing list >> Scd-forum at scd-symposium.org >> http://www.grupoakros.com.ar/mailman/listinfo/scd-forum > > _______________________________________________ > Scd-forum mailing list > Scd-forum at scd-symposium.org > http://www.grupoakros.com.ar/mailman/listinfo/scd-forum -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061014/8156fd2e/attachment.html From info at scd-symposium.org Sat Oct 14 18:18:16 2006 From: info at scd-symposium.org (SCD Symposium) Date: Sat, 14 Oct 2006 18:18:16 -0300 Subject: [SCD-FORUM] 24E RE: CLBBB. Dr. Perez Riera Message-ID: <0413A022-442E-425D-83AC-29B12BCE5DEB@scd-symposium.org> Dear Dr Jorge Ruiz Alais here answer Andr?s Ricardo P?rez Riera ffrom S?o Paulo Brazil. In apparently healthy men the Manitoba prospective population follow- Up study (1948 to 2004) showed that newly developed complete left bundle branch block (CLBBB) was a highly significant short-term risk to sudden unexpected cardiac death that diminished with time (1). Cardiac resynchronization therapy (CRT) has significant positive effects on the quality of life, enables patients to cope more efficiently with cardiopulmonary stress and leads to a reduction of total mortality in patients suffering from congestive HF NYHA classes III and IV, reduced LV function and CLBBB with a QRSd > 150 ms. Prolongation of QRSd > or =120 ms occurs in 14% to 47% of HF patients. LBBB is far more common than RBBB. Left-sided intraventricular conduction delay is associated with more advanced myocardial disease, worse LV function, poorer prognosis, and a higher all-cause mortality rate compared with narrow QRS complex. In a large number of patients suited for CRT, an additional defibrillator function seems to work out well concerning an additional prognostic improvement by means of reducing SCD. Due to partially contradictory study outcomes, it still remains to be discussed whether all patients suited for CRT really need an ICD (2). In ICD patients with HF, a wide underlying QRS complex more than doubles the cardiac mortality compared with a narrow QRS complex. There is a high incidence of an elevated defibrillation threshold at the time of ICD implantation in patients with QRS > or =200 ms. Mechanical LV dyssynchrony potentially treatable by ventricular resynchronization occurs in about 70% of HF patients with left-sided intraventricular conduction delay, a fact that would explain the lack of therapeutic response in about 30% of patients subjected to ventricular resynchronization according to standard criteria relying on QRS duration. The duration of the basal QRS complex does not reliably predict the clinical response to ventricular resynchronization, and QRS narrowing after cardiac resynchronization therapy does not correlate with hemodynamic and clinical improvement. Mechanical LV dyssynchrony is best shown by evolving echocardiographic techniques (predominantly tissue Doppler imaging) currently in the process of standardization (3). The presence of BBB is strongly associated with future high-degree atrioventricular block that was more pronounced for LBBB. Men with LBBB have a substantially increased risk of coronary death, mainly due to SCD outside the hospital setting (4). L?negre disease is the eponym of "idiopathic" progressive disease of the His-Purkinje system. It is also knows as Progressive Cardiac Conduction Defect (PCCD). The disease has been identified as an entity that affect the sodium fast channel (channelopathy entity) occasioned by mutation in the SCN5A gene. It is an allelic of Brugada disease with a different phenotypic expression. The same missence mutation in the SCN5A gene can cause both phenotypes: Brugada and L?negre disease. So, we now ask for scientific community: why L?negre has disease category and Brugada entity has not?. Is it certain? Both entities, called Progressive Cardiac Conduction Defects (PCCD), are grouped together as primary conduction diseases (Lev-Len?gre). Both Len?gre disease-known as "primary" PCCD (5)-as well as the secondary mechanic lesion-sclerosis of the left "cardiac skeleton" or Lev disease (6)- usually cause LBBB or RBBB, frequently associated with divisional blocks. Occasionally, they develop into more advanced degrees of block with a potential to cause SCD due to total AV block, to the extent that they represent the most important cause of pacemaker implantation in the first world: 0.15 per 1,000 inhabitants a year. The same mutation in novel single SCN5A missense mutation can lead either to Brugada syndrome or to an PCCD. Modifier gene(s) may influence the phenotypic consequences of a SCN5A mutation. A G-to-T mutation at position 4372 was identified by direct sequencing and was predicted to change a glycine for an arginine (G1406R) between the DIII-S5 and DIII-S6 domain of the Na+ channel protein (7). Trimetazidine (TMZ) Vartel a clinically effective antianginal agent with no negative inotropic or vascular properties, acts by optimizing cardiac energy metabolism through inhibition of free faty acid oxidation, shifting substrate utilization from fatty acids to glucose. long-term trimetazidine improves functional class and LV function in patients with HF. This benefit contrasts with the natural history of the disease, as shown by the decrease of EF in patients on standard HF therapy alone. Recently, results have demonstrated that trimetazidine improves radial artery endothelium-dependent relaxation related to its antioxidant properties. Similarly, exercise training has been demonstrated to improve diastolic filling and systolic function in patients with ischemic cardiomyopathy, in relation to enhanced perfusion and contractility of dysfunctional myocardium. Patients with viable myocardium, in theory, should have the greatest benefits because trimetazidine improves contractility of dysfunctional hibernating/stunned myocardium, whereas exercise has documented efficacy in improving endothelial vasomotor response of coronary arteries, stimulating coronary collateral circulation and small vessel growth, improving LV function, and increasing functional capacity. References 1) Cuddy TE, Tate RB. Sudden unexpected cardiac death as a function of time since the detection of electrocardiographic and clinical risk factors in apparently healthy men: the Manitoba Follow- Up Study, 1948 to 2004. Can J Cardiol. 2006;22:205-11. 2) Volkmann H, Bergmann C, Walter M.Cardiac resynchronization therapy: who is suitable? Who requires an additional ICD as a backup? Z Kardiol. 2005; 94:60-64. 3) Kashani A, Barold SS. Significance of QRS complex duration in patients with heart failure. J Am Coll Cardiol. 2005;46:2183-92. 4) Eriksson P, Wilhelmsen L, Rosengren A.Bundle-branch block in middle-aged men: risk of complications and death over 28 years. The Primary Prevention Study in Goteborg, Sweden. Eur Heart J. 2005;26:2222-3. 5) Len?gre J.The pathology of complete atrioventricular block. Progr Cardiovasc Dis 1964; 6:317-323. 6) Lev M. Anatomic basis of atrioventricular block. Am J Med 196437:742. 7) Kyndt F, Probst V, Potet F, et. al. Novel SCN5A Mutation Leading Either to Isolated Cardiac Conduction Defect or Brugada Syndrome in a Large French Family. Circulation 2001; 104: 3081-3086 All the best Andr?s Ricardo P?rez Riera Chief of Electro-Vectocardiology Sector of the Discipline of Cardiology, ABC Faculty of Medicine (FMABC), Foundation of ABC (FUABC) - Santo Andr? - S?o Paulo - Brazil. Rua Sebasti?o Afonso 885 - Zip Code: 044417-100- Jardim Miriam S.P Brazil- > > Dear friends, > Congratulations on such a novel and scientific symposium on heart > pathologies, for I am personally quite surprised with the advanced > progresses that help to clarify and understand lots of pathologies > still unknown to many colleagues. By the way, I would like to know > about the newest advancements about complete left bundle branch block > of his bundle; if this may actually be a pathology that may cause > sudden death; clarify its etiology, prognosis, proper management, > what other complications it may have, and if possible, whether there > is some innovative treatment to endure this condition. I would also > like to know what is the experience regarding the cardiac ischemia > treatment with vastarel (trimetazidine dichlorhydrate). > Thank you very much for your consideration to this message. > I remain waiting for your kind response, > > JORGE RUIZ ALAIS -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061014/7d2806fa/attachment.html From info at scd-symposium.org Sat Oct 14 20:02:08 2006 From: info at scd-symposium.org (SCD Symposium) Date: Sat, 14 Oct 2006 20:02:08 -0300 Subject: [SCD-FORUM] 22E RE: A children with LQTS. Dr. Dubner In-Reply-To: <0DEDFEC2-AB49-418F-A3F9-EDF76853B637@scd-symposium.org> References: <0DEDFEC2-AB49-418F-A3F9-EDF76853B637@scd-symposium.org> Message-ID: Dear Dr. Majid Haghoo You describe a difficult situation and I am not a pediatric specialist. Certainly, with a QTc larger than 500 msec and congenital deafness your patient is at high risk. If the child was older, an ICD should be implanted. At 5 years of age, the ICD could be more of a problem then a benefit. There are many problems with ICD implantation and maintenance in this group of patients. Under the special circumstances it is warranted, and possibly using a few different implant techniques. So certainly, recurrence of LQT symptoms on meds in this child would be an indication. If necessary and according with world experts, I'd probably try a subcutaneous coil in the back with an abdominal can. On the other hand, it seems to me that a genetic test should be important in this young patient. Identifying the genetic mutation could guide you with the pharmacological treatment. You should contact Arthur Moss, MD at Rochester University (Rochester, NY) to include your patient in the LQTS registry and make the test and Philip Saul, MD at Medical University of South Carolina (Charleston, SC) for surgical techniques. Regards Dr. Sergio Dubner -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee > > Dear Dr. Dubner, > > Thank you and your colleagues for this nice online symposium. > Recently, a > 5-year-old children with no evidence of structural heart disease > referred to > our center for evaluation of syncope. He also had history of > congenital > deafness. One of the episodes of syncope occurred 48-hrs after > betablocker > withdrawal and second episodes on 10 mg propranolol tid. Surface ECG > revealed a QTc=600 ms and heart rate of 55 bpm. We think that onset in > childhood, congenital deafness and QTc=600 would place this patient at > higher risk for cardiac events and therefore a candidate for ICD > implantation. However, small body size and future risk of > inappropriate > therapy with its devastating consequences are unfavorable aspects > of ICD > implantation. Our limitimg factors for increase in dose of > betablocker is > low baseline hear rate. What is recommendation of scientific > committee of > SCD symposium regarding our patient? > > Thank you in advance for your recommendation. > > Regards, > > Majid Haghjoo,MD > Department of Pacemaker and Electrophysiology > Rajaie Cardiovascular Medical and Research Center > Tel: +98 21 2392 2931 > Fax:+98 21 2204 8174 > Tehran, Iran -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061014/67451554/attachment.html From info at scd-symposium.org Sat Oct 14 20:40:36 2006 From: info at scd-symposium.org (SCD Symposium) Date: Sat, 14 Oct 2006 20:40:36 -0300 Subject: [SCD-FORUM] 23E RE: A female with burned-out phase of HCM? Dr. Dubner In-Reply-To: References: Message-ID: Dear Hormoz Alizadeh If your patient has complete AV block with wide ventricular escape, surely she needs electrical stimulation. If there is atrial activity, a dual chamber is indicated. You do not mention any risk factor to think about an ICD, unless you assume this is a cardiomyopathy with severe LV and RV dysfunction, and on the other hand, to indicate a CRT device it should be interesting to know if she has LBBB, if the systolic dysfunction you mention implies ventricular dyssynchrony, or if you can perform tissue Doppler or any other diagnostic method that shows dysynchrony. If there is no dyssynchrony, I should go on with a DDDR pacemaker or think about a dual chamber ICD in case you confirm a cardiomyopathy. Best regards, Sergio Dubner > > Dear Dr Dubner > > thank you for this excellent educational site > > A 38 year old femal referred to our center with new onset dyspnea > from a few month ago. She had family history of sudden cardia > death. She had CHB with wide ventricular escape rhythm. > TT.echocardiography revealed sever LV and RV systolic dysfunction, > asymetric septal hyperthrophy, pulmonary hypertention > (PAP=65-70mmhg) and without LVOT gradient.patient,s dyspnea > improved with low dose diuretics , ACE inhibitor and TPM implantation. > Do you recommend DDD pacemaker for this patient? > or > Do you recommend ICD-DR or ICD-CRT? > > what is your opinion about clinical course of her disease? > > does she need further workup before device implantation? > > sincerely yours > > Hormoz Alizadeh MD > Department of Pacemaker and Electrophysiology > Rajaie Cardiovascular Medical and Research Center > Tehran, Iran > > -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061014/18c821b4/attachment.html From info at scd-symposium.org Sun Oct 15 08:25:16 2006 From: info at scd-symposium.org (SCD Symposium) Date: Sun, 15 Oct 2006 08:25:16 -0300 Subject: [SCD-FORUM] EXPERTS ASK, EXPERTS ANSWER Message-ID: <8F966AAA-B88C-4DFD-908C-92F8D4E448E5@scd-symposium.org> Dr. Victor Moga from Romania asks - Since heart failure is a major cause of mortality, morbity and hospitalization, which is the best approach for clinical application of the cardiac resynchronisation therapy? Dr. Arthur Moss from U.S.A. answers The approved AHA/ACC/ESC criteria for cardiac resynchronization therapy (CRT) are NYHA III or IV, EF<0.30, and QRS>0.12s in ischemic or non-ischemic heart disease. In patients in sinus rhythm, dual chamber biventricular pacing should be used with the AV interval setting in the physiologic range. Optimization of the device programming usually requires concurrent echo/doppler evaluation. Left cephalic or left subclavian vein approach usually provides the easiest access to the coronary veins. In general the LV pacing electrodes should be placed in the lateral coronary vein and not in the anterior vein -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061015/0c0b4a03/attachment.html From info at scd-symposium.org Sun Oct 15 09:00:44 2006 From: info at scd-symposium.org (SCD Symposium) Date: Sun, 15 Oct 2006 09:00:44 -0300 Subject: [SCD-FORUM] 26E RE: A children with LQTS. Dr. Peter Schwartz In-Reply-To: <156D1C62-F4C9-43EE-A7BA-FE11FCDF82EF@scd-symposium.org> References: <0DEDFEC2-AB49-418F-A3F9-EDF76853B637@scd-symposium.org> <156D1C62-F4C9-43EE-A7BA-FE11FCDF82EF@scd-symposium.org> Message-ID: <8F660795-AC6D-40BA-9EC6-DEC14F724CCE@scd-symposium.org> From what I have seen, this little boy from Iran almost certainly suffers from the Jervell and Lange-Nielsen syndrome. Based on the clinical description the use of an ICD is rational, while continuing maximal therapy with beta-blockers to reduce risks of shock. I would suggest that the physicians in charge of this little boy read our extensive study on J-LN syndrome published in Circulation February 14 2006, which was based on 186 such patients. I am available to do genotyping of this child free of charge. Peter J. Schwartz Professor Chairman Dept. of Cardiology University of Pavia Pavia, Italy -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee > > Dear Dr. Hormoz Alizadeh ! > I think that you are absolutely right accounting this patient as a > candidate for ICD implantation. Most likely this boy suffers from > Jervell and Lange-Nielsen > syndrome caused both affected alleles of KCNQ1 or KCNE1 genes. In our > Research Center we dispose of the information about 15 patients > with two > independent mutations in ionic channel genes from the families who > applied > DNA Diagnostics or genetic counselling. Some of them had deafness > and QT > interval prolongation, but the other had only LQTS or SSS+BrS. Nobody > survived the age of 20 years without implanted devices (ICD or PM > in the > case of SSS). Now, we suppose that the revelation of more than one > mutation > in ionic channel genes (trans-position) is an independent risk > factor of > SCD. > We recommend genetic counselling and DNA diagnostics for the families. > Sincerely, > Dr. Elena Zaklyazminskya, MD, PhD > Russian Research Center for Medical Genetics > > dnalab at rol.ru > > -- > Dr. Sergio Dubner > President of Scientific Committee > > Dr. Edgardo Schapachnik > President of Steering Committee > > >> >> Dear Dr. Dubner, >> >> Thank you and your colleagues for this nice online symposium. >> Recently, a >> 5-year-old children with no evidence of structural heart disease >> referred to >> our center for evaluation of syncope. He also had history of >> congenital >> deafness. One of the episodes of syncope occurred 48-hrs after >> betablocker >> withdrawal and second episodes on 10 mg propranolol tid. Surface ECG >> revealed a QTc=600 ms and heart rate of 55 bpm. We think that >> onset in >> childhood, congenital deafness and QTc=600 would place this >> patient at >> higher risk for cardiac events and therefore a candidate for ICD >> implantation. However, small body size and future risk of >> inappropriate >> therapy with its devastating consequences are unfavorable aspects >> of ICD >> implantation. Our limitimg factors for increase in dose of >> betablocker is >> low baseline hear rate. What is recommendation of scientific >> committee of >> SCD symposium regarding our patient? >> >> Thank you in advance for your recommendation. >> >> Regards, >> >> Majid Haghjoo,MD >> Department of Pacemaker and Electrophysiology >> Rajaie Cardiovascular Medical and Research Center >> Tel: +98 21 2392 2931 >> Fax:+98 21 2204 8174 >> Tehran, Iran -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061015/05089453/attachment.html From info at scd-symposium.org Sun Oct 15 10:03:56 2006 From: info at scd-symposium.org (SCD Symposium) Date: Sun, 15 Oct 2006 10:03:56 -0300 Subject: [SCD-FORUM] 27E RE: A children with LQTS. Dr. Furlani In-Reply-To: <0DEDFEC2-AB49-418F-A3F9-EDF76853B637@scd-symposium.org> References: <0DEDFEC2-AB49-418F-A3F9-EDF76853B637@scd-symposium.org> Message-ID: <831A67FA-D6DC-41E4-86C6-FF5E6E9E8784@scd-symposium.org> Dear Dr Haghjoo: This a difficult case, which sounds like Jervell and Lange-Nielsen Syndrome. The patient is a 5 years old kid what makes this case even more difficult. No perfect options/treatment strategies are available in dealing with patient yet. Dr Peter Schwartz and the international registry for LQTS have helped us to start to understand this life threatening disease and learn how to manage this patients, but there is still a long way to go. In this case, the early onset of the symptoms, presence of congenital deafness, severity of QT prolongation (over 500 msecs), and lack of response (or parcial response) to beta-blocker therapy, are all markers of high risk for SCD. On the other hand, as you pointed out in your e-mail, ICD implantation in children is associated with important colateral problems, which should be kept in mind in considering this therapeutic option. Different options come to my mind in dealing with this patient: 1) Increase the dosage of propranolol (or change it for 1 mg/Kg of Nadolol Die) even in the presence of mild bradycardia. In this patients we can tolerate until 40 bpm while they are asymptomatic or mildly symptomatic. 2) Left cardiac sympathetic denervation plus Nadolol 1 mg/Kg Die. 3) ICD implantation if the patient has another episode of syncope after option 2. 4) Of course, all viable options have to include limitation of physical activity since this patients usually die while exercising and swimming is particularly hazardous. Genotyping the patient is not so useful in this case since most of the JLN patients have KvLQT1 (LQT1) and KCNE1 (LQT5) abnormalities. Hopefully, in the future gene therapy will be the first therapeutic option to actually cure these patients. Thank you. My best regards for everybody, Aldo Alberto Furlani MD Cardiac electrophysiologist Consultant Cardiologist Heart Institute of the Caribbean Mandeville Jamaica afurlani at caribbeanheart.com www.caribbeanheart.com -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee > > Dear Dr. Dubner, > > Thank you and your colleagues for this nice online symposium. > Recently, a > 5-year-old children with no evidence of structural heart disease > referred to > our center for evaluation of syncope. He also had history of > congenital > deafness. One of the episodes of syncope occurred 48-hrs after > betablocker > withdrawal and second episodes on 10 mg propranolol tid. Surface ECG > revealed a QTc=600 ms and heart rate of 55 bpm. We think that onset in > childhood, congenital deafness and QTc=600 would place this patient at > higher risk for cardiac events and therefore a candidate for ICD > implantation. However, small body size and future risk of > inappropriate > therapy with its devastating consequences are unfavorable aspects > of ICD > implantation. Our limitimg factors for increase in dose of > betablocker is > low baseline hear rate. What is recommendation of scientific > committee of > SCD symposium regarding our patient? > > Thank you in advance for your recommendation. > > Regards, > > Majid Haghjoo,MD > Department of Pacemaker and Electrophysiology > Rajaie Cardiovascular Medical and Research Center > Tel: +98 21 2392 2931 > Fax:+98 21 2204 8174 > Tehran, Iran > _______________________________________________ > Scd-forum mailing list > Scd-forum at scd-symposium.org > http://www.grupoakros.com.ar/mailman/listinfo/scd-forum -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061015/d6d863ef/attachment.html From info at scd-symposium.org Sun Oct 15 11:05:27 2006 From: info at scd-symposium.org (SCD Symposium) Date: Sun, 15 Oct 2006 11:05:27 -0300 Subject: [SCD-FORUM] 28E Young woman with LQTS. Dr. Kukla Message-ID: <7C68B657-9683-48C7-89B9-C5ECFF4C1519@scd-symposium.org> First of all I would like to say "thank you" for the nice on-line symposium on SCD. I would kindly ask Prof. Silvia Priori....worldwide famous expert on LQTS Namely, the one of my patient is a 23-yo woman.....LQTS was diagnosed 2 years ago. It was 5 months after first (and the only) partum. She had lost of conscious. QT interval in rest ECG - 515 msec, in Holter monitorng....PVc -300/day, QT in nighttime till 600 msec, dynamicity in T-wave complex, postextrasystolic TU wave augmentation. Exercise test induced momomorphic PVC (monomorphic bigeminy)......PVC disapeared totally in recovery phase. Rest ECG shows notched T wave in V2-V5 so it looks like LQTS type 2. There is no evidence of SCD in her family. We started treatment with Beta-bloker -metoprolol until dose 3 x 75 mg (this dose is well tolerated by her...with no syncope so far). (Nadolol is not available in Poland now). Because she plans to have the next pregnancy. My questions are.. 1) what should be the proper therapy during pregnancy ? 2) how to carry safe the patient during the delivery period ? 3) should I increase the dose of beta-bloker in post-partum period ? with best regard, Dr Piotr Kukla Specialistic Hospital in Gorlice Departament of Internal Medicine Gorlice, Poland -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061015/c60623ea/attachment.html From info at scd-symposium.org Sun Oct 15 18:48:05 2006 From: info at scd-symposium.org (SCD Symposium) Date: Sun, 15 Oct 2006 18:48:05 -0300 Subject: [SCD-FORUM] 29E Trimetazidine. Dr. Ben Khedda Message-ID: <92EAACAE-74EA-478D-845D-381C2EC6110E@scd-symposium.org> Dear colleagues, Congratulation for this exellent meeting; I want to ask a question to Dr Andr?s Ricardo P?rez Riera. in term of evidence based medecine is there any trial showing an improvoment of morbi mortality by using Trimetazidine (TMZ) Vartel Thank you very much for your consideration to this message. salim BEN KHEDDA cardiology division Mustapha Hospital medical Center Algeirs Algeria -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061015/3c1f0498/attachment.html From info at scd-symposium.org Sun Oct 15 18:55:00 2006 From: info at scd-symposium.org (SCD Symposium) Date: Sun, 15 Oct 2006 18:55:00 -0300 Subject: [SCD-FORUM] 30E RE: Asymptomatic patient with Brugada syndrome. Dr. Perez Riera In-Reply-To: <014619D0-FBD0-4876-99E6-C95467B9088E@scd-symposium.org> References: <014619D0-FBD0-4876-99E6-C95467B9088E@scd-symposium.org> Message-ID: <4F56646D-8BEA-441B-81B7-6AEFDBB1EBFD@scd-symposium.org> Dear Dr. Sunil Roy. MD, DM from India. Here Andres Ricardo Perez Riera. You asked to Ramon Brugada about if is there any role for microvolt T wave alternans for risk stratifications and to decide the need for ICD? T wave alternans (TWA) does not assess arrhythmic risk in patients with Brugada syndrome (BrS). It is not an appropriate test to detect arrhythmic risk in patients with BrS (1). Ikeda et al. enrolled 124 consecutive subjects with a Brugada-type ECG. Prognostic indices included: age, sex, a family history of SD, syncopal episodes, a spontaneous Type 1 ST-segment elevation, maximal magnitude of ST-segment elevation, a spontaneous change in ST segment, a mean QRSd, maximal QT interval, QT dispersion, LPs by SA-ECG, and TWA. Of the 12 risk indices, only a family history of SD, syncopal episodes, a spontaneous type 1 ST-segment elevation, a spontaneous change in ST segment, and LP had significant values (2).TWA does not assess arrhythmic risk References 1) Kirchhof P, Eckardt L, Rolf S, T wave alternans does not assess arrhythmic risk in patients with Brugada syndrome. Ann Noninvasive Electrocardiol. 2004;9:162-165.20 2) Ikeda T, Takami M, Sugi K, et al. Noninvasive risk stratification of subjects with a Brugada-type electrocardiogram and no history of cardiac arrest. 20 All the best AndrE9s Ricardo PE9rez Riera20 Chief of Electro-Vectocardiology Sector of the Discipline of Cardiology,ABC Faculty of Medicine (FMABC), Foundation of ABC (FUABC)- Santo AndrE9 -SE3o Paulo - Brazil. Rua SebastiE3o Afonso 885 - Zip Code: 044417-100- Jardim Miriam S.P.- Brazil - Phone: 5504-6243 Fax: 5506-0398. -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee El 14/10/2006, a las 9:09, SCD Symposium escribi?: > Forum of the ISHNE Sudden Cardiac Death World-Wide Internet Symposium > ______________________________________________________________________ > > SCD-HeFT trial shows ICD therapy mortality benefit: > http://www.scdheft.com > ______________________________________________________________________ > > Dear Dr. Ramon Brugada, > As you have discussed in the answer to the question for an > asymptomatic > patient with brugada syndrome, the use of EPS to risk stratify > asymptomatic patient is very controversial. The first symptom may be > sudden death in such patients. In such a scenario is there any role > for > microvolt T wave alternans for risk stratifications and to decide > the need > for ICD? > > Dr. Sunil Roy. MD, DM > Senior Lecturer in Cardiology > Medical college, Calicut, Kerala, India > Email: sunilroytn at calicutmedicalcollege.ac.in > > > -- > Dr. Sergio Dubner > President of Scientific Committee > > Dr. Edgardo Schapachnik > President of Steering Committee > > > > > _______________________________________________ > Scd-forum mailing list > Scd-forum at scd-symposium.org > http://www.grupoakros.com.ar/mailman/listinfo/scd-forum -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061015/4b916a83/attachment.html From info at scd-symposium.org Sun Oct 15 19:00:52 2006 From: info at scd-symposium.org (SCD Symposium) Date: Sun, 15 Oct 2006 19:00:52 -0300 Subject: [SCD-FORUM] 31E RE: Asymptomatic patient with Brugada syndrome. Dr. Ramon Brugada In-Reply-To: <014619D0-FBD0-4876-99E6-C95467B9088E@scd-symposium.org> References: <014619D0-FBD0-4876-99E6-C95467B9088E@scd-symposium.org> Message-ID: <49B53C31-045B-4873-9DCE-C1EB06852235@scd-symposium.org> No that i know of. There are two studies which looked at TWA (Japan and Germany) in Brugada syndrome. Neither one showed any correlation with risk. Dr. Ramon Brugada MD FACC Associate Professor of Medicine Canadian Research Chair Genetics of Arrhythmias University of Montreal Director Clinical Cardiovascular Genetics Center Montreal Heart Institute 5000 Rue Belanger Montreal, QC H1T 1C8 Canada ramon at brugada.org -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee > > Dear Dr. Ramon Brugada, > As you have discussed in the answer to the question for an > asymptomatic > patient with brugada syndrome, the use of EPS to risk stratify > asymptomatic patient is very controversial. The first symptom may be > sudden death in such patients. In such a scenario is there any role > for > microvolt T wave alternans for risk stratifications and to decide > the need > for ICD? > > Dr. Sunil Roy. MD, DM > Senior Lecturer in Cardiology > Medical college, Calicut, Kerala, India > Email: sunilroytn at calicutmedicalcollege.ac.in > > -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061015/cc6a22d3/attachment.html From info at scd-symposium.org Sun Oct 15 19:10:18 2006 From: info at scd-symposium.org (SCD Symposium) Date: Sun, 15 Oct 2006 19:10:18 -0300 Subject: [SCD-FORUM] 32E RE: A patient with typical vasovagal syncope based on history and type II Brugada pattern. Dr. Ramon Brugada In-Reply-To: References: Message-ID: Dr Alizadeh: This is not an easy decision. You have made a diagnosis of Brugada type I using flecainide. With a syncopal episode, you need to risk stratify the patient. If the syncopal episode is of cardiac origin, pretty much everybody will go ahead and implant a defibrillator (high rate of cardiac arrest at follow-up). If the syncopal episode is vasovagal, then you have again all the options, from follow-up to direct ICD implantation. We recommend EPS to decide on implantation. As previously discussed, EPS is not perfect (but so far it does not appear that there is anything better), and I am sure that neither is HUTT in syncope. According to our experience, we do have cases of typical vasovagal syncope, suspicious ecg, positive HUTT and subsequent cardiac arrest. Genetic testing i do recommend, especially for the implications in family members. thank you Dr. Ramon Brugada MD FACC Associate Professor of Medicine Canadian Research Chair Genetics of Arrhythmias University of Montreal Director Clinical Cardiovascular Genetics Center Montreal Heart Institute 5000 Rue Belanger Montreal, QC H1T 1C8 Canada ramon at brugada.org -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee > > I would like to thank the organizers of this symposium > > My question is about a patient with typical vasovagal syncope > based on history and positive HUTT, negative family history for > SCD, type II Brugada pattern without drug chalenge test and type I > Brugada pattern in ECG after flecainide test. > > Do you recommend further evaluation by electrophysiologic > testing or genetic study? > > what is the first choice for a patient with typical vasovagal > syncope based on history and type II Brugada pattern? flecainide > test or HUTT. > > Best regards > > Hormoz Alizadeh MD > > Department of Pacemaker and Electrophysiology > Rajaie Cardiovascular Medical and Research Center > Tehran, Iran > > > -- > Dr. Sergio Dubner > President of Scientific Committee > > Dr. Edgardo Schapachnik > President of Steering Committee > > > > > _______________________________________________ > Scd-forum mailing list > Scd-forum at scd-symposium.org > http://www.grupoakros.com.ar/mailman/listinfo/scd-forum -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061015/6d422605/attachment.html From info at scd-symposium.org Sun Oct 15 20:18:56 2006 From: info at scd-symposium.org (SCD Symposium) Date: Sun, 15 Oct 2006 20:18:56 -0300 Subject: [SCD-FORUM] 25R RE: Palpitations and SCD risk. Dr. Vorotniak References: <20061015224656.95B3D5299@n8.gigared.com> Message-ID: <0752D6AC-7771-44BB-9E12-E810DAEA8A9A@scd-symposium.org> Dear colleagues, Thank you for the replies to my questions about "palpitations" and Brugada syndrome. The problem of syncopes and "palpitations" is very important, mostly for physicians who work in emergency rooms. In general, when a patient presents with palpitations, we seek in ECG ischemic signs, conduction disorders, or arrhythmias. However, right precordial leads in general are overlooked. Several authors that presented their fascinating lectures in the current Symposium, mention different ECG criteria, predictors of SCD. Some of these criteria are pretty well known and used in daily practice. However, there are others that are not so well known by cardiologists, and even less by general practitioners; for instance: ST segment variations in right precordial leads, S wave morphology and duration in V1-V3, Tpeak-Tend interval, PR shortening, etc. Considering that "palpitations" constitute one of the most frequent reasons for ambulatory consultations, I propose developing a list of ECG predictors of SCD, easy to apply both in emergency rooms and in cardiology offices. Sincerely, Dr. Andriy Vorotniak (Buenos Aires, Argentina) -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061015/7b56c0bd/attachment.html From info at scd-symposium.org Sun Oct 15 23:22:47 2006 From: info at scd-symposium.org (SCD Symposium) Date: Sun, 15 Oct 2006 23:22:47 -0300 Subject: [SCD-FORUM] 33E RE: Young woman with LQTS. Dr. Klingenheben In-Reply-To: <7C68B657-9683-48C7-89B9-C5ECFF4C1519@scd-symposium.org> References: <7C68B657-9683-48C7-89B9-C5ECFF4C1519@scd-symposium.org> Message-ID: Interesting case, I have a similar patient with postpartum VF-arrest. She got an ICD and hat futer TdP-VT documented. Postpartum Cardiac arrest is almost pathognomonic for LQT-2, as are "notched" T waves. My opinion is that this particular patient is at high risk for future events and I clearly would proceed to ICD therapy - irrespective of future pregnancies. I would add a question to Prof. Priori: are there clinical data from the LQT registry concerning arrhythmic events during/after pregnancy in patients with suspected/confirmed LQT ? Thomas Klingenheben > Thomas Klingenheben, MD Assistant Professor of Medicine Kardiologische Gemeinschaftspraxis Alfred-Bucherer-Str. 6 D 53115 Bonn - Germany e-mail: Klingenheben at aol.com e-mail: Klingenheben at em.uni-frankfurt.de Web: www.bonn-kardiologie.de -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee > > First of all I would like to say "thank you" for the nice on-line > symposium > on SCD. > > I would kindly ask Prof. Silvia Priori....worldwide famous expert > on LQTS > > Namely, the one of my patient is a 23-yo woman.....LQTS was > diagnosed 2 > years ago. It was 5 months after first (and the only) partum. She > had lost > of conscious. QT interval in rest ECG - 515 msec, in Holter > monitorng....PVc > -300/day, QT in nighttime till 600 msec, dynamicity in T-wave complex, > postextrasystolic TU wave augmentation. Exercise test induced > momomorphic > PVC (monomorphic bigeminy)......PVC disapeared totally in recovery > phase. > > Rest ECG shows notched T wave in V2-V5 so it looks like LQTS type 2. > > There is no evidence of SCD in her family. We started treatment with > Beta-bloker -metoprolol until dose 3 x 75 mg (this dose is well > tolerated > by her...with no syncope so far). (Nadolol is not available in > Poland now). > Because she plans to have the next pregnancy. My questions are.. > > 1) what should be the proper therapy during pregnancy ? > > 2) how to carry safe the patient during the delivery period ? > > 3) should I increase the dose of beta-bloker in post-partum period ? > > with best regard, > > Dr Piotr Kukla > Specialistic Hospital in Gorlice > Departament of Internal Medicine > Gorlice, Poland -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061015/e1f73a8f/attachment.html From info at scd-symposium.org Sun Oct 15 23:13:02 2006 From: info at scd-symposium.org (SCD Symposium) Date: Sun, 15 Oct 2006 23:13:02 -0300 Subject: [SCD-FORUM] Bibliographical update Message-ID: Dear colleagues, We include instructions to access the bibliography of this last week. We hope this will be of your interest. To access the bibliography http://www.scd-symposium.org/files/2006_10_14.html Kind regards, Dr. Edgardo Schapachnik - Dr. Sergio Dubner -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061015/ff38eff4/attachment.html From info at scd-symposium.org Sun Oct 15 23:28:13 2006 From: info at scd-symposium.org (SCD Symposium) Date: Sun, 15 Oct 2006 23:28:13 -0300 Subject: [SCD-FORUM] 34E RE: Young woman with LQTS. Dr. Budzikowski In-Reply-To: <7C68B657-9683-48C7-89B9-C5ECFF4C1519@scd-symposium.org> References: <7C68B657-9683-48C7-89B9-C5ECFF4C1519@scd-symposium.org> Message-ID: This article in Circulation should provide some answers in managing your patient Circulation. 1998;97:451-456. Adam S. Budzikowski, M.D., Ph.D. Cardiology Unit University of Rochester Medical Center 585-275-4775 http://my.infotriever.com/adamb -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee > > First of all I would like to say "thank you" for the nice on-line > symposium > on SCD. > > I would kindly ask Prof. Silvia Priori....worldwide famous expert > on LQTS > > Namely, the one of my patient is a 23-yo woman.....LQTS was > diagnosed 2 > years ago. It was 5 months after first (and the only) partum. She > had lost > of conscious. QT interval in rest ECG - 515 msec, in Holter > monitorng....PVc > -300/day, QT in nighttime till 600 msec, dynamicity in T-wave complex, > postextrasystolic TU wave augmentation. Exercise test induced > momomorphic > PVC (monomorphic bigeminy)......PVC disapeared totally in recovery > phase. > > Rest ECG shows notched T wave in V2-V5 so it looks like LQTS type 2. > > There is no evidence of SCD in her family. We started treatment with > Beta-bloker -metoprolol until dose 3 x 75 mg (this dose is well > tolerated > by her...with no syncope so far). (Nadolol is not available in > Poland now). > Because she plans to have the next pregnancy. My questions are.. > > 1) what should be the proper therapy during pregnancy ? > > 2) how to carry safe the patient during the delivery period ? > > 3) should I increase the dose of beta-bloker in post-partum period ? > > with best regard, > > Dr Piotr Kukla > Specialistic Hospital in Gorlice > Departament of Internal Medicine > Gorlice, Poland > > > -- > Dr. Sergio Dubner > President of Scientific Committee > > Dr. Edgardo Schapachnik > President of Steering Committee > > > > > _______________________________________________ > Scd-forum mailing list > Scd-forum at scd-symposium.org > http://www.grupoakros.com.ar/mailman/listinfo/scd-forum -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061015/b54a35aa/attachment.html From info at scd-symposium.org Mon Oct 16 07:40:45 2006 From: info at scd-symposium.org (SCD Symposium) Date: Mon, 16 Oct 2006 07:40:45 -0300 Subject: [SCD-FORUM] 35C Two questions about cardiac sudden death Dr. Liu Xin Can Message-ID: <12527313-95B3-474F-BC47-98B3455F8C22@scd-symposium.org> 1. How to evaluate the predictive value of those non-invasive examinations in our clinical work? 2. For those 'Non-Acute Myocardial Infarction' patients, what's the effect of the 'catecholamine storm' on sudden cardiac death? How to evaluate it? "liu.xin.can" -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061016/17cda672/attachment.html From info at scd-symposium.org Mon Oct 16 09:09:36 2006 From: info at scd-symposium.org (SCD Symposium) Date: Mon, 16 Oct 2006 09:09:36 -0300 Subject: [SCD-FORUM] 37E Young woman with HCM? Dr. Asenjo Message-ID: <8A0F216F-031D-447D-9E54-96D3510CA292@scd-symposium.org> I write to you to introduce a clinical case. This patient is a 30 year old woman, with a family history of non obstructive HCM with genetic study that found a mutation in the Troponin T gene (TNT) located in chromosome 1. The mutation is Arg92Gln. Four cousins under 20 have suffered sudden death and one 45 year old aunt has also suffered sudden death. Her mother and 3 other cousins have a CDI because of the family history and evident HCM in Echo. Another aunt and one cousin also have a CDI because syncope and evident HCM. Currently this patient has not presented symptoms and the EKG shows a negative T wave in V1-V3 ( not present years ago), and the previously normal Echo has changed, and now shows a mild enlargement of left atrium and mild mid septum thickening of 12- 13 mm, with no abnormal aspect and no obstruction. MRI is similar to the Echo, and shows normal aspect of septum but thickness is 13mm. No arrhythmias during 24 hrs Holter nor exercise testing. She is on atenolol but we would like to have your input in this case on whether this justifies a CDI now. Besides, we would like to know your opinion about young members of this family. There are children and young persons with apparently normal Echo but they have one or two brothers with sudden death or syncope. ?Do you think they also need a CDI? Finally, what about the adult and older members of this family? Probably most have an evident HCM in Echo. Would it be enough with atenolol or with amiodarore alone, or do they also need a CDI?. We appreciate and thank you for your time and suggestions. Most Sincerely Rene Asenjo -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061016/1aeb142b/attachment.html From info at scd-symposium.org Mon Oct 16 09:03:20 2006 From: info at scd-symposium.org (SCD Symposium) Date: Mon, 16 Oct 2006 09:03:20 -0300 Subject: [SCD-FORUM] 36E RE: A female with burned-out phase of HCM? Dr. Furlani In-Reply-To: References: Message-ID: Dear Dr Alizadeh: What an interesting case! A difficult one too. It sounds like familial dilated cardiomyopathy versus final dilated phase of hypertrophic cardiomyopathy, but I would like to have more information. What exactly is her LV ejection fraction? Were there any LV wall motion abnormalities in her Echo? Has she ever had typical chest pain? What about her cholesterol levels? (a familial dyslipemia could explain an early onset of ischaemic heart disease in this patient). Does she have a history of hypertension? I mean, even though LV hypertrophy secondary to hypertension is usually concentric, sometimes severe hypertension could lead to asymetric LV hypertrophy. Assuming there is no any significant left sided valvular disease, the presence of severe RV dysfunction and moderate to severe pulmonary hypertension (PAPS 65-70 mm Hg) implies long standing severe LV dysfunction in this patient and usually poor prognosis (and lack of response to CRT). Moreover, if this patient has severe mitral regurgitation secondary to severe LV dilatation CRT is unlikely to benefit her. Regarding her medical treatment, I assume she is on low dose diuretics and ACEI but she is off beta blockers and anti-aldosterone drugs. After pacemaker implantation, an optimize medical treatment including appropriate dose of ACEI, beta-blockers and anti- aldosterone drugs will probably improve her LVsystolic function rendering unnecessary ICD/CRT therapy. Finally, There is a clear indication for permanent pacemaker implantation (complete AV block with a wide QRS complex escape suggesting infra-hisian block). RV apical pacing will induce, LV dyssynchony that, in turn, will probably lead to worsening of both LV systolic function and CHF symptoms, but high septal RV pacing is less likely to cause severe LV dyssynchrony. In summary, If her LVEF is over 30% I will go for a DDD pacemaker with an active fixation lead in the high RV septum plus optimization of medical treatment (ACEI, BB, anti-aldost). However, if her LVEF is clearly below 30% (by nuclear ventriculography ideally) I will implant either a CRT-ICD in the case of lack of severe mitral regurgitation or DDD-ICD (usually as a bridge to cardiac transplantation) when severe MR is present. The lack of response to CRT will make this young patient a good candidate to cardiac transplantation. Thank you all. Congratulations to the organizers. Aldo Alberto Furlani MD Cardiac Electrophysilogist Consultant Cardiologist Heart Institute of the Caribbean afurlani at caribbeanheart.com www.caribbeanheart.com -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee El 12/10/2006, a las 23:12, SCD Symposium escribi?: > Forum of the ISHNE Sudden Cardiac Death World-Wide Internet Symposium > ______________________________________________________________________ > > Medtronic's free physician resource center: > http://www.medtronicconnect.com > ______________________________________________________________________ > > Dear Dr Dubner > > thank you for this excellent educational site > > A 38 year old femal referred to our center with new onset dyspnea > from a few month ago. She had family history of sudden cardia > death. She had CHB with wide ventricular escape rhythm. > TT.echocardiography revealed sever LV and RV systolic dysfunction, > asymetric septal hyperthrophy, pulmonary hypertention > (PAP=65-70mmhg) and without LVOT gradient.patient,s dyspnea > improved with low dose diuretics , ACE inhibitor and TPM implantation. > Do you recommend DDD pacemaker for this patient? > or > Do you recommend ICD-DR or ICD-CRT? > > what is your opinion about clinical course of her disease? > > does she need further workup before device implantation? > > sincerely yours > > Hormoz Alizadeh MD > Department of Pacemaker and Electrophysiology > Rajaie Cardiovascular Medical and Research Center > Tehran, Iran > > -- > Dr. Sergio Dubner > President of Scientific Committee > > Dr. Edgardo Schapachnik > President of Steering Committee > > > > > _______________________________________________ > Scd-forum mailing list > Scd-forum at scd-symposium.org > http://www.grupoakros.com.ar/mailman/listinfo/scd-forum -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061016/7407b25f/attachment.html From info at scd-symposium.org Mon Oct 16 09:33:04 2006 From: info at scd-symposium.org (SCD Symposium) Date: Mon, 16 Oct 2006 09:33:04 -0300 Subject: [SCD-FORUM] 38E SV arrhythmias in patients with ARVD. Dr. Fontaine Message-ID: The incidence of SV arrhythmias in patients with obvious ARVD has been reported by our group in 1991. It was observed in 25 % of our cohort of retrospective patients. Since then we have observed that these arrhythmias can be the first presenting symptom of patients with ARVD Some of these patients have no ventricular dilatation and no tricuspid regurgitation. Therefore it is possible to suggest that the disease may have some extension on the atrium. Histology of RV is not clear because it contains physiologically fat and fibrosis. We have not studied yet alteration of gap junctions in atrial muscle in ARVD. Atrial arrhythmias is also a cause of inappropriate shocks in ARVD patients with the device especially those with fast AV conduction (Wenkeback above 200 bpm). I have suggested not to miss a complete EPS evaluating AV conduction and atrial vulnerability Am J Cardiol. 1991 May 15;67(13):1153. Frequency of supraventricular tachyarrhythmias in arrhythmogenic right ventricular dysplasia. Tonet JL, Castro-Miranda R, Iwa T, Poulain F, Frank R, Fontaine GH. Service de Rytmologie et de Stimulation Cardiaque, Hopital Jean Rostand, Ivry, France. Guy Hugues Fontaine -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061016/c9b351ef/attachment.html From info at scd-symposium.org Mon Oct 16 09:47:36 2006 From: info at scd-symposium.org (SCD Symposium) Date: Mon, 16 Oct 2006 09:47:36 -0300 Subject: [SCD-FORUM] 40E RE: Trimetazidine. Dr. Perez Riera In-Reply-To: <92EAACAE-74EA-478D-845D-381C2EC6110E@scd-symposium.org> References: <92EAACAE-74EA-478D-845D-381C2EC6110E@scd-symposium.org> Message-ID: Dear Dr Salim Ben Khedda from Cardiology division Mustapha Hospital medical Center Algeirs Algeria: Here Andr?s Ricardo P?rez Riera from Sao Paulo Brazil. They are several randomized papers mainly from Italy (Clinical Cardiology-Heart Failure Unit, Istituto Scientifico-Universita Vita/ Salute San Raffaele,Milan ) but not related directly to mortality. Please see bellow Main indexed Trimetazidine references with randomized double-blind methodology Fragasso G, Palloshi A, Puccetti P, et al. A randomized clinical trial of trimetazidine, a partial free fatty acid oxidation inhibitor, in patients with heart failure. J Am Coll Cardiol. 2006;48 (5):992-8. Clinical Cardiology-Heart Failure Unit, Istituto Scientifico- Universita Vita/Salute San Raffaele, Milan, Italy. gabriele.fragasso at hsr.it OBJECTIVES: This study sought to assess whether the long-term addition of trimetazidine to conventional treatment could improve functional class, exercise tolerance, and left ventricular function in patients with heart failure (HF). BACKGROUND: Previous small studies have shown that trimetazidine may be beneficial in terms of left ventricular function preservation and control of symptoms in patients with post-ischemic HF. METHODS: Fifty-five patients with HF were randomly allocated in an open-label fashion to either conventional therapy plus trimetazidine (20 mg three times daily) (28 patients) or conventional therapy alone (27 patients). Mean follow-up was 13 +/- 3 months. At study entry and at follow-up, all patients underwent exercise testing and two-dimensional echocardiography. Among the others, New York Heart Association (NYHA) functional class and ejection fraction (EF) were evaluated. RESULTS: In the trimetazidine group, NYHA functional class significantly improved compared with the conventional therapy group (p < 0.0001). Treatment with trimetazidine significantly decreased left ventricular end- systolic volume (from 98 +/- 36 ml to 81 +/- 27 ml, p = 0.04) and increased EF from 36 +/- 7% to 43 +/- 10% (p = 0.002). On the contrary, in the conventional therapy group, both left ventricular end-diastolic and -systolic volumes increased from 142 +/- 43 ml to 156 +/- 63 ml, p = 0.2, and from 86 +/- 34 ml to 104 +/- 52 ml, p = 0.1, respectively; accordingly, EF significantly decreased from 38 +/- 7% to 34 +/- 7% (p = 0.02). CONCLUSIONS: In conclusion, long-term trimetazidine improves functional class and left ventricular function in patients with HF. This benefit contrasts with the natural history of the disease, as shown by the decrease of EF in patients on standard HF therapy alone. PMID: 16949492 [PubMed - indexed for MEDLINE] Fragasso G, Montano C, Perseghin G, Palloshi A, et al. The anti- ischemic effect of trimetazidine in patients with postprandial myocardial ischemia is unrelated to meal composition. Am Heart J. 2006;151:1238.e1-8. Heart Failure Unit, Clinical Cardiology, Istituto Scientifico/ Universita' San Raffaele, Milan, Italy. gabriele.fragasso at hsr.it BACKGROUND: Previous studies provide evidence for a significant reduction of coronary flow reserve after ingestion of meals of different compositions. A possible role of hyperinsulinemia and increased free fatty acid levels, which are deleterious during acute myocardial ischemia and reperfusion, has been hypothesized. We assessed in patients with stable coronary disease the effects of high- fat meals (HFMs) and high-carbohydrate meals (HCMs) on ischemic threshold and stress left ventricular function on placebo and after partial fatty acid inhibition by trimetazidine (TMZ). METHODS: Ten patients (9 men, age 68 +/- 7 years) were allocated to placebo and TMZ (40 mg TID), both administered in the 24 hours preceding testing, according to a randomized double-blind study design. All patients underwent stress (treadmill exercise testing according to the Bruce protocol) echocardiography after fasting (8 hours) and after an HFM and HCM (2 hours) either on placebo or on TMZ. Time to 1-mm ST- segment depression (time to 1 mm) and stress wall motion score index (WMSI) were evaluated. RESULTS: An HFM did not affect exercise variables compared with fasting, whereas an HCM resulted in a reduction of the ischemic threshold (time to 1 mm from 402 +/- 141 to 292 +/- 123 seconds, P = .025). Compared with placebo, TMZ improved time to 1 mm after fasting, HFM, and HCM (432 +/- 153 vs 402 +/- 141, 439 +/- 118 vs 380 +/- 107, 377 +/- 123 vs 292 +/- 123, F(1,9) = 26.91, P = .0006). Compared with placebo, on TMZ, stress WMSI decreased from 1.55 +/- 0.25 to 1.29 +/- 0.14 after fasting, from 1.57 +/- 0.10 to 1.39 +/- 0.28 after HFM, and from 1.64 +/- 0.21 to 1.39 +/- 0.21 after HCM (F(1,9) = 37.04, P = .0002). Interestingly, stress WMSI on TMZ was never different from rest WMSI on placebo. CONCLUSIONS: In patients with coronary disease, exercise testing after an HCM results in more severe myocardial ischemia compared with that after an HFM. The observed beneficial effects of the partial fatty acid inhibitor TMZ seem to be unrelated to meal composition and are possibly caused by the better glucose use induced by the drug. PMID: 16781225 [PubMed - indexed for MEDLINE] Fragasso G, Perseghin G, De Cobelli F, et al.Effects of metabolic modulation by trimetazidine on left ventricular function and phosphocreatine/adenosine triphosphate ratio in patients with heart failure. Eur Heart J. 2006;27:942-948. Department of Clinical Cardiology, Heart Failure Clinic, Istituto Scientifico/Universita' San Raffaele, Via Olgettina 60, 20132 Milano, Italy. gabriele.fragasso at hsr.it AIMS: The addition of trimetazidine to standard treatment has been shown to improve left ventricular (LV) function in patients with heart failure. The aim of this study is to non-invasively assess, by means of in vivo 31P-magnetic resonance spectroscopy (31P-MRS), the effects of trimetazidine on LV cardiac phosphocreatine and adenosine triphosphate (PCr/ATP) ratio in patients with heart failure. METHODS AND RESULTS: Twelve heart failure patients were randomized in a double-blind, cross-over study to placebo or trimetazidine (20 mg t.i.d.) for two periods of 90 days. At the end of each period, all patients underwent exercise testing, 2D echocardiography, and MRS. New York Heart Association (NYHA) class, ejection fraction (EF), maximal rate-pressure product, and metabolic equivalent system (METS) were evaluated. Relative concentrations of PCr and ATP were determined by cardiac 31P-MRS. On trimetazidine, NYHA class decreased from 3.04+/-0.26 to 2.45+/-0.52 (P = 0.005), whereas EF (34+/-10 vs. 39+/-10%, P = 0.03) and METS (from 7.44+/-1.84 to 8.78+/-2.72, P = 0.03) increased. The mean cardiac PCr/ATP ratio was 1.35+/-0.33 with placebo, but was increased by 33% to 1.80+/-0.50 (P = 0.03) with trimetazidine. CONCLUSION: Trimetazidine improves functional class and LV function in patients with heart failure. These effects are associated to the observed trimetazidine-induced increase in the PCr/ ATP ratio, indicating preservation of the myocardial high-energy phosphate levels. PMID: 16510466 [PubMed - indexed for MEDLINE Iskesen I, Saribulbul O, Cerrahoglu M,et al. Trimetazidine reduces oxidative stress in cardiac surgery. Circ J. 2006 Sep;70(9):1169-73. Department of Cardiovascular Surgery, Celal Bayar University School of Medicine, Turkey. iskesen at yahoo.com BACKGROUND: Trimetazidine is an anti-ischemic agent that is used to treat angina and it has cardioprotective effects without inducing any significant hemodynamic changes. It inhibits the long-chain mitochondrial 3-ketoacyl coenzyme A thiolase enzyme in the myocyte and can improve cardiac mitochondrial metabolism, as well as scavenge free radicals. The aim of this double-blind prospective randomized study was to investigate the effect of preoperative use of trimetazidine on the reduction of oxidative stress during coronary artery bypass grafting (CABG) under cardiopulmonary bypass (CPB). METHODS AND RESULTS: The study group (group T) and the control group (group C) each comprised 12 patients. Pretreatment began 2 weeks before CABG with trimetazidine (60 mg/day po); the control group did not receive any medication. Serial blood samples were collected before and after CPB for measurement of the serum concentrations of these major endogenous antioxidant enzyme systems, which are markers for oxidative degradation of the cellular membranes; postoperative levels were significantly different between the groups (p<0.05). There were no significant difference in hemodynamic values. CONCLUSION: The findings suggest that pretreatment with trimetazidine alleviates malondialdehyde production and preserves endogenous antioxidant capacity during CABG with CPB and cardioplegic arrest. PMID: 16936431 [PubMed - indexed for MEDLINE] Chaloupka V. Trimetazidine in the treatment of stable angina pectoris TRIADA-(trimetazidine in stable angina twice daily) Vnitr Lek. 2006 Jun;52(6):609-14. [Article in Czech] Interni kardiologicka klinika Lekarske fakulty MU a FN Brno. vchaloup at fnbrno.cz Links Comment in: Vnitr Lek. 2006 Jun;52(6):556-7. Vnitr Lek. 2006 Jun;52(6):558-9. The primary objective of the national study TRIADA was to evaluate the efficacy and tolerability of Preductal MR (trimetazidine) at a dose of 35 mg twice daily which was added to current therapy involving the maximum of two antianginal drugs. The outcome was evaluated after 12 weeks of therapy and compared with baseline data. The study included 74 patients with stable exertional angina pectoris (AP) and positive exercise testing results. Trimetazidine (Preductal MR) at a dose of 35 mg twice daily was added to their current therapy involving two drugs at most. TRIADA confirmed that the use of trimetazidine in a new pharmacological form is effective and well tolerated in the treatment of angina pectoris. The study also confirmed a beneficial effect of trimetazidine on the incidence of angina pectoris paroxysms and objective manifestations of ischaemia during exercise testing. Holter monitoring clearly showed that metabolic therapy added to standard antianginal therapy would reduce the incidence of symptomatic and asymptomatic ischaemia. In addition, 12-week therapy with trimetazidine helped improve all end points of quality of life of AP patients evaluated using a questionnaire for AP patients (The Seattle Angina Questionnaire). PMID: 16871765 [PubMed - indexed for MEDLINE] Topal E, Ozdemir R, Barutcu I, et al. The effects of trimetazidine on heart rate variability in patients with slow coronary artery flow. J Electrocardiol. 2006 Apr;39(2):211-8. Cardiology Department, Faculty of Medicine, Inonu University, Malatya 34300, Turkey. OBJECTIVE: We sought to examine the effect of trimetazidine (TMZ) on heart rate variability (HRV), endothelin-1 (ET-1), NO, and anginal symptoms in patients with slow coronary artery flow (SCAF). METHODS: The 48 patients with SCAF (29 women and 19 men; mean age, 52 +/- 9 years) were included in the study. Twenty milligrams TMZ 3 times a day or matched placebo were given randomly in a double-blinded fashion for 4 weeks. Patients were divided into 4 groups as follows: exercise-positive, TMZ-given group (group A, n = 12); exercise- positive, placebo-given group (group B, n = 12); exercise-negative, TMZ-given group (group C, n = 12); and exercise-negative, placebo- given group (group D, n = 12). RESULTS: After TMZ treatment, HRV parameters, including SD of the all R-R intervals, SD of the averages of R-R intervals in all 5-minute segments of the entire recording, percentage of R-R intervals with more than 50-millisecond variation, and the square root of the mean of the sum of the squares of differences between adjacent R-R intervals, significantly improved both in exercise-positive and exercise-negative groups when compared with baseline. After TMZ treatment, ET-1 and NO levels significantly altered both in exercise-positive and exercise-negative groups when compared with baseline (17.7 +/- 2.7 vs 13.9 +/- 2.8 pg/mL [P = .01] and 18.1 +/- 3.8 vs 14.2 +/- 2.6 pg/mL [P = .01], respectively). After TMZ treatment, NO levels significantly increased in both exercise-positive and exercise-negative groups when compared with baseline (36.4 +/- 5.4 vs 43.3 +/- 6.8 micromol/L [P = .01] and 36.8 +/- 7.8 vs 43.3 +/- 4.8 micromol/L [P = .01], respectively). However, in placebo group, neither HRV parameters nor ET-1 and NO levels altered when compared with baseline. Also, after treatment, a significant correlation was detected between HRV parameters, including SD of the averages of R-R intervals in all 5-minute segments of the entire recording, SD of the all R-R intervals, percentage of R-R intervals with more than 50-millisecond variation, and the square root of the mean of the sum of the squares of differences between adjacent R-R intervals, and NO and ET-1 levels in TMZ group but not placebo. CONCLUSION: Short-term TMZ therapy improved HRV parameters and endothelial products such as ET-1 and NO as well as anginal symptom in patients with SCAF. Improvement in HRV parameters was correlated with ET-1 and NO levels. PMID: 16580422 [PubMed - indexed for MEDLINE] All the best Andr?s Ricardo P?rez Riera Chief of Electro-Vectocardiology Sector of the Discipline of Cardiology,ABC Faculty of Medicine (FMABC), Foundation of ABC (FUABC)- Santo Andr? -S?o Paulo - Brazil. Rua Sebasti?o Afonso 885 - Zip Code: 044417-100- Jardim Miriam S.P.- Brazil -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee > > Dear colleagues, > Congratulation for this exellent meeting; > I want to ask a question to Dr Andr?s Ricardo P?rez Riera. in term of > evidence based medecine is there any trial showing an improvoment > of morbi > mortality by using Trimetazidine (TMZ) Vartel > Thank you very much for your consideration to this message. > salim BEN KHEDDA > cardiology division > Mustapha Hospital medical Center > Algeirs Algeria > > -- > Dr. Sergio Dubner > President of Scientific Committee > > Dr. Edgardo Schapachnik > President of Steering Committee > > > > > _______________________________________________ > Scd-forum mailing list > Scd-forum at scd-symposium.org > http://www.grupoakros.com.ar/mailman/listinfo/scd-forum -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061016/6983473f/attachment.html From info at scd-symposium.org Mon Oct 16 09:59:56 2006 From: info at scd-symposium.org (SCD Symposium) Date: Mon, 16 Oct 2006 09:59:56 -0300 Subject: [SCD-FORUM] 39E RE: Asymptomatic patient with Brugada syndrome. Dr. Li Zhang In-Reply-To: <49B53C31-045B-4873-9DCE-C1EB06852235@scd-symposium.org> References: <014619D0-FBD0-4876-99E6-C95467B9088E@scd-symposium.org> <49B53C31-045B-4873-9DCE-C1EB06852235@scd-symposium.org> Message-ID: <53EA32B5-0499-4E89-9CEC-47C340A81013@scd-symposium.org> Then we should ask oursselves why is so Dr. Ramon Brugada. Without a clear understanding of the disease nature, we may be always in the dark in terms of risk stratification. If Brugada syndrome were a primary repolarization disorder, one would expect the repolarization instability such as microvolt T wave alternans prior to the onset of VT/VF. As a matter of fact, the majority of VT/VF patients showed positive late potentials ( Arch Cardiol Mex. 2006 Jan-Mar;76(1): 52-8 ). Recently Dr. Shimizu's group demonstrated both depolarization and repolarization abnormalities [ Pacing Clin Electrophysiol. 2006 Oct;29(10):1112-21]. In the past our main focus was the consequence of reduced INa to the phase 1 repolarization in the RV epicardium and the transmural dispersion of repolarization, which may be part of the story. The slow activation propagation in ROVT region due to reduced sodium current itself should not be overlooked Am J Physiol Heart Circ Physiol. 2006 Jul 28; [Epub ahead of print] . Channelopathies especially SCN5A mutations can lead to structural abnormalities although many are undetectable by routine work up. Those structural alterations (macroscopic or microscopic) could also be the substrate to re-entrant ventricular tachyarrhythmias and sudden VT/VF death. Sincerely, Li Zhang, MD LDS Hospital, University of Utah School of Medicine Salt Lake City, UT USA > > No that i know of. There are two studies which looked at TWA (Japan > and > Germany) in Brugada syndrome. Neither one showed any correlation > with risk. > > Dr. Ramon Brugada MD FACC > Associate Professor of Medicine > Canadian Research Chair Genetics of Arrhythmias > University of Montreal > Director Clinical Cardiovascular Genetics Center > Montreal Heart Institute > 5000 Rue Belanger > Montreal, QC H1T 1C8 > Canada > ramon at brugada.org > > -- > Dr. Sergio Dubner > President of Scientific Committee > > Dr. Edgardo Schapachnik > President of Steering Committee > >> >> Dear Dr. Ramon Brugada, >> As you have discussed in the answer to the question for an >> asymptomatic >> patient with brugada syndrome, the use of EPS to risk stratify >> asymptomatic patient is very controversial. The first symptom may be >> sudden death in such patients. In such a scenario is there any >> role for >> microvolt T wave alternans for risk stratifications and to decide >> the need >> for ICD? >> >> Dr. Sunil Roy. MD, DM >> Senior Lecturer in Cardiology >> Medical college, Calicut, Kerala, India >> Email: sunilroytn at calicutmedicalcollege.ac.in >> >> > > -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061016/dcb1ef7e/attachment.html From info at scd-symposium.org Mon Oct 16 10:49:05 2006 From: info at scd-symposium.org (SCD Symposium) Date: Mon, 16 Oct 2006 10:49:05 -0300 Subject: [SCD-FORUM] EXPERTS ASK, EXPERTS ANSWER Message-ID: Dr. Teruhisa Tanabe from Japan asks - Sudden cardiac death is most serious in patients with Brugada syndrome and the number one concern. However, there are differences in incidence of Brugada syndrome between race and country or regions? Why do you think this occurs? Dr. Andrea Sarkozy and Dr. Pedro Brugada from Belgium answer - Dear Dr Tanabe Thank you for your actual and excellent question. The intriguing differences between the incidence and perhaps other characteristics of Brugada syndrome between the different geographical regions have been supported by the strong clinical evidence; 1, Population studies revealed that the incidence of the diagnostic coved Brugada ECG pattern in the general asymptomatic Asian (Japanese) population is much more frequent than in the Caucasian population (0.1-0.4% vs. 0-0.1%). Similarly, sudden death due to Brugada syndrome is also much more frequent in the south East Asian population, then in the Caucasian; the syndrome is the leading natural cause of death in young Thai men (1). 2, Sudden Unexplained Death Syndrome (SUNDS) , first described in US immigrants, is a disorder that had been prevalent for many years in south-east Asia, particularly Thailand, Japan and the Phillipines. SUNDS is characterized by sudden unexpected death at night in apparently healthy men. 60% of the patients have the characteristics Brugada ECG pattern on the baseline ECG (2). Recently, SCN5A mutations have been identified in 3 of 10 patients with SUNDS. The gene mutation resulted in similar ?loss-of-function? channel function alterations as in Brugada syndrome. This data suggest that SUNDS and Brugada syndrome are phenotypically, genetically and functionally the same disorder (3). However, there are differences between the Brugada syndrome in the southeast Asian (SUNDS) and Caucasian patient populations; the man: female ratio is much higher in the Asian patient population (8:1 in SUNDS vs. 3:1 in the 3 European registries); the Asian patients die almost exclusively during sleep while the Caucasian patients sometimes die suddenly daytime. Evidence based explanation is missing to account for these differences, however some recent data might allow some speculations; 1, In 2002, Splawski et al provided evidence that single nucleotide common polymorphisms of the SCN5A gene can influence arrhythmia susceptibility (6). About 13.2% of African Americans carried this allele. The allele had a subtle effect on arrhythmia risk due to subclinical sodium channel function modification. It was proposed that in the presence of additional acquired risk factors, such as medications, hypokalemia and structural heart disease, the individuals with the allele have increased risk of arrhythmia. 2, Ackerman et al in 2004 described in 829 healthy subjects altogether 39 distinct missense variants of the SCN5A coding region, including the previously described known 4 common single nucleotid polymorphisms (SNP). Interestingly, 2 of the 8 most frequent polymorphisms (allelic frequency >0.5%) showed a largely ethnic specific distribution; the R1193Q single nucleotide polymorphism occurred in 8% of the Asian vs. 0.3% of the white population (and was entirely missing in the Hispanic and black population), in contrast to the H558R polymorphism which occurred in 20% of the white (29% of the black and 23% of the Hispanic population) vs. in 9% of the Asian population ( (4). 3, Recently, Bezzina et al described a similar ethnic specific distribution in SNP distributions but in the SCN5A promoter region. A certain combination of 6 single nucleotid polymorphisms (designated as haplotype B variant) only occurred in Asian subjects (at an allele frequency of 22%) and was absent in the other ethnic groups. This haplotype variant resulted in decreased sodium channel expression and function. Although it should be underlined that this haplotype variant neither caused Brugada phenotype nor was more frequent in the Brugada syndrome population, it clearly influenced conduction velocities and was responsible for longer PR and QRS intervals (5). Additionally, in the last years several case reports proved that certain SCN5A polymorphism in the presence of a Brugada syndrome causing SCN5A mutation can influence the clinical phenotype and thus clinical consequences of the mutation; the polymorphism can rescue and restore or in contrast can further worsen the sodium channel function. Putting these pieces of evidence together in one picture, it is possible that the currently best theory to answer your question is the expansion of the multi-hit theory in long QT syndrome described by Keating et al (7). The Asian population, as compared to the Caucasian population, might have a different genetic background consisting of ethnic specific SCN5A (or other ion channel function influencing genes) polymorphisms. These polymorphisms influence sodium channel function and/or expression, but only in a subclinical manner; ?decreasing the antifibrillatory reserves? in a large normally asymptomatic population. However, in these individuals, in the setting of either another mutation or similar function decreasing polymorphism (on the SCN5A or other ion channel genes) or sodium channel blocking agents or other environmental factors (gender, fever etc), the ion channel function is much more easily depressed under the critical level to cause transient action potential, and subsequent ECG abnormalities, provoking clinical arrhythmias. (1) Antzelevitch C et al: Brugada syndrome. Report of the second consensus conference Circ 2005;111:659-70 (2) Nademanee K et al: Arrhythmogenic marker for the sudden unexplained death syndrome in Thai men Circ 1997;96:2595-600 (3) Watta M et al: Genetic and biophysical basis of sudden unexplained nocturnal death syndrome (SUNDS), a disease allelic to Brugada syndrome Hum Mol Gen 2002;11:337-45 (4) Ackerman MJ et al: Spectrum and prevalence of cardiac sodium channel variants among black, white, Asian and Hispanic individuals: Implications for arrhythmogenic susceptibility and Brugada/long QT syndrome genetic testing Heart Rhythm 2004;1:600-7 (5) Bezzina CR et al: Common sodium channel promoter haplotype in Asian subjects underlies variability in cardiac conduction Circ 2006;113:338-44 (6) Splawski I et al: Variant of SCN5A sodium channel implicated in risk of cardiac arrhythmia Science 2002;297:1333-6 (7) Keating MT, Sanguinetti MC: Molecular and cellular mechanisms of cardiac arrhythmias Cell 2001;104:569-80 -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061016/72129dbf/attachment.html From info at scd-symposium.org Mon Oct 16 11:01:27 2006 From: info at scd-symposium.org (SCD Symposium) Date: Mon, 16 Oct 2006 11:01:27 -0300 Subject: [SCD-FORUM] 41E RE: Palpitations and SCD risk. Dr. Perez Riera In-Reply-To: <0752D6AC-7771-44BB-9E12-E810DAEA8A9A@scd-symposium.org> References: <20061015224656.95B3D5299@n8.gigared.com> <0752D6AC-7771-44BB-9E12-E810DAEA8A9A@scd-symposium.org> Message-ID: <97536AA2-ACE5-4EDF-9C74-9C82A4443BC2@scd-symposium.org> Dear Dr. Andriy Vorotniak from Buenos Aires, Argentina , here Andr?s Ricardo P?rez Riera from Sao Paulo Brazil. Good idea let?s go begin! About this subject I recommend the magnificent paper of Dr Shlomo Stern: ?Electrocardiogram Still the Cardiologist?s Best Friend". Circulation 2006 113: 753-756 If you have not access to full text (or other colleage) I can send to you. My e-mail is riera at uol.com.br All the best Andr?s Ricardo P?rez Riera Chief of Electro-Vectocardiology Sector of the Discipline of Cardiology, ABC Faculty of Medicine (FMABC), Foundation of ABC (FUABC)- Santo Andr? -Sao Paulo - Brazil. Rua Sebasti?o Afonso 885 - Zip Code: 044417-100- Jardim Miriam S.P.- Brazil > Dear colleagues, > > Thank you for the replies to my questions about "palpitations" and > Brugada syndrome. > The problem of syncopes and "palpitations" is very important, > mostly for physicians who work in emergency rooms. In general, when > a patient presents with palpitations, we seek in ECG ischemic > signs, conduction disorders, or arrhythmias. However, right > precordial leads in general are overlooked. > Several authors that presented their fascinating lectures in the > current Symposium, mention different ECG criteria, predictors of > SCD. Some of these criteria are pretty well known and used in daily > practice. However, there are others that are not so well known by > cardiologists, and even less by general practitioners; for > instance: ST segment variations in right precordial leads, S wave > morphology and duration in V1-V3, Tpeak-Tend interval, PR > shortening, etc. > Considering that "palpitations" constitute one of the most frequent > reasons for ambulatory consultations, I propose developing a list > of ECG predictors of SCD, easy to apply both in emergency rooms and > in cardiology offices. > > Sincerely, > Dr. Andriy Vorotniak (Buenos Aires, Argentina) > > -- > Dr. Sergio Dubner > President of Scientific Committee > > Dr. Edgardo Schapachnik > President of Steering Committee > -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061016/5067cb36/attachment.html From info at scd-symposium.org Mon Oct 16 15:14:16 2006 From: info at scd-symposium.org (SCD Symposium) Date: Mon, 16 Oct 2006 15:14:16 -0300 Subject: [SCD-FORUM] 42E Brugada patient. Can he work? Dr. Ramirez Message-ID: Congratulations and more power to the organizers of this excellent symposium. I would like to ask your expert opinion about a recent patient I encountered: a male in his mid 30s with type 1 brugada ecg pattern, negative EPS for inducible VT and no family history of sudden death. The said patient is applying as a seaman (works as a ship crew member). Can this patient be cleared for employment in such occupation without risks? MARCELLUS FRANCIS RAMIREZ Universty of Santo Tomas Manila, Philippines -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061016/5faf0324/attachment.html From info at scd-symposium.org Mon Oct 16 16:07:08 2006 From: info at scd-symposium.org (SCD Symposium) Date: Mon, 16 Oct 2006 16:07:08 -0300 Subject: [SCD-FORUM] 43E RE: Young woman with HCM? Dr. Haghjoo In-Reply-To: <8A0F216F-031D-447D-9E54-96D3510CA292@scd-symposium.org> References: <8A0F216F-031D-447D-9E54-96D3510CA292@scd-symposium.org> Message-ID: Dear Dr. Asenjo, In view of strong family history of SCD and high risk mutation of Arg92Gln on TnT, I recommend ICD for this patient. Regarding the young members of this family who have normal Echo, I think it is rational to observe closely by periodic Echo and Holter monitoring. Other old members of this family with evident HCM also need ICD. Best regards, Majid Haghjoo,MD Department of Pacemaker and Electrophysiology Rajaie Cardiovascular Medical and Research Center Tehran, Iran > > I write to you to introduce a clinical case. This patient is a 30 > year old woman, with a family history of non obstructive HCM with > genetic study that found a mutation in the Troponin T gene (TNT) > located in chromosome 1. The mutation is Arg92Gln. Four cousins > under 20 have suffered sudden death and one 45 year old aunt has > also suffered sudden death. Her mother and 3 other cousins have a > CDI because of the family history and evident HCM in Echo. Another > aunt and one cousin also have a CDI because syncope and evident HCM. > > Currently this patient has not presented symptoms and the EKG > shows a negative T wave in V1-V3 ( not present years ago), and the > previously normal Echo has changed, and now shows a mild > enlargement of left atrium and mild mid septum thickening of 12- > 13 mm, with no abnormal aspect and no obstruction. MRI is similar > to the Echo, and shows normal aspect of septum but thickness is > 13mm. No arrhythmias during 24 hrs Holter nor exercise testing. > > She is on atenolol but we would like to have your input in this > case on whether this justifies a CDI now. > > Besides, we would like to know your opinion about young members of > this family. There are children and young persons with apparently > normal Echo but they have one or two brothers with sudden death or > syncope. ?Do you think they also need a CDI? Finally, what about > the adult and older members of this family? Probably most have an > evident HCM in Echo. Would it be enough with atenolol or with > amiodarore alone, or do they also need a CDI?. > > We appreciate and thank you for your time and suggestions. > > Most Sincerely > > Rene Asenjo > > > > -- > Dr. Sergio Dubner > President of Scientific Committee > > Dr. Edgardo Schapachnik > President of Steering Committee > > > > > _______________________________________________ > Scd-forum mailing list > Scd-forum at scd-symposium.org > http://www.grupoakros.com.ar/mailman/listinfo/scd-forum -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061016/fd3613b0/attachment.html From info at scd-symposium.org Mon Oct 16 19:52:05 2006 From: info at scd-symposium.org (SCD Symposium) Date: Mon, 16 Oct 2006 19:52:05 -0300 Subject: [SCD-FORUM] 44E RE: Asymptomatic patient with Brugada syndrome. Dr. Ray Jordan In-Reply-To: <53EA32B5-0499-4E89-9CEC-47C340A81013@scd-symposium.org> References: <014619D0-FBD0-4876-99E6-C95467B9088E@scd-symposium.org> <49B53C31-045B-4873-9DCE-C1EB06852235@scd-symposium.org> <53EA32B5-0499-4E89-9CEC-47C340A81013@scd-symposium.org> Message-ID: Dr. Li, Has genetic research analysis ruled out genetic allele chimerism (at the time of organogenesis and embryological differentiation) to explain the phenotypic variety of channelopathies or numerous clinical manifestations? Another words are some alleles more likely to find or gravitate towards the eighth nerve, RV, vs LV etc., while other regions of pacemaker cells remain entirely normal? Richar-Ray/dba Richard Ray Jordan, M.D., SFM -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee > > Then we should ask oursselves why is so Dr. Ramon Brugada. Without > a clear understanding of the disease nature, we may be always in > the dark in terms of risk stratification. If Brugada syndrome were > a primary repolarization disorder, one would expect the > repolarization instability such as microvolt T wave alternans prior > to the onset of VT/VF. As a matter of fact, the majority of VT/VF > patients showed positive late potentials ( Arch Cardiol Mex. 2006 > Jan-Mar;76(1):52-8 ). Recently Dr. Shimizu's group demonstrated > both depolarization and repolarization abnormalities [ Pacing Clin > Electrophysiol. 2006 Oct;29(10):1112-21]. In the past our main > focus was the consequence of reduced INa to the phase 1 > repolarization in the RV epicardium and the transmural dispersion > of repolarization, which may be part of the story. The slow > activation propagation in ROVT region due to reduced sodium current > itself should not be overlooked Am J Physiol Heart Circ Physiol. > 2006 Jul 28; [Epub ahead of print] > . > Channelopathies especially SCN5A mutations can lead to structural > abnormalities although many are undetectable by routine work up. > Those structural alterations (macroscopic or microscopic) could > also be the substrate to re-entrant ventricular tachyarrhythmias > and sudden VT/VF death. > > Sincerely, > > Li Zhang, MD > LDS Hospital, > University of Utah School of Medicine > Salt Lake City, UT > USA > >> >> No that i know of. There are two studies which looked at TWA >> (Japan and >> Germany) in Brugada syndrome. Neither one showed any correlation >> with risk. >> >> Dr. Ramon Brugada MD FACC >> Associate Professor of Medicine >> Canadian Research Chair Genetics of Arrhythmias >> University of Montreal >> Director Clinical Cardiovascular Genetics Center >> Montreal Heart Institute >> 5000 Rue Belanger >> Montreal, QC H1T 1C8 >> Canada >> ramon at brugada.org >> >> >> >>> >>> Dear Dr. Ramon Brugada, >>> As you have discussed in the answer to the question for an >>> asymptomatic >>> patient with brugada syndrome, the use of EPS to risk stratify >>> asymptomatic patient is very controversial. The first symptom may be >>> sudden death in such patients. In such a scenario is there any >>> role for >>> microvolt T wave alternans for risk stratifications and to decide >>> the need >>> for ICD? >>> >>> Dr. Sunil Roy. MD, DM >>> Senior Lecturer in Cardiology >>> Medical college, Calicut, Kerala, India >>> Email: sunilroytn at calicutmedicalcollege.ac.in >>> >>> -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061016/319daec7/attachment.html From info at scd-symposium.org Mon Oct 16 22:28:35 2006 From: info at scd-symposium.org (SCD Symposium) Date: Mon, 16 Oct 2006 22:28:35 -0300 Subject: [SCD-FORUM] 45C RE: A children with LQTS. Dr. Cuilan Li In-Reply-To: <0DEDFEC2-AB49-418F-A3F9-EDF76853B637@scd-symposium.org> References: <0DEDFEC2-AB49-418F-A3F9-EDF76853B637@scd-symposium.org> Message-ID: Dear Dr. Haghjoo: It?s a true challenge for treating such young LQTS patient (5-y-o), especially, it is difficuilt to increase the dosage of Betablocker while patient has low heart rate. Based our experience ,my suggestion is: 1. To change betablocker from propranolol to Metoprolol extended action tablet or nadolol (depending on what you can get in your country); 2. If the patient continues to have syncope episode during betablocker administration, recommend her to receive the left sympathetic denervation surgery. We had undergo such surgery for a 6 y-o boy 5 years ago and for a 7-y-o girl last Sept. in Peking University People?s Hospital, China. It?s not unconquerable for surgery though it?s more difficult than that for adult patients. Dr. Schwartz?s group has more experiences to deal with patients who need surgery. 3. When her body size is large enough, ICD may be considered depending on her symptom at that time. It?s a wonderful experience to exchange opinions with experts from world wide, and thanks to the organizers, thanks to the participants from so many countries! Cuilan Li, Ph. D Department of Cardiology, Peking University People?s Hosp., Beijing 100044, China Tel: 86-10-68314422 Ext. 5940 licuilan at gmail.com -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee > > Dear Dr. Dubner, > > Thank you and your colleagues for this nice online symposium. > Recently, a > 5-year-old children with no evidence of structural heart disease > referred to > our center for evaluation of syncope. He also had history of > congenital > deafness. One of the episodes of syncope occurred 48-hrs after > betablocker > withdrawal and second episodes on 10 mg propranolol tid. Surface ECG > revealed a QTc=600 ms and heart rate of 55 bpm. We think that onset in > childhood, congenital deafness and QTc=600 would place this patient at > higher risk for cardiac events and therefore a candidate for ICD > implantation. However, small body size and future risk of > inappropriate > therapy with its devastating consequences are unfavorable aspects > of ICD > implantation. Our limitimg factors for increase in dose of > betablocker is > low baseline hear rate. What is recommendation of scientific > committee of > SCD symposium regarding our patient? > > Thank you in advance for your recommendation. > > Regards, > > Majid Haghjoo,MD > Department of Pacemaker and Electrophysiology > Rajaie Cardiovascular Medical and Research Center > Tel: +98 21 2392 2931 > Fax:+98 21 2204 8174 > Tehran, Iran -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061016/fe5fb2dd/attachment.html From info at scd-symposium.org Tue Oct 17 06:23:12 2006 From: info at scd-symposium.org (SCD Symposium) Date: Tue, 17 Oct 2006 06:23:12 -0300 Subject: [SCD-FORUM] 44E RE: Asymptomatic patient with Brugada syndrome. Dr. Ray Jordan In-Reply-To: <53EA32B5-0499-4E89-9CEC-47C340A81013@scd-symposium.org> References: <014619D0-FBD0-4876-99E6-C95467B9088E@scd-symposium.org> <49B53C31-045B-4873-9DCE-C1EB06852235@scd-symposium.org> <53EA32B5-0499-4E89-9CEC-47C340A81013@scd-symposium.org> Message-ID: Dr. Li, Has genetic research analysis ruled out genetic allele chimerism (at the time of organogenesis and embryological differentiation) to explain the phenotypic variety of channelopathies or numerous clinical manifestations? Another words are some alleles more likely to find or gravitate towards the eighth nerve, RV, vs LV etc., while other regions of pacemaker cells remain entirely normal? Richar-Ray/dba Richard Ray Jordan, M.D., SFM -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee > > Then we should ask oursselves why is so Dr. Ramon Brugada. Without > a clear understanding of the disease nature, we may be always in > the dark in terms of risk stratification. If Brugada syndrome were > a primary repolarization disorder, one would expect the > repolarization instability such as microvolt T wave alternans prior > to the onset of VT/VF. As a matter of fact, the majority of VT/VF > patients showed positive late potentials ( Arch Cardiol Mex. 2006 > Jan-Mar;76(1):52-8 ). Recently Dr. Shimizu's group demonstrated > both depolarization and repolarization abnormalities [ Pacing Clin > Electrophysiol. 2006 Oct;29(10):1112-21]. In the past our main > focus was the consequence of reduced INa to the phase 1 > repolarization in the RV epicardium and the transmural dispersion > of repolarization, which may be part of the story. The slow > activation propagation in ROVT region due to reduced sodium current > itself should not be overlooked Am J Physiol Heart Circ Physiol. > 2006 Jul 28; [Epub ahead of print] > . > Channelopathies especially SCN5A mutations can lead to structural > abnormalities although many are undetectable by routine work up. > Those structural alterations (macroscopic or microscopic) could > also be the substrate to re-entrant ventricular tachyarrhythmias > and sudden VT/VF death. > > Sincerely, > > Li Zhang, MD > LDS Hospital, > University of Utah School of Medicine > Salt Lake City, UT > USA > >> >> No that i know of. There are two studies which looked at TWA >> (Japan and >> Germany) in Brugada syndrome. Neither one showed any correlation >> with risk. >> >> Dr. Ramon Brugada MD FACC >> Associate Professor of Medicine >> Canadian Research Chair Genetics of Arrhythmias >> University of Montreal >> Director Clinical Cardiovascular Genetics Center >> Montreal Heart Institute >> 5000 Rue Belanger >> Montreal, QC H1T 1C8 >> Canada >> ramon at brugada.org >> >> >> >>> >>> Dear Dr. Ramon Brugada, >>> As you have discussed in the answer to the question for an >>> asymptomatic >>> patient with brugada syndrome, the use of EPS to risk stratify >>> asymptomatic patient is very controversial. The first symptom may be >>> sudden death in such patients. In such a scenario is there any >>> role for >>> microvolt T wave alternans for risk stratifications and to decide >>> the need >>> for ICD? >>> >>> Dr. Sunil Roy. MD, DM >>> Senior Lecturer in Cardiology >>> Medical college, Calicut, Kerala, India >>> Email: sunilroytn at calicutmedicalcollege.ac.in >>> >>> -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061017/a96d1c89/attachment.html From info at scd-symposium.org Tue Oct 17 07:38:00 2006 From: info at scd-symposium.org (SCD Symposium) Date: Tue, 17 Oct 2006 07:38:00 -0300 Subject: [SCD-FORUM] EXPERTS ASK, EXPERTS ANSWER Message-ID: Dr. Luciano Pereira from Paraguay asks - The Commotio Cordis is an entity increasingly being diagnosed over the last few years. Since it is highly deadly and affects young sportsmen in most cases, cardioverter defibrillators and other reanimation devices availability should not be obligatory in public and private institutions devoted to sports? Dr. Andres Perez Riera from Brazil answers - Dear Luciano: Here Andr?s Ricardo P?rez Riera from S?o Paulo Brazil. Cardiac concussion, commotio cordis, or nonpenetrating chest wall impact (traumatic blow to the chest wall causing VT/VF) is caused by a sudden, nonpenetrating, localized impact to the chest that is theorized to result in almost simultaneous SD from a disruption to the conductive system. Commotio cordis may lead to SCD due to the acute initiation of VF. VF may result from sudden stretch during a vulnerable window, which is determined by repolarization inhomogeneity. The detailed external/internal forensic examination of the body reveals no evidence of structural, pathologic, or histologic signs of trauma to the heart. A cardiac concussion is a rare and often overlooked cause of SD. This type of SD is typically seen among younger individuals participating in sports involving projectiles and, to a lesser degree, where collisions occur. Cardiac concussions are clinically, pathologically, and chemically different from a cardiac contusion1. The main cause?s of SD among young athetes (<35 years old) are: 1) Hypertrophic cardiomyopathy (HCM) is the most common form of SD in young competitive athletes; 2) Arrhythmogenic right ventricular dysplasia (ARVD); 3) Anomalous coronary origins: White-Bland-Garlad syndrome and others; 4) Marfan syndrome; 5) Premature coronary artery disease; 6) Structurally normal heart (ion channel disorders.): SD with a structurally normal heart was the leading cause of death among female young recruits during military training2. These main entities are: congenital LQTS, the SQTS, BrS and CPVT. These are pathologies with very different phenotypes and aetiologies, but which share a common final pathway in causing SD: amplification of spatial dispersion of repolarization in the form of TDR(3). 7) Inflammatory myocardial diseases: Myocarditis 8) Commotio cordis. Analyze the presence of myocardial damage in relation to official boxing matches. Low-energy chest wall impact could be responsible for SCD h, i.e. commotio cordis. As boxing is a traumatic sport in which thoracic hits usually occur, it seems interesting to know if there are any significant cardiac changes during official bouts. Fifteen amateur boxers, participating in the semifinals of the Italian Championship were investigated by Bianco et al. A standard ECG before, immediately after, 1 hour and 12 hours after the match were obtained from each athlete to analyze: 1) Atrio-ventricular conduction; 2) QRS axis and duration; 3) Ventricular repolarization. A blood sample was also obtained before and 12 hours after the match for analysis of total-creatin-phosphokinase, myoglobin, and T- troponin. After the fight, the following significant changes were encountered: 1) Higher QRS voltages; 2) Lowering of J-point and ST segment in lateral leads; 3) Higher ST-slope; 4) Lower T-wave amplitude; 5) Shorter T-wave peak time, and 6) Shorter QT interval. When the last 2 parameters were corrected for heart rate, no differences were observed for QTc, while T-wave peak time significantly increased. All these changes persisted until one hour after the match. Moreover, 3/15 boxers (20 %) showed marked ventricular repolarization anomalies in lateral leads after the contest, persisting for 12 hours in one case. However, no athlete had clinical and humoral signs of myocardial damage following the match. It was concluded that no clinical and humoral signs of myocardial damage were found after amateur boxing matches, although ventricular repolarization abnormalities can be found on ECG in 20 % of boxers, probably due to sympathetic hyper-activity related to the agonistic event(4). References 1) Koehler SA, Shakir A, Ladham S, et. al. Cardiac concussion: definition, differential diagnosis, and cases presentation and the legal ramification of a misdiagnosis. Am J Forensic Med Pathol. 2004;25:205-208. 2) Eckart RE, Scoville SL, Shry EA, Potter RN, Tedrow U. Causes of sudden death in young female military recruits.Am J Cardiol. 2006; 97:1756-1758. 3) Antzelevich C, Oliva A. Amplificaion of spatial dispersion of repolarization underlies sudden cardiac death associated with catecholaminergic J Intern Med. 2006;259:48-58 4) Bianco M, Colella F, Pannozzo A, Oradei A, et al. Boxing and "commotio cordis": ECG and humoral study. Int J Sports Med. 2005;26:151-157. All the for all Andr?s Ricardo P?rez Riera MD Chief of Electro-Vectocardiology Sector of the Discipline of Cardiology, ABC Faculty of Medicine (FMABC), Foundation of ABC (FUABC) ? Santo Andr? ? S?o Paulo ? Brazil. Sebasti?o Afonso 885 Jardim Miriam SP Brazil Zip Code: 04417-100 riera at uol.com.br -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061017/b233f36d/attachment.html From info at scd-symposium.org Tue Oct 17 10:45:25 2006 From: info at scd-symposium.org (SCD Symposium) Date: Tue, 17 Oct 2006 10:45:25 -0300 Subject: [SCD-FORUM] Today's Scientific Activity Message-ID: <229B47B9-031D-4F17-8754-BCB35705CC19@scd-symposium.org> Dear colleagues, LPM RADIO - In the sector corresponding to the LPM Radio, you can see the following webcast Talking on SCD with Douglas P. Zipes and Silvia Priori http://www.scd-symposium.org/lpmradio.php LECTURES As of today, in the Conference Room, the following Presentations are available: Electrocardiographic determinants of risk for Sudden Dead and recurrence in Brugada Syndrome Patients Josep Brugada / Pedro Brugada / Ramon Brugada / Santiago Nava http://www.scd-symposium.org/lectures.php -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061017/64132052/attachment.html From info at scd-symposium.org Tue Oct 17 07:19:08 2006 From: info at scd-symposium.org (SCD Symposium) Date: Tue, 17 Oct 2006 07:19:08 -0300 Subject: [SCD-FORUM] 47E Some questions about Brugada Syndrome. Dr. Kam Message-ID: <08A19966-633A-473A-BB8D-0CC5A3443026@scd-symposium.org> Induction of a type I Brugada pattern during febrile states has been well described. I have also observed a few cases where a Type I Brugada pattern was observed during or shortly after a strong vagal episode, such as severe abdominal pain , or a vasovagal episode, where the individual recovered quite quickly as with a vasovagal syncope, unlike a sudden cardiac arrest situation. The ECG subsequently reverted to normal or a Type II or III pattern on a separate occasion. What is the mechanism of these observations and what is the prognosis? If EPS is performed, how often is VT/VF inducible? I would like to hear the opinion of the experts in this field. Sincerely Dr Ruth Kam Consultant Cardiologist and Cardiac Electrophysiologist Ruth Kam Heart and Arrhythmia Clinic #08-06, Mt Elizabeth Medical Centre Singapore 228510 -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061017/c8b003f8/attachment.html From info at scd-symposium.org Tue Oct 17 14:10:18 2006 From: info at scd-symposium.org (SCD Symposium) Date: Tue, 17 Oct 2006 14:10:18 -0300 Subject: [SCD-FORUM] 49E RE: Asymptomatic patient with Brugada syndrome. Dr. Furlani In-Reply-To: References: <014619D0-FBD0-4876-99E6-C95467B9088E@scd-symposium.org> <49B53C31-045B-4873-9DCE-C1EB06852235@scd-symposium.org> <53EA32B5-0499-4E89-9CEC-47C340A81013@scd-symposium.org> Message-ID: Dear Dr Li: You've got a point there, but another possibility is that TWA test is not as good to detect repolarization problems in the RV as it is in the LV (being BrS a predominantely RV channelopathy).Moreover, maybe the timing of the TWA test was not the most appropriate in both German and Japanese studies cited by Dr Brugada. Why does a patinent with a Type I ECG remains asymptomatic for 5 to 10 years and then die suddenly or have 3 episodes of syncope the same day? TWA test would be abnormal in this patient before experiencing SCD or recurrent syncope? We are just warming up, there is a long way to go. My best regards, Aldo Alberto Furlani MD Cardiac Electrophysiologist Consultant Cardiologist Heart Institute of the Caribbean Mandeville Jamaica afurlani at caribbeanheart.com www.caribbenheart.com -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee > > Dr. Li, > Has genetic research analysis ruled out genetic allele chimerism > (at the > time of organogenesis and embryological differentiation) to explain > the > phenotypic variety of channelopathies or numerous clinical > manifestations? Another > words are some alleles more likely to find or gravitate towards the > eighth > nerve, RV, vs LV etc., while other regions of pacemaker cells > remain entirely > normal? > Richar-Ray/dba Richard Ray Jordan, M.D., SFM > > -- > Dr. Sergio Dubner > President of Scientific Committee > > Dr. Edgardo Schapachnik > President of Steering Committee > >> >> Then we should ask oursselves why is so Dr. Ramon Brugada. Without >> a clear understanding of the disease nature, we may be always in >> the dark in terms of risk stratification. If Brugada syndrome were >> a primary repolarization disorder, one would expect the >> repolarization instability such as microvolt T wave alternans >> prior to the onset of VT/VF. As a matter of fact, the majority of >> VT/VF patients showed positive late potentials ( Arch Cardiol Mex. >> 2006 Jan-Mar;76(1):52-8 ). Recently Dr. Shimizu's group >> demonstrated both depolarization and repolarization abnormalities >> [ Pacing Clin Electrophysiol. 2006 Oct;29(10):1112-21]. In the >> past our main focus was the consequence of reduced INa to the >> phase 1 repolarization in the RV epicardium and the transmural >> dispersion of repolarization, which may be part of the story. The >> slow activation propagation in ROVT region due to reduced sodium >> current itself should not be overlooked Am J Physiol Heart Circ >> Physiol. 2006 Jul 28; [Epub ahead of print] >> . >> Channelopathies especially SCN5A mutations can lead to structural >> abnormalities although many are undetectable by routine work up. >> Those structural alterations (macroscopic or microscopic) could >> also be the substrate to re-entrant ventricular tachyarrhythmias >> and sudden VT/VF death. >> >> Sincerely, >> >> Li Zhang, MD >> LDS Hospital, >> University of Utah School of Medicine >> Salt Lake City, UT >> USA >> >>> >>> No that i know of. There are two studies which looked at TWA >>> (Japan and >>> Germany) in Brugada syndrome. Neither one showed any correlation >>> with risk. >>> >>> Dr. Ramon Brugada MD FACC >>> Associate Professor of Medicine >>> Canadian Research Chair Genetics of Arrhythmias >>> University of Montreal >>> Director Clinical Cardiovascular Genetics Center >>> Montreal Heart Institute >>> 5000 Rue Belanger >>> Montreal, QC H1T 1C8 >>> Canada >>> ramon at brugada.org >>> >>> >>> >>>> >>>> Dear Dr. Ramon Brugada, >>>> As you have discussed in the answer to the question for an >>>> asymptomatic >>>> patient with brugada syndrome, the use of EPS to risk stratify >>>> asymptomatic patient is very controversial. The first symptom >>>> may be >>>> sudden death in such patients. In such a scenario is there any >>>> role for >>>> microvolt T wave alternans for risk stratifications and to >>>> decide the need >>>> for ICD? >>>> >>>> Dr. Sunil Roy. MD, DM >>>> Senior Lecturer in Cardiology >>>> Medical college, Calicut, Kerala, India >>>> Email: sunilroytn at calicutmedicalcollege.ac.in >>>> >>>> >>>> -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061017/ca991b3b/attachment.html From info at scd-symposium.org Tue Oct 17 14:18:03 2006 From: info at scd-symposium.org (SCD Symposium) Date: Tue, 17 Oct 2006 14:18:03 -0300 Subject: [SCD-FORUM] 50E Questions to Dr. Fontaine and Dr. Hammill. Dr. Brembilla Perrot Message-ID: <7E3D789D-45B9-4929-94C2-2151678FE288@scd-symposium.org> Dear Dr Dubner and Dr Schapachnik I have also questions to some authors as Dr Fontaine and Dr Hammill Some things seem very simple: tachycardia or syncope is of benign prognosis if there is no underlying heart disease. The significance completely changes in association with heart disease. However with the development of magnetic resonance imaging (MRI) or similar techniques, many of patients have signs of heart disease not visible at echocardiography as myocardial scar in VT, signs of ARVD in right outflow tract tachycardia, signs of beginning DCM..... A benign VT becomes a tachycardia with a risk of SD?? Is RMI necessary in all patients with VT or syncope and with apparently normal echocardiography? Does abnormal RMI in asymptomatic patient with only VPB's need others investigations, preventive beta-blocker therapy or more?? To my opinion, the cardiology becomes more and more difficult. We have learned many important data in cardiology through small studies of one or several authors; now we learn some data after very large multicentric studies engaged by big companies with the very well known problem of the criteria of exclusion and inclusion. Where is my patient? Pardon my difficulties in the real life and thank you for your recommendation. B?atrice Brembilla-Perrot -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061017/0f440c25/attachment.html From info at scd-symposium.org Tue Oct 17 16:45:43 2006 From: info at scd-symposium.org (SCD Symposium) Date: Tue, 17 Oct 2006 16:45:43 -0300 Subject: [SCD-FORUM] 51E RE: Asymptomatic patient with Brugada syndrome. Dr. Li Zhang In-Reply-To: References: <014619D0-FBD0-4876-99E6-C95467B9088E@scd-symposium.org> <49B53C31-045B-4873-9DCE-C1EB06852235@scd-symposium.org> <53EA32B5-0499-4E89-9CEC-47C340A81013@scd-symposium.org> Message-ID: <5986BDDA-974E-4E26-B6C5-DC81593D1563@scd-symposium.org> Dear Dr. Ray: Thank you so much for contributing such an excellent question to www.scd-symposium.org. I wish experts Drs. Igor Splawski, Silvia Priori, and Michael Ackerman would be able to provide you the answer in satisfactory. With very limited knowledge, my premature response to your question is no. A negative genetic testing result by a standard approach cannot ruled out the possibility of somatic mutation caused diseases. Two years ago, Dr. Splawski, et al identified the disease- causing mutation G406R in Cav1.2 calcium channel gene in most of sporadic cases with Timothy syndrome (LQT8). Two of affected children are siblings, and the DNA extracted from the parents' blood sample do not have the mutation. However, Igor found the mutation in their mother's buccal swab DNA sample (from oral mucosa), suggesting she is a mosaic. It means G406R arose de novo during her early development. (Splawski, et al Cell, 2004;119:1-20). Last night I had a great interest reading Dr. Calum MacRae's commentaries (MacRae, et al J. Clin. 2006, Invest;116:1825-28) regarding an excellent study conducted by Dr. Garcia-Gras, et al. J. Clin. Invest 2006;116:2012-21 (suppression of canonical Wnt/beta- catenin signaling by nuclear plakoglobin recapitulates phenotype of arrhythmogenic right ventricular cardiomyopathy. I wish those information could be helpful to you as well. Sincerely, Li Zhang, MD -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee > > Dr. Li, > Has genetic research analysis ruled out genetic allele chimerism > (at the > time of organogenesis and embryological differentiation) to explain > the > phenotypic variety of channelopathies or numerous clinical > manifestations? Another > words are some alleles more likely to find or gravitate towards the > eighth > nerve, RV, vs LV etc., while other regions of pacemaker cells > remain entirely > normal? > Richar-Ray/dba Richard Ray Jordan, M.D., SFM > >> >> Then we should ask oursselves why is so Dr. Ramon Brugada. Without >> a clear understanding of the disease nature, we may be always in >> the dark in terms of risk stratification. If Brugada syndrome were >> a primary repolarization disorder, one would expect the >> repolarization instability such as microvolt T wave alternans >> prior to the onset of VT/VF. As a matter of fact, the majority of >> VT/VF patients showed positive late potentials ( Arch Cardiol Mex. >> 2006 Jan-Mar;76(1):52-8 ). Recently Dr. Shimizu's group >> demonstrated both depolarization and repolarization abnormalities >> [ Pacing Clin Electrophysiol. 2006 Oct;29(10):1112-21]. In the >> past our main focus was the consequence of reduced INa to the >> phase 1 repolarization in the RV epicardium and the transmural >> dispersion of repolarization, which may be part of the story. The >> slow activation propagation in ROVT region due to reduced sodium >> current itself should not be overlooked Am J Physiol Heart Circ >> Physiol. 2006 Jul 28; [Epub ahead of print] >> . >> Channelopathies especially SCN5A mutations can lead to structural >> abnormalities although many are undetectable by routine work up. >> Those structural alterations (macroscopic or microscopic) could >> also be the substrate to re-entrant ventricular tachyarrhythmias >> and sudden VT/VF death. >> >> Sincerely, >> >> Li Zhang, MD >> LDS Hospital, >> University of Utah School of Medicine >> Salt Lake City, UT >> USA >> >>> >>> No that i know of. There are two studies which looked at TWA >>> (Japan and >>> Germany) in Brugada syndrome. Neither one showed any correlation >>> with risk. >>> >>> Dr. Ramon Brugada MD FACC >>> Associate Professor of Medicine >>> Canadian Research Chair Genetics of Arrhythmias >>> University of Montreal >>> Director Clinical Cardiovascular Genetics Center >>> Montreal Heart Institute >>> 5000 Rue Belanger >>> Montreal, QC H1T 1C8 >>> Canada >>> ramon at brugada.org >>> >>> >>> >>>> >>>> Dear Dr. Ramon Brugada, >>>> As you have discussed in the answer to the question for an >>>> asymptomatic >>>> patient with brugada syndrome, the use of EPS to risk stratify >>>> asymptomatic patient is very controversial. The first symptom >>>> may be >>>> sudden death in such patients. In such a scenario is there any >>>> role for >>>> microvolt T wave alternans for risk stratifications and to >>>> decide the need >>>> for ICD? >>>> >>>> Dr. Sunil Roy. MD, DM >>>> Senior Lecturer in Cardiology >>>> Medical college, Calicut, Kerala, India >>>> Email: sunilroytn at calicutmedicalcollege.ac.in >>>> >>>> > > -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061017/0a42ba96/attachment.html From info at scd-symposium.org Tue Oct 17 17:32:42 2006 From: info at scd-symposium.org (SCD Symposium) Date: Tue, 17 Oct 2006 17:32:42 -0300 Subject: [SCD-FORUM] EXPERTS ASK, EXPERTS ANSWER. Dr. Perez Riera In-Reply-To: References: Message-ID: Dear Teruhisa Tanabe from Japan: here Andr?s Ricardo P?rez Riera from Sao Paulo Brazil. Indeed there are differences in incidence of Brugada syndrome( BrS) among races, countries and regions because the chanelopathy eventually has a genetic component. BrS is genetically determined in a dominant autosomal mode in approximately 20% of the cases and is caused by mutations in the SCN5A gene on chromosome 3p21-23, encoding the cardiac sodium channel (1). Most cardiac channelopathies are inherited as autosomal dominant disorders. This means that any person with an inherited cardiac channelopathy has a 50% chance of passing it on to each of his or her children. The channelopaties that affect the SCN5A gene are allelic with BrS ((Any one of a series of two or more different genes that occupy the same position (locus) on a chromosome.) The majority of these mutations are missense. However, other types such as deletions, insertions, frame shifts, nonsense and splice-donor errors have also been reported.(2) We think that we must divided Brugada entities in (3): 1) Familial cases (?17%): true Brugada disease. .We proposes that this entity should be promoted to the category of disease, since it has a characteristic set of signs and symptoms, and an identified genetic defect. (4); (2) Sporadic cases (?63%): Brugada syndrome (5) (3) Acquired forms: those entities or clinical pharmacological conditions, where the Brugada phenotype or Brugada-type ECG may be found as a consequence of promoting increase in Ito channel function in the ventricular epicardium or decrease in the slow calcium channel. Because the ECG pattern can be dynamic and is often concealed, it is difficult to estimate the true prevalence of the disease in the general population. BrS causes 4 to 10 SCD per 10,000 inhabitants per year in areas like Thailand and Laos (Southeast Asia). It is particularly frequent among young men (under the age of 40) of Asian origin. In these countries, the disease represents the leading cause of death in young adult men (6) BrS deaths are second only to automobile accidents as a cause of death among young adults men in some countries around the world. The annual mortality rate in Thailand for this subgroup the. prevalence of the disease was estimated to be 26 to 38 deaths per 100,000 inhabitants(7) The prevalence, incidence and prognostic value of the Brugada-type ECG has been studied in a general adult Japanese population. The 4,788 subjects (1,956 men and 2,832 women) were under 50 years of age in 1958 and had undergone biannual health examinations, including ECG, through 1999. There were a total of 32 Brugada-type ECG cases. The prevalence and incidence were 146.2 in 100,000 persons and 14.2 persons per 100,000 person-years, respectively. The incidence was nine times higher among men than women, and the average age at presentation was 45 +/- 10.5 years. The Brugada-type ECG appeared intermittently in most cases and was found in 26% of subjects who died unexpectedly. Cox survival analysis revealed that mortality from unexpected death was significantly higher in subjects with a Brugada- type ECG than in control subjects (p < 0.01). Unexpected deaths were more frequent among subjects with the Brugada-type ECG who had a history of syncope (p < 0.05). The Brugada-type ECG is not a very rare condition in the adult Japanese population. Subjects with a Brugada-type ECG have an increased risk of unexpected death (8). The Brugada-type ECG was found in 0.70% of 13,929 study subjects (98 cases) in a community-based population in Japan, predominantly in men. The typical coved-type ST-T morphology with the RBBB pattern-an rsR' pattern in the V1 lead ("typical" Brugada-type 1)-was found in 0.12% of all subjects. The prevalence of cardiac events among male subjects with a Brugada- type ECG (81%) was significantly higher than it was for those without it. The Brugada-type ECG was found in 2.14% of male subjects, and the "typical" Brugada-type 1 was found in 0.38%. After 2.6 +/- 0.3 years of follow-up, there was one death of a subject with the Brugada-type ECG, whereas there were 139 deaths of those without the Brugada-type ECG (p = 0.9943, log-rank test). The total mortality of subjects with the Brugada-type ECG did not differ from the mortality of those without the Brugada-type ECG (9). From about 12,000 non-selected and non-cardiac patients in just one University Hospital, collected prospectively for a period of two years, there were 52 cases with the typical ECG pattern, i.e., with Brugada sign(10). In France have shown a prevalence of Brugada pattern of ECG in 1 per 1,000 normal asymptomatic individuals (11). The prevalence, incidence, and prognosis of the Brugada-type ECG were analyzed in the general population of Japan, based on a study of four decades. Thus, from a group of 4788 individuals, 32 Brugada-type ECGs were detected, the prevalence and incidence being 146.2 in 100,000 people and 14.2 per 100,000 individuals/year, respectively. This incidence was nine times higher in men than in women; the intermittence of the Brugada-type ECG was found in 26% of cases of those who underwent SCD, and the Cox survival analysis showed that unexpected mortality was significantly higher in subjects with the Brugada-type ECG than in control subjects(12). Monroe and Littmann (2000), in 12,000 consecutive ECG in non-cardiac patients, during two years, they found Brugada pattern in 52 ECGs showing a higher prevalence than thought before(13). BrS is believed to be responsible for 4-12% of all SCD and around 20% of deaths in patients with apparently structurally normal hearts(14). Prevalence and prognosis of subjects with Brugada-type ECG pattern was studied in 2479 healthy young male Air Force applicants age 18-30 years) and in 542 healthy middle-aged subjects age between 40-60 years Finnish population. Fifteen (0.61%) subjects in the first population and three subjects in the second population (0.55%) fulfilled the ECG criteria for Type 1 or 2 Brugada syndrome pattern. They had J-point elevation and a saddleback-type ST-segment configuration in the right precordial leads. Type 1 Brugada ECG pattern (coved ST-segment elevation) was not seen in any subject. The benign natural course of the patients with the "Brugada sign" suggests that in asymptomatic subjects without a family history of SCD, Type 2 or 3 Brugada ECG pattern is a normal variant rather than a specific predictor of life-threatening ventricular arrhythmias(15). The prevalence of typical drug-induced Brugada syndrome ECGs was 5 of the 1,000 patients. This value was fivefold greater than the reported prevalence of spontaneous BrS ECGs in the healthy population(16) Blangy et al studdied the prevalence of BrS among 35,309 inhabitants of Lorraine (France) screened at preventive medicine center. ECGs of 35,309 individuals (mean age = 37.2 years, 47% men) recorded over a 1- year period were reviewed and classified as (1) typical, (2) suspicious, and (3) negative. Subjects whose ECG was suspicious were offered a provocative test with flecainide, 2 mg/kg, i.v., and individuals whose ECG was typical were advised to undergo Programmed Ventricular Stimulation (PVS). In 14 men and 6 women between the ages of 24 and 77 years (mean =47.5), ECGs were typical (n=6) or suspicious (n=14). Among 6 subjects with typical ECGs, 3 underwent PVS, which was positive in 1, who received an ICD. Among 14 subjects whose ECGs were suspicious, 5 declined further investigations and 5 developed typical ECG characteristics of BrS after flecainide administration. PVS was negative in 4 subjects who consented to the procedure. Overall, among 35,309 individuals screened, 11 had ECG findings consistent with BrS and, over a follow-up of 30 months, all had remained free of adverse cardiac event. The authors estimated a prevalence of BrS of 0.3% in Lorraine. A single patient received an ICD for inducible VT during PVS, representing a potential 30 per million asymptomatic adult rate of ICD implantation for this indication(17) Shin SC et al (18) studied the prevalence of Brugada-type ECG changes from a total of 225 healthy Korean male subjects with a mean age of 44 +/-13 (20-69) years with no syncope or family history of SCD. ECGs were taken from 4th, 3rd, and 2nd intercostals spaces and examined for Brugada-type ECG changes. There were none on the routine 12-lead ECGs, but 3 (1.3%) of the 225 subjects had a Brugada-type ECG recorded from the higher intercostals spaces and 1 of them had a Brugada-type ECG recorded at both the 2nd and 3rd intercostals spaces. The prevalence of the Brugada-type ECG was 1.3% at the 3rd intercostals space, 0.4% at the 2nd intercostals space. All were type 2. The authors conclude that some healthy Korean males with normal routine ECGs show Brugada-type 2 changes on ECGs recorded from higher intercostals spaces. References 1) Napolitano C, Priori S. Brugada syndrome. Orphanet J Rare Dis. 2006;135; 2) Moric E, Herbert E, Trusz-Gluza M, Filipecki A, Mazurek U, Wilczok T. The implications of genetic mutations in the sodium channel gene (SCN5A). Europace. 2003; 5:325-334 3) de Souza D, Riera AR, Bombig MT, et al. Electrocardiographic changes by accidental hypothermia in an urban and a tropical region. J Electrocardiol. 2006 Oct 4; [Epub ahead of print] 4) Riera AR, Schapachnik E, Ferreira C. Brugada disease: chronology of discovery and paternity. Preliminary observations and historical aspects. Indian Pacing Electrophysiol J. 2003;3:253-260; 5) Schulze-Bahr E, Eckardt L, Breithardt G, Sodium channel gene (SCN5A) mutations in 44 index patients with Brugada syndrome: different incidences in familial and sporadic disease. Hum Mutat. 2003; 21:651-652. 6) Brugada J, Brugada P, Brugada R. The syndrome of right bundle branch block ST segment elevation in V1 to V3 and sudden death-the Brugada syndrome. Europace 1999; 1:156-66. 7) Nademanee KK, Veerakul G, Nimmannit, S, et.al. Arrhytmogenic marker for the sudden unexplained death syndrome in Thai men. Circulation. 1997; 96:2595-2600. 8) Matsuo K, Akahoshi M, Nakashima E, et. al. The prevalence, incidence and prognostic value of the Brugada-type electrocardiogram: a population-based study of four decades. J Am Coll Cardiol 2001;38:765-770 9) Miyasaka Y, Tsuji H, Yamada K, et al. Prevalence and mortality of the Brugada-type electrocardiogram in one city in Japan. J Am Coll Cardiol 2001; 38:771-774. 10) Monroe MH, Littmann L. Two-year case collection of the Brugada syndrome electrocardiogram pattern at a large teaching hospital. Clin Cardiol. 2000;23:849-851. 11) Hermida J, Lemoine J, Aoun FB, et al.: Prevalence of the Brugada syndrome in an apparently healthy population. Am J Cardiol; 2000; 86:91-94. 12) Matsuo K, Akahoshi M, Nakashima E, et al. The prevalence, incidence and prognostic value of the Brugada-type electrocardiogram: a population-based study of four decades. J Am Coll Cardiol 2001; 38:765-770. 13) Monroe MH, Littmann L. Two-year case collection of the Brugada syndrome electrocardiogram pattern at a large teaching hospital. Clin Cardiol. 2000; 23:849-851. 14) Juang JM, Huang SK.Brugada syndrome--an under-recognized electrical disease in patients with sudden cardiac death.Cardiology. 2004; 101:157-169. 15) Junttila MJ, Raatikainen MJ, Karjalainen J, Kauma H, Kesaniemi YA, Huikuri HV. Prevalence and prognosis of subjects with Brugada- type ECG pattern in a young and middle-aged Finnish population. Eur Heart J. 2004; 25:874-878. 16) Hermida JS, Jandaud S, Lemoine JL, Rodriguez-Lafrasse C, Delonca J, Bertrand C, Jarry G, Rochette J, Rey JL. Prevalence of drug-induced electrocardiographic pattern of the Brugada syndrome in a healthy population. Am J Cardiol. 2004; 94:230-233. 17) Blangy H, Sadoul N, Coutelour JM, et al. Prevalence of Brugada syndrome among 35,309 inhabitants of Lorraine screened at preventive medicine center Arch Mal Coeur Vaiss. 2005; 98:175-180. 18) Shin SC, Ryu HM, Lee JH, et al. Prevalence of the Brugada-Type ECG Recorded From Higher Intercostal Spaces in Healthy Korean Males. Circ J. 2005; 69:1064-1067. All the best Andr?s Ricardo P?rez Riera Chief of Electro-Vectocardiology Sector of the Discipline of Cardiology, ABC Faculty of Medicine (FMABC), Foundation of ABC (FUABC) - Santo Andr? - Sao Paulo - Brazil. Rua Sebasti?o Afonso 885 - Zip Code: 044417-100- Jardim Miriam S.P Brazil- -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee > > Dr. Teruhisa Tanabe from Japan asks > > - Sudden cardiac death is most serious in patients with Brugada > syndrome and the number one concern. However, there are > differences in incidence of Brugada syndrome between race and > country or regions? Why do you think this occurs? > > > Dr. Andrea Sarkozy and Dr. Pedro Brugada from Belgium answer > > - Dear Dr Tanabe > Thank you for your actual and excellent question. The intriguing > differences between the incidence and perhaps other characteristics > of Brugada syndrome between the different geographical regions have > been supported by the strong clinical evidence; > 1, Population studies revealed that the incidence of the diagnostic > coved Brugada ECG pattern in the general asymptomatic Asian > (Japanese) population is much more frequent than in the Caucasian > population (0.1-0.4% vs. 0-0.1%). Similarly, sudden death due to > Brugada syndrome is also much more frequent in the south East Asian > population, then in the Caucasian; the syndrome is the leading > natural cause of death in young Thai men (1). 2, Sudden Unexplained > Death Syndrome (SUNDS) , first described in US immigrants, is a > disorder that had been prevalent for many years in south-east Asia, > particularly Thailand, Japan and the Phillipines. SUNDS is > characterized by sudden unexpected death at night in apparently > healthy men. 60% of the patients have the characteristics Brugada > ECG pattern on the baseline ECG (2). Recently, SCN5A mutations have > been identified in 3 of 10 patients with SUNDS. The gene mutation > resulted in similar ?loss-of-function? channel function alterations > as in Brugada syndrome. This data suggest that SUNDS and Brugada > syndrome are phenotypically, genetically and functionally the same > disorder (3). > However, there are differences between the Brugada syndrome in the > southeast Asian (SUNDS) and Caucasian patient populations; the man: > female ratio is much higher in the Asian patient population (8:1 in > SUNDS vs. 3:1 in the 3 European registries); the Asian patients die > almost exclusively during sleep while the Caucasian patients > sometimes die suddenly daytime. > Evidence based explanation is missing to account for these > differences, however some recent data might allow some speculations; > 1, In 2002, Splawski et al provided evidence that single nucleotide > common polymorphisms of the SCN5A gene can influence arrhythmia > susceptibility (6). About 13.2% of African Americans carried this > allele. The allele had a subtle effect on arrhythmia risk due to > subclinical sodium channel function modification. It was proposed > that in the presence of additional acquired risk factors, such as > medications, hypokalemia and structural heart disease, the > individuals with the allele have increased risk of arrhythmia. > 2, Ackerman et al in 2004 described in 829 healthy subjects > altogether 39 distinct missense variants of the SCN5A coding > region, including the previously described known 4 common single > nucleotid polymorphisms (SNP). Interestingly, 2 of the 8 most > frequent polymorphisms (allelic frequency >0.5%) showed a largely > ethnic specific distribution; the R1193Q single nucleotide > polymorphism occurred in 8% of the Asian vs. 0.3% of the white > population (and was entirely missing in the Hispanic and black > population), in contrast to the H558R polymorphism which occurred > in 20% of the white (29% of the black and 23% of the Hispanic > population) vs. in 9% of the Asian population ( (4). > 3, Recently, Bezzina et al described a similar ethnic specific > distribution in SNP distributions but in the SCN5A promoter region. > A certain combination of 6 single nucleotid polymorphisms > (designated as haplotype B variant) only occurred in Asian subjects > (at an allele frequency of 22%) and was absent in the other ethnic > groups. This haplotype variant resulted in decreased sodium channel > expression and function. Although it should be underlined that this > haplotype variant neither caused Brugada phenotype nor was more > frequent in the Brugada syndrome population, it clearly influenced > conduction velocities and was responsible for longer PR and QRS > intervals (5). > Additionally, in the last years several case reports proved that > certain SCN5A polymorphism in the presence of a Brugada syndrome > causing SCN5A mutation can influence the clinical phenotype and > thus clinical consequences of the mutation; the polymorphism can > rescue and restore or in contrast can further worsen the sodium > channel function. > Putting these pieces of evidence together in one picture, it is > possible that the currently best theory to answer your question is > the expansion of the multi-hit theory in long QT syndrome described > by Keating et al (7). The Asian population, as compared to the > Caucasian population, might have a different genetic background > consisting of ethnic specific SCN5A (or other ion channel function > influencing genes) polymorphisms. These polymorphisms influence > sodium channel function and/or expression, but only in a > subclinical manner; ?decreasing the antifibrillatory reserves? in a > large normally asymptomatic population. However, in these > individuals, in the setting of either another mutation or similar > function decreasing polymorphism (on the SCN5A or other ion channel > genes) or sodium channel blocking agents or other environmental > factors (gender, fever etc), the ion channel function is much more > easily depressed under the critical level to cause transient action > potential, and subsequent ECG abnormalities, provoking clinical > arrhythmias. > > > (1) Antzelevitch C et al: Brugada syndrome. Report of the second > consensus conference Circ 2005;111:659-70 > (2) Nademanee K et al: Arrhythmogenic marker for the sudden > unexplained death syndrome in Thai men Circ 1997;96:2595-600 > (3) Watta M et al: Genetic and biophysical basis of sudden > unexplained nocturnal death syndrome (SUNDS), a disease allelic to > Brugada syndrome Hum Mol Gen 2002;11:337-45 > (4) Ackerman MJ et al: Spectrum and prevalence of cardiac sodium > channel variants among black, white, Asian and Hispanic > individuals: Implications for arrhythmogenic susceptibility and > Brugada/long QT syndrome genetic testing Heart Rhythm 2004;1:600-7 > (5) Bezzina CR et al: Common sodium channel promoter haplotype in > Asian subjects underlies variability in cardiac conduction Circ > 2006;113:338-44 > (6) Splawski I et al: Variant of SCN5A sodium channel implicated in > risk of cardiac arrhythmia Science 2002;297:1333-6 > (7) Keating MT, Sanguinetti MC: Molecular and cellular mechanisms > of cardiac arrhythmias Cell 2001;104:569-80 > > -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061017/0eab5514/attachment.html From info at scd-symposium.org Tue Oct 17 17:14:35 2006 From: info at scd-symposium.org (SCD Symposium) Date: Tue, 17 Oct 2006 17:14:35 -0300 Subject: [SCD-FORUM] 52E Comments to clinical case Cuilan Li et al. Clinical patient with catecholamine sensitive polymorphic VT. Dr. Makarov Message-ID: Dear collegues! I want to add my comments to clinical case thad presented Dr. Cuilan Li et al. ?Clinical presentation and management of a patient with catecholamine sensitive polymorphic ventricular tachycardia (CPVT)?. By my mention it is typical clinical and ECG pattern for children and young patients with some of kind of catecholaminergic VT (CVT) that we described before (Makarov L. et al. Short PR interval, high circadian index and bradycardia ? pattern with high risk of syncope and sudden death in children with catecholaminergic ventricular tachycardia. Europ Heart J 2004; 25: 222, Suppl. Abstract) and during this symposium: bidirectional VT, bradycardia, short PR interval, and double tachycardia (VT and SVT). Some of this patients also demonstrated of the shortening of the QT interval (possibly as result of calcium overload of cardiomyocites). I am sure in arrythmogenic origin of syncope in present patient and useful of the active anthyarrhythmic therapy for it. From drugs more effective Beta-blockers (1-2 mg/kg) or/and calcium antagonists (verapamil), but authors had detected proarrhythmia from verapamil, that not typical for our experience. In the some patients the propafenone can be effective. Most of our patients with CVT showed improvement in their clinical symptoms: syncope events and ventricular tachycardia did not recur. But in a numerous patients that tolerant to therapy with recurrent syncope implantation of the ICD could be useful. I am not sure in useful of the EPI study for this patient, repetitive of Holter, stress test and/or IV infusion of isoproterenol more frequently detected typical bidirectional VT. Concern authors question # 3 (The catecholamine concentration in blood was normal for this patient. What do we expect of the catecholamine concentrations in CPVT patients in general?)- Fisher J.et al. (JACC1999; 34 (7): 2015-22) showed normal or lower level of plasma catecholapimes in rest in this patients, but parid increasing of it during exercise, to compare with normal range. Dr. Leonid Makarov M.D., Ph.D. professor (Pediatry) Moscow Institute pediatry and children surgery. 125412 Taldomskaya str.2 , Moscow, Russia. leo at oss.ru -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061017/e9f0d50f/attachment.html From info at scd-symposium.org Tue Oct 17 22:34:57 2006 From: info at scd-symposium.org (SCD Symposium) Date: Tue, 17 Oct 2006 22:34:57 -0300 Subject: [SCD-FORUM] 46R Electro-mechanical association. Dr. Iabluchanski Message-ID: <0D1656DE-5CE8-43B9-AEA1-F5DF2F633FA7@scd-symposium.org> Dear Sergio, It is known that the QT interval reflects the electrical systole; on the other hand, with the help of the echocardiogram, the mechanical systole can be evaluated. I would like to know how is the electro- mechanical relationship between these two systoles in relation to QT interval duration and T wave morphology (for instance, with the Tpeak- Tend interval). Sincerely, Dr.Nikolai Iabluchanski (Kharkiv, Ucrania) my at univer .kharkov.ua -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061017/7137c623/attachment.html From info at scd-symposium.org Wed Oct 18 07:18:50 2006 From: info at scd-symposium.org (SCD Symposium) Date: Wed, 18 Oct 2006 07:18:50 -0300 Subject: [SCD-FORUM] 48E RE: Young woman with HCM? Dr. Cano In-Reply-To: <8A0F216F-031D-447D-9E54-96D3510CA292@scd-symposium.org> References: <8A0F216F-031D-447D-9E54-96D3510CA292@scd-symposium.org> Message-ID: <80E62EC1-B0F3-445C-9EB6-7E43C08B65AE@scd-symposium.org> Dear Organizers of the Symposium, Thank you very much for this extraordinary Symposium!!! I was very impressed with the HCM cases. I work since 1998 with patients carriers of obstructive HCM, very symptomatic. We perform septal ablation by alcohol on them (already 37 patients). In the histories they describe, no patient had echo stress to determine if significant gradient appears with stress, if there is hypotension with strain. Often times it is difficult for them to articulate their symptoms, and when asked, we see that they are virtually invalid; they hardly leave their houses. They don't walk; well, they live in a secluded way. We only have one patient with ICD and it never shocked. The symptoms only improved after the obstruction was removed, and pulmonary hypertension disappeared when we decompressed the atrium. Silvia Judith Fortunato de Cano -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee > I write to you to introduce a clinical case. This patient is a 30 > year old woman, with a family history of non obstructive HCM with > genetic study that found a mutation in the Troponin T gene (TNT) > located in chromosome 1. The mutation is Arg92Gln. Four cousins > under 20 have suffered sudden death and one 45 year old aunt has > also suffered sudden death. Her mother and 3 other cousins have a > CDI because of the family history and evident HCM in Echo. Another > aunt and one cousin also have a CDI because syncope and evident HCM. > > Currently this patient has not presented symptoms and the EKG > shows a negative T wave in V1-V3 ( not present years ago), and the > previously normal Echo has changed, and now shows a mild > enlargement of left atrium and mild mid septum thickening of 12- > 13 mm, with no abnormal aspect and no obstruction. MRI is similar > to the Echo, and shows normal aspect of septum but thickness is > 13mm. No arrhythmias during 24 hrs Holter nor exercise testing. > > She is on atenolol but we would like to have your input in this > case on whether this justifies a CDI now. > > Besides, we would like to know your opinion about young members of > this family. There are children and young persons with apparently > normal Echo but they have one or two brothers with sudden death or > syncope. ?Do you think they also need a CDI? Finally, what about > the adult and older members of this family? Probably most have an > evident HCM in Echo. Would it be enough with atenolol or with > amiodarore alone, or do they also need a CDI?. > > We appreciate and thank you for your time and suggestions. > > Most Sincerely > > Rene Asenjo -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061018/71d5b968/attachment.html From info at scd-symposium.org Wed Oct 18 08:36:54 2006 From: info at scd-symposium.org (SCD Symposium) Date: Wed, 18 Oct 2006 08:36:54 -0300 Subject: [SCD-FORUM] 55S Cardiorespiratory arrest and sudden cardiac death. Dr. Ropero Message-ID: <21A7F30F-B2BB-4B38-B48C-FB4F8931AAA6@scd-symposium.org> Dear colleagues, I am writing to this list because I would to have some concepts clarified to me regarding the subject of discussion. I have looked through several articles, books and journals, and they are not clear in this sense. For instance: the Spanish guidelines for CPR mention that there is a very narrow and arbitrary limit between the concepts of cardiorespiratory arrest and sudden cardiac death, leaving one for statistic limits, and the cardiorespiratory arrest as a clinical approach of the problem. My question for the experts: Is there some way of defining both concepts, or do they overlap in such a way that they can be used without distinction in related studies and papers? Other authors point out ?reanimation from sudden cardiac death?. Could it be reanimation from a cardiac arrest? Is it not death precisely that, ?death?? I hope you can clarify this dilemma for me, and thanking you in advance, Dr. Oscar Ruiz Ropero Intensivista Hospital General Docente. Guantanamo. Cuba. -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061018/573697a8/attachment.html From info at scd-symposium.org Wed Oct 18 10:21:45 2006 From: info at scd-symposium.org (SCD Symposium) Date: Wed, 18 Oct 2006 10:21:45 -0300 Subject: [SCD-FORUM] READING THE LECTURE BY DR. VEENHUYZEN and DR WYSE. THREE EXPERT QUESTIONS BY DR. SERGE BOVEDA Message-ID: <1AB96FC9-B4D9-4048-A9E2-91B6C1C6010C@scd-symposium.org> Reversible Causes of VT/VF: Fact or Fiction? Case presentation and review of the literature G. D. Veenhuyzen, MD D. George Wyse, MD, PhD Dr. Serge Boveda (France) - Your case report clearly shows that transient causes are more likely to occur in patients suffering from severe heart disease: most of the time they should be implanted with an ICD for primary prevention. Don?t you think that guidelines concerning ICD implantation among patients with VT/VF due to transient or reversible disorders should mainly concern patients with no (or mild) structural heart disease? Dr. George Veenhuyzen and George Wyse (Canada) - The current guidelines do not advise an ICD for VT/VF with transient or reversible causes but are slilent on the issue of co- existing heart disease. It is true our case had extensive heart disease and it may be true such patients are more likely to have recurrence of the so-called transient or reversible cause as the substrate for arrhythmia continues to exist. The difficulties are twofold. First, in patients with other conditions such as renal and lung disease, the transient or correctable causes (electrolyte abnormalities and hypoxemia) continue to recurr unpredicatably. Second, it is difficult to be sure there is not a continued substrate. For example, in a patient rescued from drowning and found to have VF, it may later be difficult to exclude long QT syndrome. Thus, as stated in our paper, it requires a great deal of clinical judgment to determine if VT/VF had a truly tansient or correctable cause and is unlikely to recur. I do not think there will ever be a simple set of rules or conditions that replace clinical judgment and would favor a guideline that is more permissive in this area. Dr. Serge Boveda (France) - Concerning the ?trigger? of VT/VF, do you think that successful ablation of the ?triggering VPB?s? (as demonstrated by Michel Ha?ssaguerre for Purkinje or Brugada patients?) should be considered as a reversible cause and by the way, avoid ICD implantation in patients with no (or mild) structural heart disease? Dr. George Veenhuyzen (Canada) - I think it is too early to tell if ablating "triggering" VPBs will be complete treatment and in which cases. So far there has been few reports in relatively selected patients. Brugada and other channelopathies are a rather small number of VT/VF cases. The thing about VPBs are they are rather ubquitous, like weeds in your garden. When you remove one it is soon replaced by another. Thus, I am doubtful at the moment that this would be a complete solution in many patients. Dr. Serge Boveda (France) - Regarding with literature data, how long would you consider that VT/ VF is a transient or reversible cause after an acute myocardial ischemia? Dr. George Veenhuyzen (Canada) - I think ischemia is a truly reversible cause mostly in the absence of scar (previous infarction) until a defintive treatment like revascularization is applied. The best example is vasospastic angina that causes VT/VF in an otherwise normal heart. Good medical therapy (two or more vasodilators and a statin) can be effective treatment. I have a few such cases with defibrillators who have never had a therapy from there ICD in over 10 years. However, it is important to remember that ischemia can return in the case of atherosclerotic disease due to progression of disease or incomplete revascularization. -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061018/2e32220a/attachment.html From info at scd-symposium.org Wed Oct 18 10:24:17 2006 From: info at scd-symposium.org (SCD Symposium) Date: Wed, 18 Oct 2006 10:24:17 -0300 Subject: [SCD-FORUM] 53E RE: Some questions about Brugada Syndrome. Dr. Perez Riera to Dr. Kam In-Reply-To: <08A19966-633A-473A-BB8D-0CC5A3443026@scd-symposium.org> References: <08A19966-633A-473A-BB8D-0CC5A3443026@scd-symposium.org> Message-ID: Dear Dr Ruth Kam from Singapore. Here Andr?s Ricardo P?rez Riera from Sao Paulo Brazil. During phase 1 of AP, although brief, it is possible to observe several categories of important ion channels for its profile determination: Ito1, Ito2, IKATP, ICl.swell and Na+ outward movement through the Na+/Ca2+ exchanger operating in reverse mode (Na+/Ca2+). The Ito1 channel, ItoA, transient outward current sensitive to 4- aminopyridine (4-AP), calcium-independent transient outward current, initial repolarization, Ca2+ independent, voltage-operated channel, voltage-dependent Ca2+ independent transient outward current, Itof or Io-fast . It is a of voltage and time dependent, besides determining the initial phase configuration of repolarization of the AP profile, it is fundamental in its duration (APD) and in determining the repolarization heterogeneity in the ventricular myocardium thickness. Some evidence point that the cloned subunit Kv4.3 is similar to the human Ito1. The Ito current density depends on a number of factors: age group (absent in newborn babies), sex, heart rate (more noticeable in bradycardia), cell type studied, localization in ventricular wall thickness, topography of myocardium and pathologic circumstances with or without organic substrate. In BrS, an entity without apparently structural heart disease, with the fast Na+ current as genetic determinant of the channelopathy, the initial outward K+ Ito1 current and the slow inward Ca2+ current in phase 2 are essential regarding J point and ST segment level in surface ECG, and consequently, in triggering reentry in phase 2(RF2), and triggering bursts of PVT/IVF. Several drugs, such as quinidine, disopyramide, flecainide, ajmaline, procainamide, pilsicainide, etc., by modifying the functional state of the Ito1 current, alter J point and ST segment level in right precordial leads or from V1 to V3 in BrS. Rarely (8% of cases), the early repolarization syndrome (ERS) may be confused with BrS, for presenting a Brugada-like electrocardiographic pattern. There are clinical-electrocardiographic elements that help in making this important differentiation. Phase 1 of AP, of initial, early or fast repolarization, coincides with J point in surface ECG (end of QRS complex and beginning of ST segment), being essentially dependent on fast inward Na+ current closure and transient opening of outward K+ currents. During the short phase 1, several channels get started: 1) Ito1, ItoA, transient outward current sensitive to 4- aminopyridine (4-AP), calcium-independent transient outward current, voltage-operated channel, voltage-dependent Ca2+ independent transient outward current, Itof or Ito-fast since it has fast activation and inactivation kinetics. Inactivation is also time- dependent. The Kv4.3 current has been identified as the major and main cloned subunit similar to the Ito1 current in humans(1); 2) Ito2, Itob, Ca2+-activated current, ICl.Ca, Ca2+ activated chloride (Cl-) current, calcium-activated transient outward chloride current, current component of the transient outward current, 4- aminopyridine resistant transient outward current - carried by Cl- ions, slow activation current, Ito-s or Ito-slow, 4-AP-resistant component; 3) Variant activated by the fall in intracellular supply of ATP when it reaches a certain critical level (IKATP); CLcAMP or time- independent chloride Cl- current regulated by the cAMP/adenylate cyclase pathway. Activation of the ATP-sensitive potassium current, IKATP, is sufficient to cause ST elevation during acute ischemia; 4) Swelling-activated Cl- current (ICl(swell)): Characteristics and functions of the cardiac swelling-activated Cl current are considered in physiologic and pathophysiologic settings. I(Cl,swell) is broadly distributed throughout the heart and is stimulated not only by osmotic and hydrostatic increases in cell volume, but also by agents that alter membrane tension and direct mechanical stretch. The current is outwardly rectifying, reverses between the plateau and resting potentials, and is time-independent over the physiologic voltage range. Consequently, I(Cl,swell) shortens APD, depolarizes, and acts to decrease cell volume. Because it is activated by stimuli that also activate cation stretch-activated channels, I(Cl,swell) should be considered as a potential effector of mechanoelectrical feedback. I(Cl,swell) is activated in ischemic and non-ischemic dilated cardiomyopathies and perhaps during ischemia and reperfusion. The current plays a role in arrhythmogenesis, myocardial injury, preconditioning, and apoptosis of myocytes. As a result, I(Cl,swell) potentially is a novel therapeutic target(2); 5) The Na+ outward movement through the Na+/Ca2+ exchanger operating in reverse mode: The sarcolemmal Na+/Ca2+ exchanger is regulated by intracellular Ca2+ at a high affinity Ca2+ binding site separate from the Ca2+ transport site. The Ca2+ regulatory site is located on the large intracellular loop of the Na+/Ca2+ exchange protein. Secondary Ca2+ regulation with the exchanger in the forward or Ca2+ efflux mode. The Ca2+ regulation modifies transport properties and does not only control the fraction of exchangers in an active state. CHARACTERISTICS OF MODALITIES OF THE CHANNELS THAT AFFECT PHASE 1 OF AP Ito1, IA, transient outward K+ current 1, 4-aminopyridine or 4-AP- sensitive current, the Ca2+ independent Ito1, activated during phase 1, Itof or Ito-fast This channel activity occurs in phase 1 of AP in early or fast repolarization. Phase 1 coincides with the J point of surface ECG Ito1 channel is voltage-operated, and therefore, it is opened by changes in voltage in a range around the 0mV (from +30mV to -10mV). The Ito channel is activated or inactivated, depending on instantaneous voltage. Thus, the activation is processed in the band between - 30mV and +10mV. The inactivation process is time-dependent, too. The Ito1 current is not found in newborn babies, and it only becomes manifest after three to five months in dogs, which explains the absence of notch in epicardial and M cells in newborn babies (age heterogeneity). The predominance of the Brugada phenotype in males is a result of the presence of a more prominent Ito in males versus females(3-4). Male predominance of the phenotype observed in SUDS does not apply to a large European family with a missense mutation, R367H, previously associated with SUDS suggesting that factors other than the specific mutation determine the gender distinction(5). According to Antzelevitch et al, the consequences of this unequal distribution of Ito1 channels in ventricular myocardial thickness are(6) 1) Alterations of the ST segment, variously referred to as J wave, junctional wave, late delta wave, Osborn wave, camel-hump sign, and hump-like deflection found characteristically in severe hypothermia. J wave is not pathognomonic of sever hypothermia and also it has also been described in other clinical entities not associated with hypothermia, such as acute brain injury (subarachnoid hemorrhage) (7);, accidental cocaine overdose(8), cardiac arrest, dysfunction of cervical sympathetic system, hypercalcemia(9) and BrS. 2) Unequal sensitivity to drugs: acetylcholine, isoproterenol, Ca2+ antagonists, Na+ channel blockers, K+ channel openers, amiodarone; 3) Greater dependence of AP duration in epicardial cells regarding heart rate. The epicardial AP when compared with that of endocardium shows a smaller phase 0 amplitude, a much more prominent phase 1, and a phase 2 amplitude that is greater than that of phase 0. Epicardial APs, unlike those of endocardium, display a "spike and dome" morphology that becomes progressively more accentuated at slower stimulation rates (10); 4) AP of epicardial cells more sensitive to K+: changes in T wave. Voltage gradients created by heterogeneities of the slow-delayed rectifier potassium current( IKs) inscribe the T wave and T-wave polarity and width are strongly influenced by the degree of intercellular coupling through gap-junctions. Changes in K+ modulate the T wave through their effect on the rapid-delayed rectifier IKr. Alterations of IKs , IKr, I and I(Na) (fast sodium current) in long- QT syndrome (LQT1, LQT2, and LQT3, respectively) are reflected in characteristic QT-interval and T-wave changes; LQT1 prolongs QT without widening the T wave. Accelerated inactivation of I(Na) on the background of large epicardial I(to) results in ST elevation (Brugada phenotype) that reflects the degree of severity. Activation of the ATP-sensitive potassium current, I(K(ATP)), is sufficient to cause ST elevation during acute ischemia.; 5) Presence of supernormal phase just in the epicardium, and not in the endocardium; 6) In the "M" cells, the Ito1 channel is found only in the epicardium, and not in the ventricular endocardium. A transmural voltage gradient during initial ventricular repolarization, which results from the presence of a prominent Ito mediated AP notch in the epicardium, but not endocardium, manifests as a J-wave on the ECG. The J-wave is associated with the ERS, BrS and others entities. ST-segment elevation, as seen in BrS and acute myocardial ischemia, cannot be fully explained by using the classic concept of an "injury current" that flows from injured to uninjured myocardium. Rather, ST-segment elevation may be largely secondary to a loss of the AP dome in the epicardium, but not endocardium. The T-wave is a symbol of transmural dispersion of repolarization. The R-on-T phenomenon (an extrasystole originating on the T-wave of a preceding ventricular beat) is probably due to transmural propagation of F2R early after depolarization that could potentially initiate PVT/ VF (11). The Ito, inward rectifier IK, IKATP, IK-Ach and delayed rectifier potassium channels ( IKS, IKr and IKur) are blocked by quinidine. This drug of the IA class, with intermediate kinetics of uptake and release with the Na+ current (4 to 8 seconds), moderately reduces maximal velocity and it extends AP, and consequently, the effective refractory period by block of the multiple outward K+ currents in phases 1 to 3, increasing JTc and QTc intervals and fostering the appearance of EADs; and these in turn, foster the triggered activity that will lead to a higher tendency to TdP. It is very important understand that quinidine and disopyramide block the Ito1 current, but other members of the class don't, such as procainamide and ajmaline. This subtle difference is very significant in PVT/VF genesis in BrS. By its nonspecific potassium channel blocking action, quinidine may also reduce arrhythmia recurrence. Additionally, it could improve repolarization due to its vagolytic effect (M2 muscarinic receptor block) and to the exacerbation of reflex sympathetic tone. Oral quinidine has a role in the treatment of electrical storm (ES) in BrS(12-13). The Ito1 current is more visible, causing a greater notch, during slow cardiac rates, and it plays an important role in the early phase of AP and it influences on phase 2, plateau or dome, and consequently, in AP duration (APD). Ito1 current density is very reduced and consequently, it extends AP in genetically-conditioned and salt-induced high blood pressure, in after-constriction hypertrophy of pulmonary artery, 21 days after acute infarction by remodeling and in heart failure (pathologic heterogeneity) (14). The latter leads to a significant reduction of Ito1 density and a marked prolongation in APD. The mechanism of this reduction is unknown. The alpha subunit of the K+ current, a homologue of the Drosophila Shal family, is very probably an encoder of all or a part of the native Ito current (15). II) Ito2, ItoB, Ca2+ activated channel, ICl.Ca, Ca2+ activated chloride (Cl-) current, Ca2+ channel activated chloride (Cl-) current, 4-aminopyridine-resistant transient outward current carried by Cl- ions, slow activation Ito-s or Ito-slow current. The evidence of the Ito2 current existence is partially founded on the pharmacological effect of several Cl- current blockers. The Ca2+- activated Cl(-) current [I(Cl(Ca2+] contributes to the repolarization of the cardiac AP under physiological conditions. I(Cl Ca2+) is known to be primarily activated by Ca2+ release from the sarcoplasmic reticulum (SR). L-type Ca2+ current represents the major trigger for Ca2+ release in the heart. Recent evidence, however, suggests that Ca2 + entry via reverse-mode Na+/Ca2+ exchange promoted by voltage and/or Na+ current may also play a role (16). The Ito2 channel could be activated by: 1) Increase in intracellular Ca2+ concentration, which in turn releases the sarcoplasmic reticulum cation(17); 2) Acetylcholine that hyperpolarizes potential and shortens AP. The latter is found in the sinus node, AV node and atrial muscles; 3) Arachidonic acid and its metabolites. The Ito2 channel is blocked by disulphonic stilbenes derivatives (SITS-DIDS) (18); III) Variant activated by fall in ATP supply when it reaches a given critical level (IK ATP), CLcAMP, or time-independent chloride Cl- current regulated by the cAMP/adenylate cyclase pathway. Activation of the ATP-sensitive potassium current, IKATP, is sufficient to cause ST elevation during acute ischemia; IV) Swelling-activated Cl- current or ICl-swell. Characteristics and functions of the cardiac swelling-activated Cl current or ICl-swell are considered in physiologic and pathophysiologic settings. ICl- swell is broadly distributed throughout the heart and is stimulated not only by osmotic and hydrostatic increases in cell volume, but also by agents that alter membrane tension and direct mechanical stretch. The current is outwardly rectifying, reverses between the plateau and resting potentials and is time-independent over the physiologic voltage range. Consequently, I Cl-swell shortens APD, depolarizes, and acts to decrease cell volume. Because it is activated by stimuli that also activate cation stretch-activated channels, ICl-swell should be considered as a potential effector of mechanoelectrical feedback. ICl-swell is activated in ischemic and non-ischemic dilated cardiomyopathies and perhaps during ischemia and reperfusion. ICl-swell plays a role in arrhythmogenesis, myocardial injury, preconditioning, and apoptosis of myocytes. As a result, ICl- swell potentially is a novel therapeutic target.() This channel is inhibited by 9-anthracene carboxylic acid. Its activation causes AP shortening; V) Na+ outward movement through the Na+/Ca2+ exchanger operating in reverse mode. This mechanism exchanges 3 Na+ cations for 1 of Ca2+. The direction of the Na+ movement depends on membrane potential and intra and extracellular Na+ and Ca2+ concentration. The inflow mediated by this current of Na+/Ca2+ exchange can trigger Ca2+ release in the sarcoplasmic reticulum system. CHARACTERISTICS AND ROLE OF THE Ito1 CURRENT IN VENTRICULAR REPOLARIZATION Not all of the myocardial cells have the Ito1 current and its concentration or density depends on the area being studied. The myocardial cells that have a high density of this channel are characterized for presenting a prominent notch in phase 1 of AP, showing a profile with a spike-and-dome configuration. Thus, in the ventricular myocardium, only the fast Purkinje fibers, the M cells of the middle myocardium, and those of the subepicardium have a significant notch (regional heterogeneity). There are marked differences in phases 1 to 3 in ventricular myocardium cells AP and contractile cells when we consider thickness. Thus, we distinguish three areas besides the Purkinje cells present in the cardiac conduction system. This unequal distribution of the Ito1 current in ventricular myocardial thickness is responsible for: 1) Idiopathic J wave, Junctional wave, injury potential, late d, Osborn wave, camel-hump sign or hump-like deflection, which could possibly be found in the J point region of surface ECG in hypothermia (19), brain lesion(20), over come coma, hypercalcemia(21), massive ingestion of cocaine(22), and others. When present in right precordial leads V1-V2 or from V1 to V3 in a patient without structural heart disease, it is known as Brugada sign. Rarely (8% of cases) it has been reported in the athlete as a benign Early Repolarization Syndrome (ERS) (23); 2) Unequal sensitivity to different drugs: acetylcholine, isoproterenol, Ca2+ antagonists, Na+ current blockers, K+ current openers and amiodarone; 3) Higher dependency of APD of epicardial cells in relation to heart rate changes; 4) Epicardial cellular AP, more sensitive to K+, and consequently, there are changes in the aspect of T wave polarity; 5) Presence of supernormal phase only in the epicardium and not in the endocardium; 6) The depth of phase 1 Ito1 dependent is more marked in the right ventricle (RV) when compared to the left one, which explains the higher vulnerability of the RV in arrhythmias triggering in acute ischemia conditions(24). In atrial cells, there are Ito currents that are opened by vagal acetylcholine release. These currents are coupled in the acetylcholine uptake in the sarcolemma. BrS is considered an ion channel entity or channelopathy (25). The main affected channels in the BrS are primarily the fast Na+ current, and secondarily the initial outward K+ current, and the L- type slow or long-lasting calcium channel ICa-L type ICa2+-L. Others channels affected with minor importance are Ito2, IK-ATP and IKr. The presence of a deeply notched AP or with spike-and-dome configuration in the epicardium of the RVOT, but not in the endocardium, is responsible for the duration of the dome or phase 2 lasting approximately a 70% less, causing a marked decrease in APD in the epicardium in relation to the endocardium in ventricular wall thickness of the RVOT. The phenomenon originates a ventricular transmural gradient due to the coved type elevation( convex to the top) of the J point and the ST segment in the right precordial leads V1-V2 or on anteroseptal wall V1 to V3 (Brugada sign), sometimes followed by inverted T wave(26).. The J wave is a deflection with a dome that appears on the ECG after the QRS complex. A transmural voltage gradient during initial ventricular repolarization, which results from the presence of a prominent AP notch mediated by the transient outward potassium current or initial outward K+ current in epicardium but not endocardium, is responsible for the registration of the J wave on the ECG. Another variety of J point and ST segment elevation that may be observed in BrS is a less characteristic one, that of the saddleback type, conditioned by just a partial loss of dome, plateau or phase 2 in the RV epicardium. In it, the degree of dispersion is minimal, with a much lower tendency to appearance of PVT/VF (27). The coved- type J point and ST segment elevation may rarely be observed in the inferior wall leads in absence of hypothermia, ischemia or electrolytic disorders in patients without structural heart disease, configuring the so-called atypical Brugada pattern or latent type (27-28-29-30-31). Certain blockers of the fast Na+ current, such as Class IA and IC antiarrhythmic drugs ajmaline, procainamide, propafenone, flecainide, pilsicadine. and acetylcholine (vagal stimulation) (32), enhance phase 1 notch in RV epicardial cells, with a subsequent shortening in dome or phase 2 duration. This fact results in a non-homogeneous and more heterogeneous repolarization dispersion in the ventricular myocardial thickness, between the subendocardium and the subepicardium, fostering the substrate for developing reentry in phase 2, a mechanism responsible for IPVT/IVF in BrS. When the outward current shift is marked, premature repolarization occur in epicardial myocardium and the resulting gradient may precipitate P2R. Flecainide shortens the QT interval of variant 3 of congenital long QT syndrome (LQT3), so its oral administration has been proposed to treat this variant. Additionally, in these patients it can cause "Brugada-like" J point and ST segment elevation(33). Flecainide may induce ST segment elevation in LQT3 patients, raising concerns about the safety of flecainide therapy and demonstrating the existence of an intriguing overlap between LQT3 and BrS(34). Low- dose, oral flecainide consistently shortened the QTc interval and normalized the repolarization T-wave pattern in LQT3 patients with SCN5A:DeltaKPQ mutation(35). A class IB sodium channel blocker, mexiletine, significantly shortens QTc, thus preventing the appearance of TdP. Strangely, the drug does not shorten long QT in congenital LQTS, which affects the K+ current (HERG defect of the K+ current) or variant 2 of LQTS. Mexiletine, is most effective in abbreviating QT interval in LQT3, but effectively reduces transmural dispersion of repolarization (TDR) and prevents the development of Td P in all LQT1, LQT2 and LQT3 models, suggesting its potential as an adjunctive therapy in LQT1 and LQT2(36). The use of drugs that inhibit the Ito1 current or that stimulate Ca2+ inward movement can decrease the degree of J point and ST segment elevation and improve repolarization in this entity . Thus, the Ito1 blocker with 4-aminopyridine (1 to 2mmol/L) or quinidine (5 micromol/ L) increase phase 2 or dome duration and normalize ST segment elevation preventing TV/FV(37). Oral quinidine suppress the electrical storm and prevented VF episodes in BsS patients(38). Oral quinidine reduces phase 1 extent mediated by Ito1, normalizing ST segment elevation in right precordial leads or from V1 to V3. IA class drugs that block Na+ current and additionally Ito1, such as quinidine and disopyramide, improve ECG in BrS, while those of the same class, such as procainamide and ajmaline, which block exclusively the Na+ current without affecting the Ito1 current, worsen ST segment elevation and may trigger fatal tachyarrhythmias in BrS(39). Oral quinidine induce ECG normalization in patients with BrS (40). Publications report the employment of the drug in malignant forms of the entity(41). Associated with adrenergic beta1-agonist and the parasympathetic antagonist was used (42). The presence of mild ischemia and vagotony act sinergically with the electrophysiologic substrate of BrS, elevating ST segment and triggering PVT/IVF bursts. This observation suggests that the Brugada Patients are under a higher risk of SCD in coexistence with ischemia (43). On the contrary, isoproterenol restores phase 2 or dome in the epicardium, reducing J point and ST segment elevation. The vasodilator cilostazol acts through a similar mechanism: increase ICa +2-L, and for this reason may be effective in reducing episodes of PVT/VF(44).For this reason, isoproterenol is the drug of choice in ES in BrS associated with general anesthesia and cardiopulmonary "bypass" diminishing the ST elevation in right precordial leads disappearance of the short-coupled premature beats and in removing ES crisis of VF(45). This ominous-sounding event consists of the incessant appearing of recurring episodes and multiple VF or VT: 20 or more per day or 4 or more per hour, eventually observed in BrS. The ECG pattern in BrS can be intermittent and become manifest in latent cases due to some IA class (procainamide and ajmaline) and IC class (flecainide) antiarrhythmic agents and by night vagotony(46) These facts support the hypothesis that J point and ST segment elevation and the subsequent triggering of PVT/VF are dependent of a prominent Ito current and spike-and-dome morphology in the RV epicardium(47). In early repolarization syndrome (ERS), a normal benign variant, found in 1% to 2% of the population, and 13% to 48%(48) in emergency rooms in patients with precordial pain, J point and ST segment elevation usually presents a concavity higher >/=1mm in limb leads and >/=2 in precordial leads, in at least two adjacent leads and with notch or slurring of the R terminal portion of the QRS complex, followed by T waves of enhanced voltage and concordant polarity in the intermediate leads from V2 to V4. The most important differential diagnosis of ERS is pericarditis, acute infarction and acute coronary syndromes that could be treated mistakenly with fibrinolysis or unnecessary angiography(49). In doubtful cases, besides a careful anamnesis, the following must be conducted: echocardiogram, enzyme and troponin I dosage(50). There are evident differences and potent gradients in Ito1 between the three cardiac cell types, especially between Epi and Endo cells. These differences are among the prominent manifestations of right ventricular electrical heterogeneity, and may form an important ionic basis and prerequisite for some malignant arrhythmias in the right ventricle, including those arising from BrS and other diseases(51). ERS can be confused as well, with ventricular aneurysm. ERS is very frequent in athletes, in whom it is observed in more than 80% of the cases. Rarely (8%), it can present a configuration that reminds the Brugada sign or is Brugada-like. In such cases, the following are elements in favor of ERS (modified from Bianco.) (23). 1) Family history: negative in ERS and frequently positive for SCD in BrS; 2) HR: tendency to bradycardia in ERS. In BrS, heart rate is usually normal; 3) SAQRS: in ERS it tends to be vertical, and in BrSe in a 9% of cases it presents an extreme deviation to the left; 4) PR interval: tendency to be short or normal and mildly depressed in ERS. In BrS, it is long and in a 50% of cases (first- degree AV block) by increase of HV; 5) QRS duration: larger in BrS (110msec +/-2msec) than in athletes carriers of ERS (90msec+/-1msec) 6) Transition area in precordial leads: it is usually abrupt in ERS by counterclockwise rotation in longitudinal axis. This is not observed in BrS; 7) Degree of ST segment elevation: much larger in BrS (4.4 +/-0.7mm) than in athletes (2.3+/-0.6mm) or non-athletes (1.2 +/-0.8mm) carriers of ERS; 8) Race: it predominates in the black race in ERS. In BrS, in the yellow race. 9) U wave: it is usually very visible in V3 due to bradycardia in ERS. It is not frequent in BrS. Coincidences between BrS and benign ERS: 1) Exercise can normalize ST segment elevation; 2) Isoproterenol can normalize ST segment elevation; 3) More frequent in males; 4) Predominantly observed in young adults in productive age and under 50 years old(54). 5) Both can have ST segment elevation concave to the top, saddleback type, and frequently persistent; The Ito current has a decisive role in the aspect of the early repolarization phase. Additionally, it influences on inward and outward movement of other ions in the next phase (phase 2) and in AP refractoriness. References 1) Greenstein JL, Wu R, Po S, Tomaselli GF, Winslow RL Role of the calcium-independent transient outward current I(to1) in shaping action potential morphology and duration. Circ Res. 2000; 87:1026-1033. 2) Baumgarten CM, Clemo HF. Swelling-activated chloride channels in cardiac physiology and pathophysiology. Prog Biophys Mol Biol. 2003;82:25-42. 3) Di Diego JM, Cordeiro JM, Goodrow RJ, et al. Ionic and cellular basis for the predominance of the Brugada syndrome phenotype in males. Circulation 2002;106: 2004-2011. 4) Antzelevitch C. Androgens and male predominance of the Brugada syndrome phenotype. Pacing Clin Electrophysiol. 2003;26:1429-1431. 5) Hong K, Berruezo-Sanchez A, Poungvarin N, et al. Phenotypic characterization of a large European family with Brugada syndrome displaying a sudden unexpected death syndrome mutation in SCN5A J Cardiovasc Electrophysiol. 2004;15:70-71. 6) Antzelevitch C, Litovski SH, Lukas A: Epicardium vs. Endocardium. Electrophysiology and pharmacologhy. In Zipes DP, and Jalife J, eds: Cardiac Electrophysiology: From Cell to Bedside. NY, W.B. Saunders, 1990, pp386-395. 7) Carrillo-Esper R, Limon-Camacho L, Vallejo-Mora HL, ET AL. subarachnoid hemorrhage(Non-hypothermic J wave in subarachnoid hemorrhage Cir Cir. 2004;72:125-129. 8) Grigorov V, Goldberg L, Foccard JP. Cardiovascular complications of acute cocaine poisoning: a clinical case report. Cardiovasc J S Afr. 2004;:139-142. 9) Topsakal R, Saglam H, Arinc H, et al. Electrocardiographic J wave as a result of hypercalcemia aggravated by thiazide diuretics in a case of primary hyperparathyroidism. Jpn Heart J. 2003;44:1033-1037. 10) Litovsky SH, Antzelevitch C. Transient outward current prominent in canine ventricular epicardium but not endocardium. Circ Res. 1988; 62:116-126. 11) Yan GX, Lankipalli RS, Burke JF, Ventricular repolarization components on the electrocardiogram: cellular basis and clinical significance. J Am Coll Cardiol. 2003;42:401-409. 12) Bettiol K, Gianfranchi L, Scarfo S, Successful treatment of electrical storm with oral quinidine in Brugada syndrome. Ital Heart J. 2005; 6:601-602. 13) Marquez MF, Rivera J, Hermosillo AG, Arrhythmic storm responsive to quinidine in a patient with Brugada syndrome and vasovagal syncope. Pacing Clin Electrophysiol. 2005; 28:870-873. 14) Nabauer M, Beuckelmann DJ, Erdmann E.Characteristics of transient outward current in human ventricular myocytes from patients with terminal heart failure. Circ Res. 1993; 73:386-394. 15) K??b S; Dixon J; Duc J. et al. Molecular Basis of Transient Outward Potassium Current Down regulation in Human Heart Failure A Decrease in Kv4.3 mRNA Correlates With a Reduction in Current Density Circulation. 1998;98:1383-1393. 16) Sun H, Chartier D, Nattel S, Ca(2+)-activated Cl(-) current can be triggered by Na(+) current-induced SR Ca(2+) release in rabbit ventricle. Am J Physiol. 1999;277:H1467-H477. 17) Coreboeuf E, Carmeliet E. Existence of two transient outward currents in sheep cardiac Purkinje fibers. Plugers Arch 1982;392:352.) ( Hiraoka M Kawano S. Calcium-sensitive and insensitive transient outward in rabbit ventricular myocytes. J Phisiol (London) 1989;410:187. 18) Wang HS, Dixon JE, McKinnon D.Unexpected and differential effects of Cl- channel blockers on the Kv4.3 and Kv4.2 K+ channels. Implications for the study of the I(to2) current. Circ Res. 1997;81:711-718. 19) Tomaszewski W. Changements electrocardiographiques observes ches un homme mort de froid. Arch Mal Coeur 1938;31:525-528. 20) Mayer SA.: Systemic Hypotermia for acute cerebral infarction. Rev Soc Cardiol Estado de S?o Paulo. 1999;9:600-605. 21) Sridharan, MR, Horan LG.: Electrocardiographic J waves of hypercalcemia. Am J. Cardiol 1984; 54:672-673. 22) Ortega-Carnicer J, Bertos-Polo J, Gutierrez-Tirado C. Aborted sudden death, transient Brugada pattern, and wide QRS dysrrhythmias after massive cocaine ingestion. J Electrocardiol 2001;34:345-349. 23) Bianco M, Bria S, Gianfelici A, Does early repolarization in the athlete have analogies with the Brugada syndrome? Eur Heart J. 2001;22:504-510. 24) Wettwer E Amos S, Posival H et al. Transient outward current in humam ventricula myocytes of subepicardial and subendocardial origin. Circ Res 1994;75:473. 25) Balser JR. Sodium "channelopathies" and sudden death: Must you be so sensitive? Circ Res 1999; 85:872-874. 26) Kurita T, Shimizu W, Inagaki M, et al. The electrophysiologic mechanism of ST-segment elevation in Brugada syndrome. J Am Coll Cardiol 2002;40:330-333. 27) Oliva A, Figueiredo M. Electrocardiographic diagnosis of Brugada syndrome: Medico-legal implications. J Electrocardiol 2001;34:321. 28) Kalla H, Yan GX, Marinchak R. Ventricular fibrillation in a patient with prominent J (Osborn) waves and ST segment elevation in the inferior electrocardiographic leads: a Brugada syndrome variant? J Cardiovasc Electrophysiol 2000;95-98. 29) Takagi M, Aihara N, Takaki H, et al. Clinical characteristics of patients with spontaneous or inducible ventricular fibrillation without apparent heart disease presenting with J wave and ST segment elevation in inferior leads. J Cardiovasc Electrophysiol 2000;11:844-848. 30) Sahara M, Sagara K, Yamashita T, Abe T, Kirigaya H, Nakada M, Iinuma H, Fu LT, Watanabe H. J wave and ST segment elevation in the inferior leads: a latent type of variant Brugada syndrome? Jpn Heart J 2002;43:55-60. 31) Riera AR, Ferreira C, Schapachnik E, Brugada syndrome with atypical ECG: downsloping ST-segment elevation in inferior leads. J Electrocardiol. 2004;37:101-104) ( Ogawa M, Kumagai K, Yamanouchi Y, et al.Spontaneous onset of ventricular fibrillation in Brugada syndrome with J wave and ST-segment elevation in the inferior leads. Heart Rhythm. 2005;2:97-99. 32) Arai M, Nakazawa K, Takagi A, Brugada syndrome-like ST-segment elevation increase exacerbated by vomiting. Circ J. 2004;68:712-714. 33) Viswanathan PC, Bezzina CR, George AL Jr, Roden DM, Wilde AA, Balser JR.Gating-dependent mechanisms for flecainide action in SCN5A- linked arrhythmia syndromes. Circulation 2001;104:1200-1205. 34) Priori SG, Napolitano C, Schwartz PJ, The elusive link between LQT3 and Brugada syndrome: the role of flecainide challenge. Circulation. 2000;102:945-947.] 35) Windle JR, Geletka RC, Moss AJ, Normalization of ventricular repolarization with flecainide in long QT syndrome patients with SCN5A:DeltaKPQ mutation. Ann Noninvasive Electrocardiol. 2001;6:153-158. 36) Shimizu W, Aiba T, Antzelevitch C. Specific therapy based on the genotype and cellular mechanism in inherited cardiac arrhythmias. Long QT syndrome and Brugada syndrome. Curr Pharm Des. 2005;11:1561-1572. 37) Antzelevitch C, Yan GX. Cellular and ionic mechanisms responsible for the Brugada syndrome. J Electrocardiol 2000;33 Suppl: 33-39. 38) Bettiol K, Gianfranchi L, Scarfo S et al.Successful treatment of electrical storm with oral quinidine in Brugada syndrome. Ital Heart J. 2005;6:601-602. 39) Marquez MF, Salica G, Hermosillo AG, et al. Drug therapy in Brugada syndrome. Curr Drug Targets Cardiovasc Haematol Disord. 2005;5:409-417. 40) Alings M, Dekker L, Sadee A, Wilde A. Quinidine induced electrocardiographic normalization in two patients with Brugada syndrome. Pacing Clin Electrophysiol 2001;24:1420-1422. 41) Haghjoo M, Arya A, Heidari A, Suppression of electrical storm by oral quinidine in a patient with Brugada syndrome. J Cardiovasc Electrophysiol. 2005;16:674. 42) Suzuki H, Torigoe K, Numata O, et al. Infant case with a malignant form of Brugada syndrome. J Cardiovasc Electrophysiol 2000;11:1277-1280. 43) Noda T, Shimizu W, Taguchi A, et al. ST-segment elevation and ventricular fibrillation without coronary spasm by intracoronary injection of acetylcholine and/or ergonovine maleate in patients with Brugada syndrome. J Am Coll Cardiol 2002; 40:1841-1847. 44) Tsuchiya T, Ashikaga K, Honda T, et al. Prevention of ventricular fibrillation by cilostazol, an oral phosphodiesterase inhibitor, in a patient with Brugada syndrome. J Cardiovasc Electrophysiol. 2002;13:698-701. 45) Miyazaki T, Mitamura H, Miyoshi S, et al. Autonomic and antiarrhythmic drug modulation of ST segment elevation in patients with Brugada syndrome.J Am Coll Cardiol. 1996;27:1061-1070. 46) Matsuo K, Yano K. Brugada syndrome Nippon Rinsho 2002;60:1408-1414. 47) Matsuo K, Shimizu W, Kurita T, et al.Dynamic changes of 12-lead electrocardiograms in a patient with Brugada syndrome. J Cardiovasc Electrophysiol 1998;9:508-512. 48) Hasbak P, Engelmann MD. Early repolarization. ST-segment elevation as a normal electrocardiographic variant] Ugeskr Laeger 2000;162:5928-5929. 49) Brady WJ, Chan TC. Electrocardiographic manifestations: benign early repolarization. J Emerg Med 1999;17:473-478. 50) Guo ZG, Peng J, Meng SR, Wang P. Differential diagnosis of early repolarization syndrome in patients with ST-segment elevation: report of 5 cases 2002; 22:1033. 51) Yang XC, Zhou P, Li CL. Electrical heterogeneity of canine right ventricular transient outward potassium currents.Chin Med J (Engl). 2004;117:528-531. 52) Mehta M, Jain AC, Mehta A. Early repolarization. Clin Cardiol 1999;22:59-65. All the best Andr?s Ricardo P?rez Riera. Chief of Electro-Vectocardiology Sector of the Discipline of Cardiology, ABC Faculty of Medicine (FMABC), Foundation of ABC (FUABC) - Santo Andr? - Sao Paulo - Brazil. Rua Sebastiao Afonso 885 - Zip Code: 044417-100- Jardim Miriam S.P Brazil -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee > > Induction of a type I Brugada pattern during febrile states has > been well > described. > > I have also observed a few cases where a Type I Brugada pattern was > observed > during or shortly after a strong vagal episode, such as severe > abdominal > pain , or a vasovagal episode, where the individual recovered quite > quickly > as with a vasovagal syncope, unlike a sudden cardiac arrest > situation. The > ECG subsequently reverted to normal or a Type II or III pattern on a > separate occasion. > > What is the mechanism of these observations and what is the > prognosis? If > EPS is performed, how often is VT/VF inducible? > > I would like to hear the opinion of the experts in this field. > > Sincerely > > > Dr Ruth Kam > Consultant Cardiologist and Cardiac Electrophysiologist > Ruth Kam Heart and Arrhythmia Clinic > #08-06, Mt Elizabeth Medical Centre > Singapore 228510 > > -- > Dr. Sergio Dubner > President of Scientific Committee > > Dr. Edgardo Schapachnik > President of Steering Committee > > > > > _______________________________________________ > Scd-forum mailing list > Scd-forum at scd-symposium.org > http://www.grupoakros.com.ar/mailman/listinfo/scd-forum -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061018/cce1fa29/attachment.html From info at scd-symposium.org Wed Oct 18 15:49:11 2006 From: info at scd-symposium.org (SCD Symposium) Date: Wed, 18 Oct 2006 15:49:11 -0300 Subject: [SCD-FORUM] 54R RE: Two questions about cardiac sudden death Dr. Iabluchanski In-Reply-To: <12527313-95B3-474F-BC47-98B3455F8C22@scd-symposium.org> References: <12527313-95B3-474F-BC47-98B3455F8C22@scd-symposium.org> Message-ID: ?Catecholaminergic storm? is an unspecific reaction from the organism that is determined at different levels and depends on the evolution of the myocardial infarction. If the infarction does not affect the main vital functions, mostly cardiac biomechanics (e.g. a well organized effect that does not affect the regulation pathways), then this reaction does not vary either. As regards to SD, it is a direct product from ?catecholaminergic storm? and behaves in the same manner. ?Catecholaminergic storm? always implies an increase in HR with a decrease in its dispersion; therefore, HR variability decreases and its spectrum is redistributed in lower rates area. For more information, I suggest my book (in Russian): http:// www.medicusamicus.com/index.php?action=im1-0 Sincerely, Dr. Nikolai Iabluchanski -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee > > 1. How to evaluate the predictive value of those non-invasive > examinations in our clinical work? > 2. For those 'Non-Acute Myocardial Infarction' patients, what's the > effect of the 'catecholamine storm' on sudden cardiac death? How to > evaluate it? > > "liu.xin.can" -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061018/b167b299/attachment.html From info at scd-symposium.org Wed Oct 18 22:39:42 2006 From: info at scd-symposium.org (SCD Symposium) Date: Wed, 18 Oct 2006 22:39:42 -0300 Subject: [SCD-FORUM] 58S RE: Some questions about Brugada Syndrome. Dr. Asensio In-Reply-To: <08A19966-633A-473A-BB8D-0CC5A3443026@scd-symposium.org> References: <08A19966-633A-473A-BB8D-0CC5A3443026@scd-symposium.org> Message-ID: Could Brugada type I pattern respond to changes derived from vagal shock and Bezold-Jarish type response of neurocardiogenic syncope, in which apparently, the main problem is in preload/venous return? Enrique Asensio L. M?xico -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee > > Induction of a type I Brugada pattern during febrile states has > been well > described. > > I have also observed a few cases where a Type I Brugada pattern was > observed > during or shortly after a strong vagal episode, such as severe > abdominal > pain , or a vasovagal episode, where the individual recovered quite > quickly > as with a vasovagal syncope, unlike a sudden cardiac arrest > situation. The > ECG subsequently reverted to normal or a Type II or III pattern on a > separate occasion. > > What is the mechanism of these observations and what is the > prognosis? If > EPS is performed, how often is VT/VF inducible? > > I would like to hear the opinion of the experts in this field. > > Sincerely > > > Dr Ruth Kam > Consultant Cardiologist and Cardiac Electrophysiologist > Ruth Kam Heart and Arrhythmia Clinic > #08-06, Mt Elizabeth Medical Centre > Singapore 228510 > > -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061018/a4f8aabe/attachment.html From info at scd-symposium.org Wed Oct 18 22:57:56 2006 From: info at scd-symposium.org (SCD Symposium) Date: Wed, 18 Oct 2006 22:57:56 -0300 Subject: [SCD-FORUM] 57S RE: Cardiorespiratory arrest and sudden cardiac death. Dr. Asensio In-Reply-To: <21A7F30F-B2BB-4B38-B48C-FB4F8931AAA6@scd-symposium.org> References: <21A7F30F-B2BB-4B38-B48C-FB4F8931AAA6@scd-symposium.org> Message-ID: From a certain point of view, cardiac arrest is the time of death, although we all know there is a margin between brain death, legally considered in many countries as "death" proper, and cardiac arrest or else, tachyarrythmias that condition low output. It would be necessary to differentiate also VT/VF from electrical activity with no pulse, or asystole as cause of death. We know that on many occasions, they are a continuum that we attempt to stop with different interventions that are variably successful according to the opportunity and efficacy. From my personal point of view, the difference may be more semantic than practical, due to the great amount of variables to take into account: do we consider cardiac arrest as the disappearance of pulse? However, many times there is VT or VF, why can we start CPR even when there are still slow pulses (escape rhythms or AV blocks in ECG)? Again we are involved in a discussion of terms very hard to define because of the number of pathophysiological concepts and mostly, due to the "dynamics" of events and their sequences. What do experts think? Enrique Asensio L. M?xico -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee > > Dear colleagues, > I am writing to this list because I would to have some concepts > clarified to me regarding the subject of discussion. > I have looked through several articles, books and journals, and > they are not clear in this sense. For instance: the Spanish > guidelines for CPR mention that there is a very narrow and > arbitrary limit between the concepts of cardiorespiratory arrest > and sudden cardiac death, leaving one for statistic limits, and the > cardiorespiratory arrest as a clinical approach of the problem. > My question for the experts: > Is there some way of defining both concepts, or do they overlap in > such a way that they can be used without distinction in related > studies and papers? > Other authors point out ?reanimation from sudden cardiac death?. > Could it be reanimation from a cardiac arrest? Is it not death > precisely that, ?death?? > > I hope you can clarify this dilemma for me, and thanking you in > advance, > > Dr. Oscar Ruiz Ropero > Intensivista > Hospital General Docente. Guantanamo. Cuba. > > > -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061018/d7ab763e/attachment.html From info at scd-symposium.org Thu Oct 19 11:27:18 2006 From: info at scd-symposium.org (SCD Symposium) Date: Thu, 19 Oct 2006 11:27:18 -0300 Subject: [SCD-FORUM] 59E RE: Brugada patient. Can he work? Dr. Perez Riera In-Reply-To: References: Message-ID: <10B4FD41-7DFD-4F54-9712-E3849D14E4A3@scd-symposium.org> Dear Marcellus Francis Ramirez from Mannila Philippines: Here Andr?s Ricardo P?rez Riera from Sao Paulo Brazil answer. I think that your patient has high risk of SCD. First dear colleague you live in a Country were the disease is endemic. In Philippines, the entity is known as Bangungut (wailing followed by SCD during sleep) The death certificate classification of sudden unexplained nocturnal deaths (SUDS) in Manila has changed considerably, obscuring an increase in incidence. SUDS appears to be a regional phenomenon in Southeast Asia and environmental causes are likely because the deaths are seasonal, increased over the timespan studied, and are more common among migrants to Manila than among those born there. A high incidence of SUND has been reported among young Asian males. These deaths are known as Pokkuri in Japan, Bangungut in the Philippines and SUND in the USA. SUNDS AND BRUGADA DISEASE ARE THE SAME DISEASE. You say that the patient has spontaneous ECG type 1 pattern and additionally he belongs to male gender. Male gender predicts a more malignant natural history. Asymptomatic individuals with a spontaneous abnormal type 1 ECG developed an arrhythmic event during a mean follow-up period of only 27?29 months. A "spontaneous pattern" is defined as an ECG showing the patterns established for the first European Consensus about the syndrome (1): presence of repolarization disorders occurred in the right precordial leads (V1 and V2) or in the anteroseptal wall (V1 to V3) with ST-segment elevation coved to the top ?coved type? or type 1A of my classification or rectilinear (type 1B of my classification) equal or mayor than 2mm (0.2mV), and followed by negative T wave (Brugada phenotype). See the classification http://www.scd-symposium.org/files/clasification.pdf The following are markers of a poor prognosis in BrS patients: 1) Patients with an a spontaneously abnormal ECG type 1 pattern (Types 1A or 1B); 2) Patients with inducibility of sustained ventricular arrhythmias at PES: concensus does not exist on the value of PES to identify the subjects with risk of spontaneous occurrence of VF. Brugada brothers think that theses patients should receive an ICD. (2-3) On the other hand, Priori et al from 200 patients using the life-table method of Kaplan-Meier used to define the cardiac arrest- free interval in patients undergoing PES failed to demonstrate an association between PES inducibility and spontaneous occurrence of VF (4) ; Eckardt et al studied during a mean follow-up of 40 months a numerous universe of a collaborative large cohort 212 individuals who presented Brugada type 1 electrocardiographic pattern, from which 125 (59%) was spontaneous, and the rest only after pharmacological test with a class I drug. The authors verified that 58% were asymptomatic; 31% had suffered >/= 1episodes of syncope with unknown origin and 11% had been resuscitated from a VF episode (aborted sudden death). A history of syncope or aborted sudden death was predictor of adverse outcome. The degree of elevation of the T segment was greater between symptomatic individuals: 2.3 mm higher than asymptomatic ones (mean 1.9 mm ???: I think that this is a byes of the manuscript because type 1 is 2mm). In the latter, it was observed that the incidence of events was very low, and PES had a very low accuracy in predicting evolution. This paper attempts to clarify the controversial issue, which still persists, between Priori's group and Brugada's group, regarding the predictive value of PES, agreeing with the former. The data regarding the risk of events in patients with BrS are controversial and depend on the cohort of patients studied. This collaborative paper describes long-term follow up of a large cohort of well-identified BrS patients as well as explores predictive value of PES. In contrary to some previous papers on the topic, in this study the authors could not demonstrate significant prognostic value of PES testing. The risk of arrhythmic events in asymptomatic patients is very low indicating that they could be considered as patients of much lesser risk than it was previously considered. This observation might have impact on both diagnostic triage and therapy approach in BrS patients (pharmacological approach). 3) Male gender predict a more malignant natural history; 4) Symptomatic patients: A history of syncope or aborted sudden death is predictor of adverse outcome. 5) Spontaneous ST-segment elevation in leads V1 through V3 combined with the history of syncope is a powerful marker to identify individuals who had cardiac arrest. 6) A spontaneous change in ST segment is associated with the highest risk for subsequent events in subjects with a Brugada-type 1 ECG. The presence of syncopal episodes, a history of familial sudden death, and/or LP may increase its value (6). 7) A history of syncope or SCD, the presence of a spontaneous Type 1 Brugada ECG, and male gender predict a more malignant natural history. The use of a family history of SCD, the presence of an SCN5A gene mutation, or EPS to guide the management of patients with a Brugada ECG is not supported(7); 8) A genetic defect on the SCN5A gene is not associated with a higher risk of events, suggesting that genetic analysis is a most useful diagnostic parameter but it is not helpful for risk stratification(8). The Brugada phenotype ECG) is much more prevalent than the manifest BrS. Although invasive electrophysiologic investigations have been proposed as a risk stratifier, their value is controversial, and alternative noninvasive techniques may be preferred. Ikeda et al (6) sought a noninvasive strategy to detect a high-risk group in a long-term follow-up study of subjects with a Brugada-type ECG, and no history of cardiac arrest. The study enrolled 124 consecutive subjects with a Brugada-type ECG. Prognostic indices included: age; sex, a family history of SCD, syncopal episodes, a spontaneous coved-type ST-segment elevation, maximal magnitude of ST- segment elevation, a spontaneous change in ST segment, a mean QRSd, maximal QT interval, QT dispersion, LPs by SA-ECG, and TWAs. Of the 124 subjects, 20 consenting subjects had an ICD before follow-up. During a 40 +/- 19-month follow-up, 12 subjects (9.7%) reached one of the endpoints (SCD or VT). Of the 12 risk indices, a family history of SCD, syncopal episodes, a spontaneous coved-type ST-segment elevation, a spontaneous change in ST segment, and LP had significant values. In multivariate analysis, a spontaneous change in ST segment had the most significance (a relative hazard, 9.2; P = 0.036). Combined assessment of this index and other significant indices obtained higher positive predictive values (43-71%). The authors concluded that a spontaneous change in ST segment is associated with the highest risk for subsequent events in subjects with a Brugada- type ECG. The presence of syncopal episodes, a history of familial sudden death, and/or LP may increase its value. References 1) Wilde AA, Antzelevitch C, Borggrefe M, Brugada J, Brugada R, Brugada P, Corrado D, Hauer RNm Kass RS, Nademanee K, Priori SG, Towbin JA. Proposed diagnostic criteria for the Brugada syndrome Eur Heart J 2002; 23:1648-1654. 2) Brugada P, Brugada R, Mont L, Rivero M, Geelen P, Brugada J. Natural history of Brugada syndrome: the prognostic value of Programmed electrical stimulation of the heart. J Electrophysiol 2003; 14: 455-457. 3) Brugada J, Brugada R, Brugada P. Right bundle-branch block and ST-segment elevation in leads V1 through V3: a marker for sudden death in patients without demonstrable structural heart disease. Circulation. 1998; 97: 457?460. 4) Priori SG, Napolitano C, Gasparini M, et al. Natural history of Brugada syndrome: insights for risk stratification and management. Circulation. 2002; 105: 1342-1347. 5) Eckardt L, Probst V, Smits JP, Bahr ES, Wolpert C, Schimpf R, Wichter T, Boisseau P, Heinecke A, Breithardt G, Borggrefe M, Lemarec H, Bocker D, Wilde AA. Long-Term Prognosis of Individuals With Right Precordial ST-Segment-Elevation Brugada Syndrome.Circulation. 2005; 111: 257-262; 111: 257-263. 6) Ikeda T, Takami M, Sugi K, Noninvasive risk stratification of subjects with a Brugada-type electrocardiogram and no history of cardiac arrest. Ann Noninvasive Electrocardiol. 2005; 10:396-403. 7) Gehi AK, Duong TD, Metz LD, et al. Risk stratification of individuals with the brugada electrocardiogram: a meta-analysis. J Cardiovasc Electrophysiol. 2006; 17:577-583. 8) Priori SG, Napolitano C, Gasparini M, et al. Natural history of Brugada syndrome: insights for risk stratification and management. Circulation. 2002; 105: 1342-1347. All the best Andr?s Ricardo P?rez Riera. Chief of Electro-Vectocardiology Sector of the Discipline of Cardiology, ABC Faculty of Medicine (FMABC), Foundation of ABC (FUABC) - Santo Andr? - Sao Paulo - Brazil. Rua Sebastiao Afonso 885 - Zip Code: 044417-100- Jardim Miriam S.P Brazil- Phone: 5504-6243 Fax: 5506-0398 El 16/10/2006, a las 15:14, SCD Symposium escribi?: > Forum of the ISHNE Sudden Cardiac Death World-Wide Internet Symposium > ______________________________________________________________________ > > Medtronic's free physician resource center: > http://www.medtronicconnect.com > ______________________________________________________________________ > > Congratulations and more power to the organizers of > this excellent symposium. > I would like to ask your expert opinion about a recent > patient I encountered: a male in his mid 30s with type > 1 brugada ecg pattern, negative EPS for inducible VT > and no family history of sudden death. The said > patient is applying as a seaman (works as a ship crew > member). Can this patient be cleared for employment in > such occupation without risks? > > MARCELLUS FRANCIS RAMIREZ > Universty of Santo Tomas > Manila, Philippines > > -- > Dr. Sergio Dubner > President of Scientific Committee > > Dr. Edgardo Schapachnik > President of Steering Committee > > > > > _______________________________________________ > Scd-forum mailing list > Scd-forum at scd-symposium.org > http://www.grupoakros.com.ar/mailman/listinfo/scd-forum -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061019/2ba4e77a/attachment.html From info at scd-symposium.org Thu Oct 19 11:57:28 2006 From: info at scd-symposium.org (SCD Symposium) Date: Thu, 19 Oct 2006 11:57:28 -0300 Subject: [SCD-FORUM] READING THE LECTURE BY DR. LEXIN WANG.THREE EXPERT QUESTIONS BY DR. MERINO Message-ID: Left cardiac sympathectomy to manage beta-blocker resistant LQT patients Lexin Wang Dr. Jos? Luis Merino (Spain) - LCSD should be considered in all types of long QT syndrome (LQTS) or there are specific types (i.e. LQTS 3) which are specially adequate or inadequate for it? Dr. Lexin Wang (Australia) There is limited knowledge about the therapeutic effects LCSD and genotypes of LQTS. In most reported cases including my own, LCSD was performed in patients who failed to respond to full dose beta- blockers, regardless of the types of LQTS. One interesting observation, however, is that LCSD has no significant effect on resting heart rate. This is probably an advantage for treating LQT3 patients who often have a disproportionally prolonged QT interval (and risk of SCD) at a lower heart rate during beta-blocker therapy. Dr. Jos? Luis Merino (Spain) - LCSD should be limited to a few centers with high experience in the treatment of this patients or is a technique which can be easily implemented and performed in regular centers? Dr. Lexin Wang (Australia) - The surgical techniques for LCSD have evolved significantly over the recent years. The development of video-assisted endoscopic LCSD (Li J, et al. PACE 2003; 26:870-873) means that the procedure can be performed by thoracic surgeons who are familiar with the anatomy of left cardiac sympathetic ganglion in a very short period of time (20-30 min in our experiences) with little complication or blood loss. Patients can be discharged 2-3 days after the surgery. It does, however, require close collaboration between cardiologists and surgeons in terms of pre- and post-surgical care and follow-ups. Dr. Jos? Luis Merino (Spain) - Do you consider LCSD without ICD implantation in any patient? Dr. Lexin Wang (Australia) - This is an excellent but rather complex question. In my cohort of patients who underwent LCSD, ICD was not an option becasue of financial constraints. None of them received an ICD after the LCSD but they were doing very well 3-4 years after the surgery. On the other hand, patients who have already received an ICD, LCSD would substantially reduce the number of shocks, improving the longevity of the ICD and patients' quality of life. As to do we need to consider ICD after LCSD, I would suggest that we have a debate on this throughout the course of the symposium. My answer to this question is that it will depend on the risk of SCD, access to the ICD devices and quality of life considerations. -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061019/28b4dd05/attachment.html From info at scd-symposium.org Thu Oct 19 21:04:44 2006 From: info at scd-symposium.org (SCD Symposium) Date: Thu, 19 Oct 2006 21:04:44 -0300 Subject: [SCD-FORUM] 63C An Unusual QRST Pattern in A Patient with Severe Hypokalemia. Dr. Wang Message-ID: <6E78CCEE-F432-46C5-ACA1-E331A164A35A@scd-symposium.org> An Unusual QRST Pattern in A Patient with Severe Hypokalemia A 31-year-old male was admitted with muscle weakness of the extremities, paralysis and dyspnea. The patient felt tired for 12 hours with nasal discharge, nausea and one episode of vomiting, but had no fever or cough. The patient had been sent to a local clinic where he received IV hydration. His muscle weakness got worse and he developed paralysis 6 hours before the admission. In the past, the patient was in good general health. There was no history of allergy to mediation or food. He denied history of hepatitis or tuberculosis. There was no history of contact with industrial poison and radioactive substances. There was no history of surgery or trauma. Physical examination: T: 36.4?C,P: 66/min,R: 20/min,BP: 130/60 mmHg. The patient was in no acute distress. There was exophthalmos bilaterally. The thyroid was enlarged and felt to be somewhat solid, with normal mobility but no flow murmur. Lungs were clear. Heart showed some irregularity, but no murmur. Physical reflexes were within the normal range, but the extremity muscle strength was zero degree. Muscular tension decreased in the arms, but increased in the legs. Stat electrocardiogram (EKG) showed Sinus arrhythmia with a rate of 61/min. The P-R, QRS and Q-T intervals measured 260, 120 and 360 ms, respectively. QU interval=670 ms. There was an rSr? pattern of the QRS complex in leads V1 through V3, with r < r?. There was ST segment downsloping elevation of 0.2 mV with inverted T wave in leads V1 and V2. T wave was flat in lead V3. There was an Rs pattern with a tall R wave (3.8 mV) and a late broad S wave in lead V5. There were prominent U waves in all the leads,more so in the precordial leads. http://www.scd-symposium.org/files/wang1.pdf EKG diagnose:1) Sinus arrhythmia; 2) Io atrioventricular block; 3) High voltage of the left ventricle; 4) Prominent U wave and QU prolongation suggesting hypokalemia. 5) Atypucal Brugada ECG pattern. Stat blood test revealed K+ was only 1.46 mmol/l,which was improved to 4.46mmol/l with potassium supplement over 24 hours. A follow up EKG showed:Normal sinus rhythm with a rate of 83/min. The P-R, QRS and Q-T intervals measured 160ms, 80 and 380 ms, respectively. There remained an rSr? pattern of the QRS complex in leads V1 and V2,but r> r?. The latter mimic a J wave, with a much lower amplitude when compared to the prior electrocardiogram. There remained ST segment downsloping elevation in leads V1 and V2 (0.1?0.2mv) and T wave inversion in lead V1. But there was no longer T wave inversion in leads V2 or V3. An upright R wave was appreciated in V3 as well as in leads V4 and V5. There was a qRs pattern in lead V5, with a prominent J wave. The R wave remained to be tall in V5 (3.9mV). The u wave was no longer present in any leads. http://www.scd-symposium.org/files/wang2.pdf EKG diagnosis:1) Normal sinus rhythm. 2) High voltage of the left ventricle; 3) Atypical Brugada EKG pattern. The patient was subsequetly found to have elevated T3 and T4, therefore the final clinical diagnosis was ?thyrotoxicosis with periodic paralysis?. Questions to the ECG expert Dr. Perez Riera: 1) Do you consider whether this patient has a Brugada pattern or not? 2) Want is your interpretation of the ECG, especially considering the unusual QRST pattern in the right precordial leads? Thank you, Hongyu Wang, MD Jiangping Li, MD Second Hospital of Shanxi Medical University PR. China -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061019/cc20acbc/attachment.html From info at scd-symposium.org Fri Oct 20 12:04:51 2006 From: info at scd-symposium.org (SCD Symposium) Date: Fri, 20 Oct 2006 12:04:51 -0300 Subject: [SCD-FORUM] EXPERTS ASK, EXPERTS ANSWER Message-ID: Dr. Leonid Makarov from Russia asks - If there was a need to perform ECG screening in the healthy population to detect patients in high risk of noncoronary arrhythmogenic sudden death, which and how many electrocardiographic signs would you use for this purpose? Dr. Wojciech Zareba from U.S.A. answers - Identification of the SCD risk in general population is of major interest and there is no single test that could be considered as a gold standard due to a complexity of mechanisms leading to SCD. ECG screening is an attractive and easily obtained option. In particular, a standard 12-lead ECG could be considered useful. Evaluating step-by- step prognostic information coming from an ECG, one has to focus first on: 1. Rhythm ? presence of non-sinus rhythm in otherwise healthy population is rather infrequent, nevertheless, in cohorts of elderly healthy it is likely to find at least few percent of subjects with undiagnosed atrial fibrillation. Atrial fibrillation is first risk factor which is associated with an increased (at least 2-fold) risk of cardiac death and increased risk of SCD. 2. Rate of heart rate ? tendency to resting tachycardia, especially >80bpm usually indicates additional underlying medical problem and should also be considered as a risk factor. The association between elevated resting heart rate and mortality is observed in postinfarction patients and patients with heart failure, but data on healthy subjects are less convincing. Increased heart rate should prompt physicians to investigate further the reason for this finding. 3. QRS ? conduction abnormalities, namely left bundle branch block, are associated with increased risk of cardiac events and SCD in healthy cohorts with hazard ratios >2.0. Data on RBBB do not show significant association with mortality. QRS prolongation is associated with increased risk of death and SCD in healthy cohorts. LVH is of lesser prognostic importance assuming that QRS duration is analyzed. 4. QT-T wave ? repolarization parameters are considered as an important marker of increased risk in healthy subjects. Elevated QTc (>0.44 sec) in healthy cohort, especially in elderly subjects, was reported to be associated with significantly increased risk of cardiac death and SCD. Abnormal T wave morphology (usually inverted or flat T wave or abnormal T wave axis) also serves as a sign of increased risk for cardiac death and SCD in cohorts of healthy subjects. Therefore patients with atrial fibrillation and those in sinus rhythm with wide QRS complex (LBBB) or patients with abnormal repolarization (either prolonged QTc or abnormal T wave morphology) are at increased risk of cardiac death and SCD. Such subjects identified in the screening process require further attention to elucidate causes of the abnormalities and to consider preventive/therapeutic measures. It is worth adding that exercise ECG testing with analysis of heart rhythm recovery (difference between heart rate at peak exercise and heart rate at 1-minute recovery) seems to be useful in identifying high-risk healthy individuals. The heart rate recovery <12bpm was reported as indicator of SCD in healthy cohorts. Electrocardiology has a limited option in identifying patients with a vulnerable plaque, and ischemic incidents are the major causes of SCD. Therefore, the above screening should be combined with additional measures reflecting an increased risk of atherosclerotic changes including lipid profile and some inflammatory markers (sedimentation rate, CRP, etc?) while we are awaiting novel more specific markers of plaque vulnerability. -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061020/6bf7cea9/attachment.html From info at scd-symposium.org Fri Oct 20 12:26:26 2006 From: info at scd-symposium.org (SCD Symposium) Date: Fri, 20 Oct 2006 12:26:26 -0300 Subject: [SCD-FORUM] 61S Brugada's pattern detection in a son of SCD's patient. Dr. Cagnalotti Message-ID: Dear Sergio, About a case of reanimation of sudden death in a 54-year-old patient, with no structural heart disease, with all the studies made: coronary angiography, MNR, Holter, EPS. Everything appears to be normal. Monomorphic arrhythmic storm is detected at the moment the patient is admitted. The patient is provided life support and he recovers in 24 hs. ICD implantation is decided. After three months he displays three shocks by arrhythmic storm, incessant ventricular tachycardia. Mapping and focus ablation is decided, in outflow tract with mismatch. To this moment, the patient has not displayed any event. It's been three months, and in a Holter register in one of his sons, typical Brugada pattern is observed. The patient is 17 years old, asymptomatic. I cannot perform pharmacological test. What should I do next? Dr. Cagnolatti -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061020/05a95927/attachment.html From info at scd-symposium.org Fri Oct 20 12:45:13 2006 From: info at scd-symposium.org (SCD Symposium) Date: Fri, 20 Oct 2006 12:45:13 -0300 Subject: [SCD-FORUM] 60S RE: Cardiorespiratory arrest and sudden cardiac death. Dr. Sanchez Verduzco In-Reply-To: References: <21A7F30F-B2BB-4B38-B48C-FB4F8931AAA6@scd-symposium.org> Message-ID: <9DC5866D-B844-4CDA-9D1E-2B9135BBFE2D@scd-symposium.org> I can't see hardly any complication, not even semantic, in what we consider cardiac arrest or better, cardiorespiratory arrest, whichever the cause. We may simply consider that any patient that requires cardiorespiratory reanimation is because he/she has: lack of effective cardiac output. Dr. Miguel Angel Sanchez Verduzco medicina intensiva. Guadalajara jalisco M?xico -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee > > From a certain point of view, cardiac arrest is the time of death, > although we all know there is a margin between brain death, legally > considered in many countries as "death" proper, and cardiac arrest > or else, tachyarrythmias that condition low output. It would be > necessary to differentiate also VT/VF from electrical activity with > no pulse, or asystole as cause of death. We know that on many > occasions, they are a continuum that we attempt to stop with > different interventions that are variably successful according to > the opportunity and efficacy. > From my personal point of view, the difference may be more semantic > than practical, due to the great amount of variables to take into > account: do we consider cardiac arrest as the disappearance of > pulse? However, many times there is VT or VF, why can we start CPR > even when there are still slow pulses (escape rhythms or AV blocks > in ECG)? Again we are involved in a discussion of terms very hard > to define because of the number of pathophysiological concepts and > mostly, due to the "dynamics" of events and their sequences. What > do experts think? > > Enrique Asensio L. > M?xico > > -- > Dr. Sergio Dubner > President of Scientific Committee > > Dr. Edgardo Schapachnik > President of Steering Committee > > >> >> Dear colleagues, >> I am writing to this list because I would to have some concepts >> clarified to me regarding the subject of discussion. >> I have looked through several articles, books and journals, and >> they are not clear in this sense. For instance: the Spanish >> guidelines for CPR mention that there is a very narrow and >> arbitrary limit between the concepts of cardiorespiratory arrest >> and sudden cardiac death, leaving one for statistic limits, and >> the cardiorespiratory arrest as a clinical approach of the problem. >> My question for the experts: >> Is there some way of defining both concepts, or do they overlap in >> such a way that they can be used without distinction in related >> studies and papers? >> Other authors point out ?reanimation from sudden cardiac death?. >> Could it be reanimation from a cardiac arrest? Is it not death >> precisely that, ?death?? >> >> I hope you can clarify this dilemma for me, and thanking you in >> advance, >> >> Dr. Oscar Ruiz Ropero >> Intensivista >> Hospital General Docente. Guantanamo. Cuba. >> -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061020/5e64c3e9/attachment.html From info at scd-symposium.org Fri Oct 20 14:42:54 2006 From: info at scd-symposium.org (SCD Symposium) Date: Fri, 20 Oct 2006 14:42:54 -0300 Subject: [SCD-FORUM] 65E ECG predictors of SCD. Dr. Madias Message-ID: <4490731C-9784-4D4C-9313-621243915579@scd-symposium.org> Dear colleagues: Greetings!!! Big Ccongratulations to the Organizers (!!!), for this magificent symposium, a scientific interaction, to which we have started becoming addictive. Regarding the comment of Dr. Andriy Vorotniak, on the "palpitations" and Brugada syndrome, and his suggestion that we should work toward compiling a list of ECG predictors of SCD, for us cardiologists and physicians working in the ambulatory medicine setting, I am afraid that many od such criteria may turn out to have a low sensitivity for SCD. Besides, we have not as yet resolved the problem of the marked variability in the precordial component of serial ECGs of individual patients, as it is apparent nowadays in hospital elecronic ECG files. Routine perusal of serial ECGs of patients evaluated in "consult rounds" discloses massive differences from ECG to ECG in the amplitude and morphology of V1-V6 leads. How one could rely on ECG predictors of SCD (often depending on the precordial leads) when the eproducibility is so poor? In addition experience has alrready been published that for Brugada stndrome the characteristic type I pattern was seen in some patients only when, other than the conventional V1-V3) thoracic electrode positioning was employed, something not expected to be available in routine standard ECG recordings. I beleive we should start from insisting that the ECGs obtained in ALL our patients are recorded appropriately, so that at least we resolve the problem of the poor reproducibility in serial ECG recordingsin the same patients. This is a prerequisite befoer we start delving into the matter of "ECG predictors of SCD". Warmest Regards to ALL Sincerely, John E. Madias, MD, FACC, FAHA, Professor of Medicine (Cardiology) Mount Sinai School of Medicine of the New York University, Cardiology Division, Elmhurst Hospital Center e-mail::madias at nychhc.org -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061020/1ffb6267/attachment.html From info at scd-symposium.org Fri Oct 20 14:46:16 2006 From: info at scd-symposium.org (SCD Symposium) Date: Fri, 20 Oct 2006 14:46:16 -0300 Subject: [SCD-FORUM] 64E RE: An Unusual QRST Pattern in A Patient with Severe Hypokalemia. Dr. Perez Riera In-Reply-To: <6E78CCEE-F432-46C5-ACA1-E331A164A35A@scd-symposium.org> References: <6E78CCEE-F432-46C5-ACA1-E331A164A35A@scd-symposium.org> Message-ID: <150E6691-A783-4E3B-9137-507A637ACB08@scd-symposium.org> Dear colleague from China: This nice case report is a very interesting presentation of acquired forms of the Brugada syndrome (BrS) Both Hypokalemia and Hyperkalemia increases transient outward current in phase1, adenosine triphosphate-sensitive potassium current, delayed modifier potassium current or decreases inward currents: L-type calcium current, fast Na+ current at the end of phase 1 of the AP can accentuate or unmask ST-segment elevation, similar to that found in the BrS, thus producing acquired forms (1). Drugs that reduce the Na+ current may precipitate the BrS ECG type 1 pattern: Na+ channel blockers and membrane depolarization by Hyperkalemia. Hypokalemia increases the risk of paroxysmal atrial fibrillation in BrS (2). Hyperkalemia secondary to chronic renal failure may cause ECG changes mimicking the BrS (3). See the case in http://www.scd-symposium.org/files/Hyperkalemia.ppt Propofol Infusion Syndrome (PRIS): entity characterized by association of metabolic acidosis, rhabdomyolysis, hyperkalemia, and SCD after long-term, high-dose propofol infusion is other cause related of acquired BrS (4). Finally, hyperkalemia induced by overdose of diphenhydramine is other cause of acquired form of BrS (5). References 1) Shimizu W. Acquired forms of the Brugada syndrome. J Electrocardiol. 2005;38:22-25. 2) Notarstefano P, Pratola C, Toselli T, et al. Atrial fibrillation and recurrent ventricular fibrillation during hypokalemia in Brugada syndrome. Pacing Clin Electrophysiol. 2005;28:1350-1353. 3) Ortega-Carnicer J, Benezet J, Ruiz-Lorenzo F, Alcazar R. Transient Brugada-type electrocardiographic abnormalities in renal failure reversed by dialysis. Resuscitation. 2002;55:215-219 4) Vernooy K, Delhaas T, Cremer OL,.Electrocardiographic changes predicting sudden death in propofol-related infusion syndrome. Heart Rhythm. 2006;3:131-137 5) Lopez-Barbeito B, Lluis M, Delgado V, et al. Diphenhydramine overdose and Brugada sign. Pacing Clin Electrophysiol. 2005;28:730-732. All the best Andr?s Ricardo P?rez Riera Chief of Electro-Vectocardiology Sector of the Discipline of Cardiology, ABC Faculty of Medicine (FMABC), Foundation of ABC (FUABC) - Santo Andr? - Sao Paulo - Brazil. Rua Sebastiao Afonso 885 - Zip Code: 044417-100- Jardim Miriam S.P Brazil > > An Unusual QRST Pattern in A Patient with Severe Hypokalemia > A 31-year-old male was admitted with muscle weakness of the > extremities, paralysis and > dyspnea. The patient felt tired for 12 hours with nasal discharge, > nausea and one episode of > vomiting, but had no fever or cough. The patient had been sent to a > local clinic where he > received IV hydration. His muscle weakness got worse and he > developed paralysis 6 hours > before the admission. > In the past, the patient was in good general health. There was no > history of allergy to > mediation or food. He denied history of hepatitis or tuberculosis. > There was no history of > contact with industrial poison and radioactive substances. There > was no history of surgery or > trauma. > Physical examination: T: 36.4?C,P: 66/min,R: 20/min,BP: 130/60 > mmHg. The patient > was in no acute distress. There was exophthalmos bilaterally. The > thyroid was enlarged and > felt to be somewhat solid, with normal mobility but no flow murmur. > Lungs were clear. Heart > showed some irregularity, but no murmur. Physical reflexes were > within the normal range, > but the extremity muscle strength was zero degree. Muscular tension > decreased in the arms, > but increased in the legs. > Stat electrocardiogram (EKG) showed Sinus arrhythmia with a rate of > 61/min. The P-R, > QRS and Q-T intervals measured 260, 120 and 360 ms, respectively. > QU interval=670 ms. > There was an rSr? pattern of the QRS complex in leads V1 through > V3, with r < r?. There was > ST segment downsloping elevation of 0.2 mV with inverted T wave in > leads V1 and V2. T > wave was flat in lead V3. There was an Rs pattern with a tall R > wave (3.8 mV) and a late > broad S wave in lead V5. There were prominent U waves in all the > leads,more so in the > precordial leads. > > http://www.scd-symposium.org/files/wang1.pdf > > > EKG diagnose:1) Sinus arrhythmia; 2) Io atrioventricular block; 3) > High > voltage of the left ventricle; 4) Prominent U wave and QU > prolongation suggesting > hypokalemia. 5) Atypucal Brugada ECG pattern. > > Stat blood test revealed K+ was only 1.46 mmol/l,which was improved > to 4.46mmol/l > with potassium supplement over 24 hours. A follow up EKG > showed:Normal sinus rhythm > with a rate of 83/min. The P-R, QRS and Q-T intervals measured > 160ms, 80 and 380 ms, > respectively. There remained an rSr? pattern of the QRS complex in > leads V1 and V2,but r> > r?. The latter mimic a J wave, with a much lower amplitude when > compared to the prior > electrocardiogram. There remained ST segment downsloping elevation > in leads V1 and V2 > (0.1?0.2mv) and T wave inversion in lead V1. But there was no > longer T wave inversion in > leads V2 or V3. An upright R wave was appreciated in V3 as well as > in leads V4 and V5. There > was a qRs pattern in lead V5, with a prominent J wave. The R wave > remained to be tall in V5 > (3.9mV). The u wave was no longer present in any leads. > > http://www.scd-symposium.org/files/wang2.pdf > > EKG diagnosis:1) Normal sinus > rhythm. 2) High voltage of the left ventricle; 3) Atypical Brugada > EKG pattern. > The patient was subsequetly found to have elevated T3 and T4, > therefore the final clinical > diagnosis was ?thyrotoxicosis with periodic paralysis?. > Questions to the ECG expert Dr. Perez Riera: > 1) Do you consider whether this patient has a Brugada pattern or > not? > 2) Want is your interpretation of the ECG, especially > considering the unusual QRST > pattern in the right precordial leads? > Thank you, > Hongyu Wang, MD > Jiangping Li, MD > Second Hospital of Shanxi Medical University > PR. China -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061020/bc214b8c/attachment.html From info at scd-symposium.org Fri Oct 20 14:53:07 2006 From: info at scd-symposium.org (SCD Symposium) Date: Fri, 20 Oct 2006 14:53:07 -0300 Subject: [SCD-FORUM] 66E RE: Brugada's pattern detection in a son of SCD's patient. Dr. Ramon Brugada In-Reply-To: References: Message-ID: <04C86DC3-7BAE-4D95-8295-4B98D58752BC@scd-symposium.org> If type I ecg is present, pharmacologic test is not indicated, the diagnosis is made. Some investigators will go ahead and implant a defibrillator, because of the family history Others will wait until he develops any symptoms I recommend an EPS to risk stratify. If positive then ICD implant. Ramon Brugada MD FACC Associate Professor of Medicine Canadian Research Chair Genetics of Arrhythmias University of Montreal Director Clinical Cardiovascular Genetics Center Montreal Heart Institute 5000 Rue Belanger Montreal, QC H1T 1C8 Canada ramon at brugada.org > > Dear Sergio, > About a case of reanimation of sudden death in a 54-year-old > patient, with no structural heart disease, with all the studies > made: coronary angiography, MNR, Holter, EPS. > Everything appears to be normal. Monomorphic arrhythmic storm is > detected at the moment the patient is admitted. The patient is > provided life support and he recovers in 24 hs. ICD implantation is > decided. After three months he displays three shocks by arrhythmic > storm, incessant ventricular tachycardia. Mapping and focus > ablation is decided, in outflow tract with mismatch. > To this moment, the patient has not displayed any event. It's been > three months, and in a Holter register in one of his sons, typical > Brugada pattern is observed. The patient is 17 years old, > asymptomatic. I cannot perform pharmacological test. What should I > do next? > > Dr. Cagnolatti > > -- > Dr. Sergio Dubner > President of Scientific Committee > > Dr. Edgardo Schapachnik > President of Steering Committee > > > > > _______________________________________________ > Scd-forum mailing list > Scd-forum at scd-symposium.org > http://www.grupoakros.com.ar/mailman/listinfo/scd-forum -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061020/daa60aa3/attachment.html From info at scd-symposium.org Fri Oct 20 16:44:55 2006 From: info at scd-symposium.org (SCD Symposium) Date: Fri, 20 Oct 2006 16:44:55 -0300 Subject: [SCD-FORUM] 68E RE: Brugada's pattern detection in a son of SCD's patient. Dr. Haghjoo In-Reply-To: References: Message-ID: Dear Dr. Cagnolatti, In a patient with typical Brugada type ECG, there is no need for performing pharmacological challenge test. According to clinical data provided, there are no signs of brugada syndrome in patient's father (negative family history). Therefore, it is better to risk stratify patient by EPS. Majid Haghjoo,MD Department of Pacemaker and Electrophysiology Rajaie Cardiovascular Medical and Research Center Tehran, Iran -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee > > Dear Sergio, > About a case of reanimation of sudden death in a 54-year-old > patient, with no structural heart disease, with all the studies > made: coronary angiography, MNR, Holter, EPS. > Everything appears to be normal. Monomorphic arrhythmic storm is > detected at the moment the patient is admitted. The patient is > provided life support and he recovers in 24 hs. ICD implantation is > decided. After three months he displays three shocks by arrhythmic > storm, incessant ventricular tachycardia. Mapping and focus > ablation is decided, in outflow tract with mismatch. > To this moment, the patient has not displayed any event. It's been > three months, and in a Holter register in one of his sons, typical > Brugada pattern is observed. The patient is 17 years old, > asymptomatic. I cannot perform pharmacological test. What should I > do next? > > Dr. Cagnolatti > -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061020/b70fac6c/attachment.html From info at scd-symposium.org Fri Oct 20 17:49:36 2006 From: info at scd-symposium.org (SCD Symposium) Date: Fri, 20 Oct 2006 17:49:36 -0300 Subject: [SCD-FORUM] 69E RE: Cardiorespiratory arrest and sudden cardiac death. Dr. Perez Riera In-Reply-To: <9DC5866D-B844-4CDA-9D1E-2B9135BBFE2D@scd-symposium.org> References: <21A7F30F-B2BB-4B38-B48C-FB4F8931AAA6@scd-symposium.org> <9DC5866D-B844-4CDA-9D1E-2B9135BBFE2D@scd-symposium.org> Message-ID: <1399A135-A510-4B23-946C-A65DC689DC8F@scd-symposium.org> Dear Miguel Angel Sanchez Verduzco from Guadalajara Jalisco Mexico. Here Andr?s Ricardo P?rez Riera from Sao Paulo Brazil answer. Sudden death (SD) is defined as a death that occurs suddenly, develops during an unpredictable course, and is due to natural or unnatural causes. Although there is no universally standardized definition on how "sudden" a SD is, WHO defines SD as a death that occurs within 24 hours after the onset of symptoms. Sudden Cardiac Death (SCD) is defined as unexpected natural death due to cardiac causes, heralded by abrupt loss of consciousness within one hour after the onset of symptoms in a person without any prior condition that would appear fatal(1). SCD is the sudden, abrupt loss of heart function (ie, cardiac arrest) in a person who may or may not have been diagnosed with heart disease. The time and mode of death are unexpected. It occurs instantly or shortly after the onset of symptoms. Out-of-hospital SCD is a frequent cause of death. Survival rates remain low despite increasing efforts in medical care. Recently information on cases of out-of-hospital SCD was collected in the Berlin, Germany, and emergency medical system via a questionnaire by Muller et al.(2) Bystander interviews were performed by the emergency physician on scene immediately after declaration of death or return of circulation. Of 5831 rescue missions, 406 involved patients with presumed cardiac arrest. 66% had a known cardiac disease. In 72%, the arrest occurred at home, and in 67%, it occurred in the presence of an eyewitness. Information on symptoms immediately preceding the arrest was available in 80% of all 406 patients. Typical angina was present for a median of 120 minutes in 25% of the 274 patients with witnessed arrest and in 33% with a symptom duration of less than 1 hour. The authors conclude that SCD occurs most often at home in the presence of relatives and after a longer period of typical warning symptoms. Although the much-hailed use of public access external defibrillation is supported by several studies, the present results raise the question of whether educational measures and targeted educational programs tailored for patients at risk and their relatives should have a higher priority. SCD is a dramatic and/or spontaneous death that is thought to be (and usually is) caused by a heart condition and may have been brought on by exercise. The mechanisms of SCD are the following: 1) VF; 2) VT and ventricular flutter with subsequent VF; 3) TdP followed by VF; 4) Bradyarrhythmias and 5) Asystolic arrest. White the main risk factor is the presence of coronary artery disease (CAD), any organic or functional disease of the heart can predispose for SCD. SCD (also called sudden cardiac arrest (SCA) or cardiovascular collapse,) is death resulting from an abrupt loss of heart function. The victim may or may not have diagnosed heart disease. The time and mode of death are unexpected. It occurs within 60 minutes after symptoms appear in a person with known or unknown cardiac disease in whom no previously diagnosed fatal condition is apparent. The most common underlying reason for patients to die suddenly from SCA is CHD. Approximately half of all cardiac deaths can be classified as SCDs. SCD occurs as the first expression of cardiac disease in many individuals presenting as out-of-hospital patients with SCA. To evaluate the risk of SCD noninvasive (Holter, echocardiography, exercise test, SA-ECG, MTWA, and often invasive PES tests are necessary. The therapy is based on drugs (mainly beta blockers and amiodarone), coronary revascularization, catheter ablation techniques and the implantation of a CDI. The latter appears to be the most promising approach(3). Sudden Arrhythmia Death Syndrome (SADS) In about 1 in every 20 cases of SCD, no definite cause of death can be found, even after the heart has been examined by an expert cardiac pathologist. This is then called Sudden Arrhythmic Death Syndrome or SADS. (In the past it has also been called Sudden Adult Death Syndrome or Sudden Death Syndrome but, because it affects children too, the term Sudden Arrhythmic Death Syndrome is now used.) It is thought that cot death (Sudden Infant Death Syndrome, or SIDS) may be partly due to the same causes responsible for SADS. SADS is a disorder of the electrical system of the heart that can lead to the death of apparently healthy people without any warning. The condition is caused by dysfunction ion channels. These channels control the flow of ions like Ca++, Na+ and K+. The flow of these ions in and out of the cells produces the electrical activity of the heart. Abnormalities of these channels can be acquired or inherited. The acquired form is caused by certain medications or physiopatological conditions. References 1) Zheng ZJ, Croft JB, Giles WH, et al. Sudden cardiac death in the United States, 1989 to 1998. Circulation. 2001; 104: 2158?2163. 2) Muller D, Agrawal R, Arntz HR. How sudden is sudden cardiac death? Circulation. 2006; 114:1146-1150. 3) Evequoz D, Zuber M, Erne P.Sudden cardiac death: definition, mechanisms and risk factors Synonyms and related keyword: sudden arrest: Schweiz Rundsch Med Prax. 1996; 85:188-196. All the best Andr?s Ricardo P?rez Riera Chief of Electro-Vectocardiology Sector of the Discipline of Cardiology, ABC Faculty of Medicine (FMABC), Foundation of ABC (FUABC) - Santo Andr? - Sao Paulo - Brazil. Rua Sebastiao Afonso 885 - Zip Code: 044417-100- Jardim Miriam S.P Brazil -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee > > I can't see hardly any complication, not even semantic, in what we > consider cardiac arrest or better, cardiorespiratory arrest, > whichever the cause. We may simply consider that any patient that > requires cardiorespiratory reanimation is because he/she has: lack > of effective cardiac output. > > Dr. Miguel Angel Sanchez Verduzco > medicina intensiva. Guadalajara jalisco M?xico >> >> From a certain point of view, cardiac arrest is the time of death, >> although we all know there is a margin between brain death, >> legally considered in many countries as "death" proper, and >> cardiac arrest or else, tachyarrythmias that condition low output. >> It would be necessary to differentiate also VT/VF from electrical >> activity with no pulse, or asystole as cause of death. We know >> that on many occasions, they are a continuum that we attempt to >> stop with different interventions that are variably successful >> according to the opportunity and efficacy. >> From my personal point of view, the difference may be more >> semantic than practical, due to the great amount of variables to >> take into account: do we consider cardiac arrest as the >> disappearance of pulse? However, many times there is VT or VF, why >> can we start CPR even when there are still slow pulses (escape >> rhythms or AV blocks in ECG)? Again we are involved in a >> discussion of terms very hard to define because of the number of >> pathophysiological concepts and mostly, due to the "dynamics" of >> events and their sequences. What do experts think? >> >> Enrique Asensio L. >> M?xico >> >> >>> >>> Dear colleagues, >>> I am writing to this list because I would to have some concepts >>> clarified to me regarding the subject of discussion. >>> I have looked through several articles, books and journals, and >>> they are not clear in this sense. For instance: the Spanish >>> guidelines for CPR mention that there is a very narrow and >>> arbitrary limit between the concepts of cardiorespiratory arrest >>> and sudden cardiac death, leaving one for statistic limits, and >>> the cardiorespiratory arrest as a clinical approach of the problem. >>> My question for the experts: >>> Is there some way of defining both concepts, or do they overlap >>> in such a way that they can be used without distinction in >>> related studies and papers? >>> Other authors point out ?reanimation from sudden cardiac death?. >>> Could it be reanimation from a cardiac arrest? Is it not death >>> precisely that, ?death?? >>> >>> I hope you can clarify this dilemma for me, and thanking you in >>> advance, >>> >>> Dr. Oscar Ruiz Ropero >>> Intensivista >>> Hospital General Docente. Guantanamo. Cuba. -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061020/37cd9efb/attachment.html From info at scd-symposium.org Sat Oct 21 07:35:01 2006 From: info at scd-symposium.org (SCD Symposium) Date: Sat, 21 Oct 2006 07:35:01 -0300 Subject: [SCD-FORUM] 77E Jervell and Lange-Nielsen syndrome and cardiomyopathy. Dr. Didier Klug Message-ID: <5AE25CFF-0BC8-42CD-8C4A-B0205B81BB39@scd-symposium.org> We follow a young boy with a Jervell and Lange-Nielsen syndrome (KCNQ1) with syncope and torsade de pointe despite Nadolol requiring PM implantation at 6 month y.o. Torsade de pointes disappeared after right ventricular pacing + nadolol without syncope after a 8 years FU. But last echocardiography has shown a major left ventricular dilatation with ejection fraction = 18%. We have implanted a dual chamber ICD with MVP mode and atrial pacing without AV block. The FU is too short but at 3 months we have the feeling that EF improved (28%). What do you think about this association Jerwell cardiomyopathy. Can we incriminate desynchronisation with ventricular pacing or a deleterious effect of rapid pacing in patient with major alteration of their repolarization and perhaps calcium overload? Didier Klug, MD, PhD H?pital cardiologique de Lille University of Lille France -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061021/00428d06/attachment.html From info at scd-symposium.org Sat Oct 21 07:32:16 2006 From: info at scd-symposium.org (SCD Symposium) Date: Sat, 21 Oct 2006 07:32:16 -0300 Subject: [SCD-FORUM] 76E ICD implantation in children. Dr Didier Klug Message-ID: ICD implantation in children is a major problem. The first problem is the implantation of the ICD lead inside the right ventricle with coil unfitted for the size of the cavity and development of adherences between the coil and the tricuspid valve. In this population we will have to extract these leads during the FU of these patients and in our experience this extraction is challenging even on recent leads. Hence, we have recently used the technique describe by GASPARINI et al (J Cardiovasc Electrophysiol, Vol. 16, pp. 1381-1383) with a coil in the inferior vena-cava. What is your experience with this technique? Didier Klug, MD, PhD H?pital cardiologique de Lille University of Lille France -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061021/f748d8c0/attachment.html From info at scd-symposium.org Sat Oct 21 07:23:11 2006 From: info at scd-symposium.org (SCD Symposium) Date: Sat, 21 Oct 2006 07:23:11 -0300 Subject: [SCD-FORUM] 75E RE: Brugada's pattern detection in a son of SCD's patient. Dr. Makarov In-Reply-To: <04C86DC3-7BAE-4D95-8295-4B98D58752BC@scd-symposium.org> References: <04C86DC3-7BAE-4D95-8295-4B98D58752BC@scd-symposium.org> Message-ID: I think asymptomathic patient with 1 type of Brugada syndrome need in follow up with ECG control, Holter and information to avoid potentially dangerouse drugs and conditions: antyarrhythmic drugs (Na blockers), high temperature, rapid vagus stimulation vagus (diving and other). One of our patients with transient 1 type of Brugada symptom had synsope only during eating and high temperature. Leonid Makarov M.D., PhD. Professor of Medicine (Pediatry). Moscow Institute pediatry and children surgery. Center for children arrhythmia, Dpt diagnostic of arrhythmia. 125412 Moscow, Taldomskaa str. 2 Russsia leo at oss.ru -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee > > If type I ecg is present, pharmacologic test is not indicated, the > diagnosis > is made. > > Some investigators will go ahead and implant a defibrillator, > because of the > family history > > Others will wait until he develops any symptoms > > I recommend an EPS to risk stratify. If positive then ICD implant. > > > Ramon Brugada MD FACC > Associate Professor of Medicine > Canadian Research Chair Genetics of Arrhythmias > University of Montreal > Director Clinical Cardiovascular Genetics Center > Montreal Heart Institute > 5000 Rue Belanger > Montreal, QC H1T 1C8 > Canada > > ramon at brugada.org > > >> >> Dear Sergio, >> About a case of reanimation of sudden death in a 54-year-old >> patient, with no structural heart disease, with all the studies >> made: coronary angiography, MNR, Holter, EPS. >> Everything appears to be normal. Monomorphic arrhythmic storm is >> detected at the moment the patient is admitted. The patient is >> provided life support and he recovers in 24 hs. ICD implantation >> is decided. After three months he displays three shocks by >> arrhythmic storm, incessant ventricular tachycardia. Mapping and >> focus ablation is decided, in outflow tract with mismatch. >> To this moment, the patient has not displayed any event. It's been >> three months, and in a Holter register in one of his sons, typical >> Brugada pattern is observed. The patient is 17 years old, >> asymptomatic. I cannot perform pharmacological test. What should I >> do next? >> >> Dr. Cagnolatti >> -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061021/83171a5d/attachment.html From info at scd-symposium.org Sat Oct 21 08:09:15 2006 From: info at scd-symposium.org (SCD Symposium) Date: Sat, 21 Oct 2006 08:09:15 -0300 Subject: [SCD-FORUM] 71S HCM in 49-year-old man. Dr. de la Torre Message-ID: First of all, greetings. Congratulations for such an interesting event. I have a case I would like to share. It is a young man (49 years old) with hypertrophic cardiomyopathy diagnosed years ago. Now he is very symptomatic, with angina in rest and crises of palpitations accompanied by dizziness, and we could not document which type they are. In echocardiogram I have found besides septal hypertrophy of 23 mm, double gradient, one in LVOT, peak of 68 mmHg, and another intraventricular, of 145 mmHg. He is being treated with propranolol 40 mgs per 8 hours. My question is: would he benefit with a dual chamber pacemaker? Would it be essential to implant ICD? Which of the options is more appropriate (pacemaker, myectomy, or septal ablation)? Once again, congratulations, Dr. Rafael S. Le?n de la Torre Cardi?logo-Ecocardiografista Centro de atenci?n cardiovascular de Camaguey Cuba -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061021/80ae4760/attachment.html From info at scd-symposium.org Sat Oct 21 10:15:09 2006 From: info at scd-symposium.org (SCD Symposium) Date: Sat, 21 Oct 2006 10:15:09 -0300 Subject: [SCD-FORUM] WATCHING THE WEBCAST BY DR. FRANK MARCUS. THREE EXPERT QUESTIONS BY DR. KATARZYNA WLODARSKA Message-ID: Right Ventricular Dysplasia/Cardiomyopathy Frank Marcus Dr. Katarzyna Wlordaska (Poland) - Is ARVC/D a dysplasia? From the patophysiological point of view the term "dysplasia" should be reserved for Uhl's anomaly, which in my opinion should not be included to "right ventricular cardiomyopathy", even in broad meaning. Am I right? Dr. Frank Marcus (U.S.A.) - I will attempt to answer the questions posed by Dr.Wlodarska. Historically, the term right ventricular dysplasia was proposed by Dr. Fontaine and colleagues when they first described the disease in 1977, and this term has persisted. Dr. Fontaine maintaines that the term "dysplasia" is appropriate since it is defined as "a trouble of development". Dr. Katarzyna Wlordaska (Poland) - Do you think "Arrhythmogenic right ventricular cardiomyopathy/ dysplasia" is a synonyme of the right ventricular cardiomyopathy? If not, where is its place in the new Classification of Cardiomyopathies according to AHA Scientific Statement (Circulation, 2006,113,1807-1816)? Dr. Frank Marcus (U.S.A.) - This disease fits the classification proposed in the article "Contemporary Definitions and Classification of the Cardiomyopathies" by Maron B.J. et al in Circulation 2006;113:1807-1816. Their proposed definition of a cardiomyopathy was"Cardiomyopathies are a heterogeneous gruop of diseases of the myocardium associated with mechanical and/or electrical dysfunction that usually (but not invariably) exhibit inappropriate ventricular hypertrophy or dilatation and are due to a variety of causes that frequently are genetic" It would be well if those of us who are involved in the study of this entity agree on a terminology. This may be a point of discussion at a proposed conference to reasses the Task Force Criteria for the diagnosis of this disease. Until then we are left with ARVC, ARVD, ARVC/D or ARVD/C. Dr. Katarzyna Wlordaska (Poland) - I absolutely share your opinion on low diagnostic value of MRI in ARVC/D. I am afraid that overestimation of its value and lack of knowledge about the essential role of ECG is very common. However, ECG changes depend on extensiveness of the disease. What is a diagnostic value of ECG in borderline cases? Does ECG differentiate ARVC/D from Uhl's anomaly? Dr. Frank Marcus (U.S.A.) - You raise the question of the diagnostic value of the ECG in borderline cases. I think the ECG is exremely valuable under these circumstances, particularly T wave inversion beyond V2.. When I am presented with a patient in whom the differential diagnosis is idiopathic (RVOT) tachycardia, and the ECG fits the above description, it greatly heightens my suspicion that the patient has ARVC/D. T wave inversion in V1-V3 is present in less than 3% of apparently healthy subjects who are 19-45 years of age but is found in well over 50% of patients with ARVC/D. Ref. Marcus F. Am. J. Cardiol 2005;95;1070-1071. We are currently evaluating the extensive information from the North American ARVC/D database to further study the relation of the ECG changes with the extent of right ventricular involvement. Uhls anomaly is so rare that it does not enter into my differential diagnosis of ARVC/D. -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee From info at scd-symposium.org Sat Oct 21 12:23:24 2006 From: info at scd-symposium.org (SCD Symposium) Date: Sat, 21 Oct 2006 12:23:24 -0300 Subject: [SCD-FORUM] 70S Brugada pattern post-PAF reversion. Dr. Diangelo In-Reply-To: <31929680-3314-449C-B955-CB13DF7729F2@scd-symposium.org> Message-ID: <5C6346DB-4127-4086-BC7A-E3DD9D61BF4C@scd-symposium.org> I would like to ask Dr. Andres Perez Riera about a 35-year-old, male patient, with no structural heart disease or family history of SCD, baseline ECG with incomplete right bundle branch block with superior axis, who presents for the first time with Paroxysmal Atrial Fibrillation, which after being reverted with oral propafenone 600 mg (UD), induces ECG with Brugada pattern. What is the prognosis? Therapeutics? Does he need an additional screening (genetic, EPS)? Is he really a Brugada patient or is this just the effect of the drug, producing Brugada-like pattern? Thank you very much. Kind regards, Dr. Silvano Diangelo Argentina -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061021/7f1bb251/attachment.html From info at scd-symposium.org Sat Oct 21 12:03:38 2006 From: info at scd-symposium.org (SCD Symposium) Date: Sat, 21 Oct 2006 12:03:38 -0300 Subject: [SCD-FORUM] 78E RE: Brugada's pattern detection in a son of SCD's patient. Dr. Ramon Brugada In-Reply-To: References: <04C86DC3-7BAE-4D95-8295-4B98D58752BC@scd-symposium.org> Message-ID: <12598A91-127C-4776-ACDC-454B3A4AE813@scd-symposium.org> Every single patient who is symptomatic has been asymptomatic for several years, usually 40 . Most, but not all, will have premonitory symptoms like syncope. From experience, at the time of the syncopal episode the majority have not requested medical advise (they were young and thought not to be important). In summary, the first symptom for which they seek medical help may be aborted cardiac arrest.To follow up an aymptomatic is an option, however, a small percentatge will die. HOLTER In a meeting a few years ago there was the discussion regarding the use of a reveal in Brugada patients. I quote one clinician who said at that time "Reveal in a patient with Brugada syndrome may show two things. 1- nothing 2- malignant arrhythmia causing sudden cardiac death". Brugada patients are characterized by having minimal amount or arrhythmias, Holter will rarely show anything worrisome. thanks Ramon Brugada -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee > > I think asymptomathic patient with 1 type of Brugada syndrome need > in follow up with ECG control, Holter and information to avoid > potentially dangerouse drugs and conditions: antyarrhythmic drugs > (Na blockers), high temperature, rapid vagus stimulation vagus > (diving and other). One of our patients with transient 1 type of > Brugada symptom had synsope only during eating and high temperature. > Leonid Makarov M.D., PhD. Professor of Medicine (Pediatry). Moscow > Institute pediatry and children surgery. Center for children > arrhythmia, Dpt diagnostic of arrhythmia. > 125412 Moscow, Taldomskaa str. 2 Russsia leo at oss.ru > > -- > Dr. Sergio Dubner > President of Scientific Committee > > Dr. Edgardo Schapachnik > President of Steering Committee > > >> >> If type I ecg is present, pharmacologic test is not indicated, the >> diagnosis >> is made. >> >> Some investigators will go ahead and implant a defibrillator, >> because of the >> family history >> >> Others will wait until he develops any symptoms >> >> I recommend an EPS to risk stratify. If positive then ICD implant. >> >> >> Ramon Brugada MD FACC >> Associate Professor of Medicine >> Canadian Research Chair Genetics of Arrhythmias >> University of Montreal >> Director Clinical Cardiovascular Genetics Center >> Montreal Heart Institute >> 5000 Rue Belanger >> Montreal, QC H1T 1C8 >> Canada >> >> ramon at brugada.org >> >> >>> >>> Dear Sergio, >>> About a case of reanimation of sudden death in a 54-year-old >>> patient, with no structural heart disease, with all the studies >>> made: coronary angiography, MNR, Holter, EPS. >>> Everything appears to be normal. Monomorphic arrhythmic storm is >>> detected at the moment the patient is admitted. The patient is >>> provided life support and he recovers in 24 hs. ICD implantation >>> is decided. After three months he displays three shocks by >>> arrhythmic storm, incessant ventricular tachycardia. Mapping and >>> focus ablation is decided, in outflow tract with mismatch. >>> To this moment, the patient has not displayed any event. It's >>> been three months, and in a Holter register in one of his sons, >>> typical Brugada pattern is observed. The patient is 17 years old, >>> asymptomatic. I cannot perform pharmacological test. What should >>> I do next? >>> >>> Dr. Cagnolatti >>> -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061021/86f11b90/attachment.html From info at scd-symposium.org Sat Oct 21 18:11:23 2006 From: info at scd-symposium.org (SCD Symposium) Date: Sat, 21 Oct 2006 18:11:23 -0300 Subject: [SCD-FORUM] 72S RE: Brugada pattern post-PAF reversion. Dr. Rondon In-Reply-To: <5C6346DB-4127-4086-BC7A-E3DD9D61BF4C@scd-symposium.org> References: <5C6346DB-4127-4086-BC7A-E3DD9D61BF4C@scd-symposium.org> Message-ID: <22C459B8-153A-468F-B209-170CA8F65D12@scd-symposium.org> Dr. Silvano Diangelo Argentina Dear Dr. I dare to express an opinion about this case, because I have had 6 cases of young boys with Brugada pattern, 1 triggered by exercise, 2 triggered by drugs, and 3 with spontaneous expression, who were studied with SA-ECG, with presence of late potentials and echocardiographic evaluation with maintained LV function. I think the EPS is very important to be accurate about malignant arrhythmia induction, even when there are cases in which it is not possible to induce sustained VT. I think that a patient with stratification of: Brugada pattern in any of its manifestations With SA-ECG with evidence of late potentials Evidence in EPS of sustained malignant arrhythmia induction. Should go to protection with ICD, even when I am well aware that it is not easy to implant a system in asymptomatic patients; but the idea is to protect them from SCD events, which could be the only and last arrhythmic manifestation in this patient. Dr Mauricio Rond?n Caracas - Venezuela -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee > > I would like to ask Dr. Andres Perez Riera about a 35-year-old, > male patient, with no structural heart disease or family history of > SCD, baseline ECG with incomplete right bundle branch block with > superior axis, who presents for the first time with Paroxysmal > Atrial Fibrillation, which after being reverted with oral > propafenone 600 mg (UD), induces ECG with Brugada pattern. > What is the prognosis? Therapeutics? > Does he need an additional screening (genetic, EPS)? > Is he really a Brugada patient or is this just the effect of the > drug, producing Brugada-like pattern? > > Thank you very much. > > Kind regards, > Dr. Silvano Diangelo > Argentina > > -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061021/5b90c20c/attachment.html From info at scd-symposium.org Sat Oct 21 19:21:21 2006 From: info at scd-symposium.org (SCD Symposium) Date: Sat, 21 Oct 2006 19:21:21 -0300 Subject: [SCD-FORUM] 79E RE: Brugada's pattern detection in a son of SCD's patient. Dr. Dubner In-Reply-To: References: Message-ID: Dear Cagnolatti: Very interesting question that Ramon Brugada already answered it. I am sure we will listen some other oppinon about it, either from Pedro and Josep or Silvia and her group. On the other hand, evidence emerging from the study of fibrillation both in the atria and the ventricle suggests an important role for triggers arising from the Purkinje network or the RVOT in the initiation of VF. Initial experience in patients with IVF and even those with VF associated with LQTS Brugada syndrome and genuine Idiopathic VF suggests that long term suppression of recurrent VF may be feasible by the elimination of these triggers. With the development of new mapping and ablation technologies, and greater physician experience, catheter ablation of VF, with the ultimate aim of curing such patients at risks of SCD, may not be an unrealistic goal in the future (2). Haissaguerre et al(3), localized by mapping the earliest endocardial activity and by focal radiofrequency ablation of PTV/VF in three patients with Brugada Syndrome. The authors conclude that triggers from the Purkinje arborization or the RVOT have a crucial role in initiating VF associated with Brugada syndrome and LQTS. These can be eliminated by focal radiofrequency ablation. In a highly symptomatic 18-year-old-male with BS, frequent episodes of VF, fast PVT, and fast S-MVT were observed. The episodes were classified as VT or VF and as a consequence received appropriate therapies with the ICD. Precipitating VPBs that were stored in the ICD memory and on the electrocardiogram (ECG) exhibited the same morphology as frequent isolated VPBs. During the PES, right and left atrial tachycardia with one-to-one atrioventricular conduction were induced and successfully ablated. VF was ablated using the same noncontact mapping (NCM) system- triggering VPBs from RVOT(4). Yu e al (5) presented a case of recurrent syncope diagnosed as recurrent VF by an implanted loop recorder (ILR). The VF was eliminated by RFCA of triggering ventricular premature complexes (VPCs). The characteristics of VT in the BrS are: very fast polymorphic ventricular tachycardia (PVT) is frequent (from 260 to 352bpm), with very short onset extrasystole coupling (388 +/- 28msec), generally preceded by premature ventricular contractions (PVCs) that are identical to the beating that starts IPVT (6) Spontaneous episodes of VF in patients with BrS are triggered by specific PVCs (7) Arrhythmic events occur in 93% of the cases during sleep or at dawn in 92% of the cases when the patients present significant ST segment elevation. It has been well established that the degree of ST segment elevation is responsible for arrhythmias. A loss of phase 2 or PAT dome in the RV epicardium (where the onset potassium outward Ito current is more prominent) and not in the endocardium, causes ST segment elevation. The normal heterogeneity existing between the epicardium and the endocardium is increased in this entity, leading to repolarization abnormalities in ECG and to a greater possibility of arrhythmia by the mechanism called phase 2 reentry. These forms resemble the very fast torsades de pointes (TdP) observed in patients with normal QTc; nevertheless, there are clear differences between both atypical tachyarrhythmias. Observation: Only in exceptional cases, bursts of spontaneous Monomorphic Idiopathic Ventricular Tachycardia (MIVT) may happen (8); however, the monomorphic form of VT is observed only when induced by drugs. There are references about S-MVT appearance after the administration of ajmaline, because the drug increases heterogeneity even more in ventricular thickness repolarization(9). There is a reference of incessant monomorphic ventricular tachycardia during febrile illness in a patient with BrS (10). An automatic mechanism mediated by the beta-receptor seems to hold an important role in the S-MVTs that originate in the RVOT. The place of origin of the event could be very near to the lesion that causes ST elevation(11). In these cases in which monomorphic ventricular tachycardia (MVT) is inducible by drugs, an automatic mechanism is pointed out as electrophysiological substrate, produced in an activity focus triggered by late after-depolarization located in the RVOT (12). Warmest regards, Sergio Dubner and Andres Ricardo Perez Riera References 1) McGregor M, Chen J. Should the implantable cardiac defibrillator be used for primary prevention of sudden death? A review of the issues relevant to hospital decision making. Can J Cardiol. 2004; 20:1199-1204. 2) Sanders P, Hsu LF, Hocini M, et al. Mapping and ablation of ventricular fibrillation.Minerva Cardioangiol. 2004; 52:171-181.) (Weerasooriya R, Hsu LF, Scavee C, et al. Catheter Ablation of Ventricular Fibrillation in Structurally Normal Hearts Targeting the RVOT and Purkinje Ectopy. Herz. 2003; 28:598-606. 3) Haissaguerre M, Extramiana F, Hocini M, et al. Mapping and ablation of ventricular fibrillation associated with long-QT and Brugada syndromes. Circulation. 2003; 108:925-928. 4) Darmon JP, Bettouche S, Deswardt P, et al. Radiofrequency Ablation of Ventricular Fibrillation and Multiple Right and Left Atrial Tachycardia in a Patient with Brugada Syndrome. J Interv Card Electrophysiol. 2004; 11:205-209. 5) Yu CC, Tsai CT, Lai LP, Lin JL. Successful radiofrequency catheter ablation of idiopathic ventricular fibrillation presented as recurrent syncope and diagnosed by an implanted loop recorder. Int J Cardiol. 2006;110:112-3. 6) Kakishita M, Kurita T, Matsuo K, et al. Mode of onset of ventricular fibrillation in patients with Brugada syndrome detected by implantable cardioverter defibrillator therapy. J Am Coll Cardiol 2000; 36:1646-1653. 7) Gang ES, Priori SS, Chen PS. Short Coupled Premature Ventricular Contraction Initiating Ventricular Fibrillation in a Patient with Brugada Syndrome. J Cardiovasc Electrophysiol. 2004; 15:837. 8) Sastry BK, Narasimhan C, Soma Raju B. Brugada syndrome with monomorphic ventricular tachycardia in a one-year-old child. Indian Heart J 2001; 53:203-205. 9) Pinar Bermudez E, Garcia-Alberola A, Martinez Sanchez J, et. al.: Spontaneous sustained monomorphic ventricular tachycardia after administration of ajmaline in a patient with Brugada syndrome. Pacing Clin Electrophysiol 2000; 23:407-409. 10) Dinckal MH, Davutoglu V, Akdemir I, Soydinc S, Kirilmaz A, Aksoy M. Incessant monomorphic ventricular tachycardia during febrile illness in a patient with Brugada syndrome: fatal electrical storm. Europace. 2003; 5:257-61. 11) Shimada M, Miyazaki T, Miyoshi S, et al. Sustained monomorphic ventricular tachycardia in a patient with Brugada syndrome. Jpn Circ J 1996; 60: 364-370, 12) Ogawa M, Kumagai K, Saku K. Spontaneous right ventricular outflow tract tachycardia in a patient with Brugada syndrome. J Cardiovasc Electrophysiol 2001; 12:838-840. Sergio J. Dubner, MD, FACC Director Arrhythmias and Electrophysiology Service Clinica y Maternidad Suizo Argentina Arenales 2463 3 A 1124 Buenos Aires - Argentina -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee El 20/10/2006, a las 12:26, SCD Symposium escribi?: > Forum of the ISHNE Sudden Cardiac Death World-Wide Internet Symposium > ______________________________________________________________________ > > Medtronic ICDs with world-leading features: > http://www.medtronic.com/physician/tachy/ > ______________________________________________________________________ > > Dear Sergio, > About a case of reanimation of sudden death in a 54-year-old > patient, with no structural heart disease, with all the studies > made: coronary angiography, MNR, Holter, EPS. > Everything appears to be normal. Monomorphic arrhythmic storm is > detected at the moment the patient is admitted. The patient is > provided life support and he recovers in 24 hs. ICD implantation is > decided. After three months he displays three shocks by arrhythmic > storm, incessant ventricular tachycardia. Mapping and focus > ablation is decided, in outflow tract with mismatch. > To this moment, the patient has not displayed any event. It's been > three months, and in a Holter register in one of his sons, typical > Brugada pattern is observed. The patient is 17 years old, > asymptomatic. I cannot perform pharmacological test. What should I > do next? > > Dr. Cagnolatti > > -- > Dr. Sergio Dubner > President of Scientific Committee > > Dr. Edgardo Schapachnik > President of Steering Committee > > > > > _______________________________________________ > Scd-forum mailing list > Scd-forum at scd-symposium.org > http://www.grupoakros.com.ar/mailman/listinfo/scd-forum -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061021/3e5b1fac/attachment.html From info at scd-symposium.org Sat Oct 21 20:53:01 2006 From: info at scd-symposium.org (SCD Symposium) Date: Sat, 21 Oct 2006 20:53:01 -0300 Subject: [SCD-FORUM] 74S Alleged apical hypertrophic cardiomyopathy. Dr. Wolman In-Reply-To: <6F87EC52-C28E-4822-AAB2-6597A0367BEC@grupoakros.com.ar> Message-ID: <1B1C7853-1326-4C25-9036-1F6126068CC3@scd-symposium.org> Hello, friends from the cyberspace, Congratulations and thank you for this new great event. I would like to ask about the best management to follow in the following clinical case: this is a 56-year-old patient, who consulted 10 years ago as a routine. In that moment he had a very pathological ECG, with negative and huge T waves in all the anterior side. We conducted ergometer test, 2D echo, Holter and coronary angiography, and all of them resulted normal. Since then studies have been repeated annually, and he remains completely asymptomatic. The ECG persists with no changes and the two last echoes report: apical ventricular hypertrophy. A spect gamma camera does not report the alleged apical hypertrophy. We have scheduled a cardiac resonance. The question is: should this patient receive any treatment? For instance, beta blockers? At first I had thought that this was a case of genetic hypertrophic cardiomyopathy (troponin?) without phenotype. Now, I'm not sure that's correct. I would like to have your opinion about what management to follow. The father of the patient died when he was 56 years old, suddenly, but he ignores if the father was a coronary patient, and the sons of the patient do not show any pathology to this date. Thank you very much, Luis Wolman -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061021/94049335/attachment.html From info at scd-symposium.org Sat Oct 21 20:40:04 2006 From: info at scd-symposium.org (SCD Symposium) Date: Sat, 21 Oct 2006 20:40:04 -0300 Subject: [SCD-FORUM] 73S RE Brugada pattern post-PAF reversion. Dr. Perez Riera In-Reply-To: <5C6346DB-4127-4086-BC7A-E3DD9D61BF4C@scd-symposium.org> References: <5C6346DB-4127-4086-BC7A-E3DD9D61BF4C@scd-symposium.org> Message-ID: Dear Silvano Diangelo from Argentina, I'm Andres Ricardo Perez Riera from Brazil, and I'll answer your question. A young adult (35 years old), male, with electrocardiographic pattern of IRBBB with superior axis and that presents a crisis of paroxysmal AF with no apparent cardiac or extracardiac cause (I am certain that you investigated hyperthyroidism and other causes for AF), falls in the category of idiopathic AF: essential, primary, lonely, or as Anglo-Saxon literature call it, "lone atrial fibrillation", which is defined as AF that appears with no evidence (clinical or lab) of structural heart disease or systemic disease. It represents 6% to 10% of the cases of AF, and in many cases it translates an increase of vagal tone, generally during the night (AF of vagotonic type), transitory and in rest. These apparently idiopathic cases may have a genetic basis, and stop being so. AF apparently without cause has been mapped in several chromosomes: 1) Dominant autosomal chromosome 10 in region 10q22-q23 (1); 2) One of the mapped genes, 10q22 in region DLG5, is a member of the family of the proteins called MAGUKs (Membrane Associated Gyanylate Kinase). This family mediates intracellular signs with function in the formation of cellular binds, in maintenance of cell form, and clustering of protein channels in cell surface (2); 3) A family with the mutation (S140G) in gene KCNQ1 (KvKQT1) of chromosome 11p15.5 was also identified. This KCNQ1 gene, encodes the pore that makes up the alpha subunit of I(Ks) channel. The analysis of this mutation revealed a gain in function in KCNQ1/KCNE1 and KCNQ1/ KCNE channels. This fact is in contrast with the negative effect or loss of function in KCNQ1 observed in long QT syndrome. Thus, the S140G mutation starts and maintains AF by reducing the duration of AP, and the refractory period of atrial cardiomyocytes (3). First conclusion: in this patient it is necessary to perform a genetic study. Not only because of suspicion of Brugada syndrome, but to determine if AF is idiopathic or genetic. This patient may have a cancelled from of Brugada that was unmasked by use of endovenous propafenone. Several papers show that Brugada syndrome may be unmasked with propafenone in this way (4). Propafenone in Brugada syndrome may unmask VT crisis, both polymorphic and monomorphic (5). It may cause J wave in inferior lead when injected in patients carriers of the so-called variant Brugada syndrome (6). A case very similar to yours was presented last year (2005) by Aksay et al in Turkey, in which the patient presented acute AF, and when propafenone was administered to reverse AF, Brugada pattern appeared (7). A similar situation was observed by Beldner et al (8) in patients with AF treated with propafenone or with flecainide. Second conclusion: your patient seems to have Brugada syndrome, and should be electrophysiologically studied. "It looks like a lion, and it is a lion." If positive, ICD. References 1) Brugada R, Tapscott T, Czernuszewic GZ, et al. Identification of a genetic locus for familial atrial fibrillation N Engl J Med 1997; 336:905-911); 2) Shah G, Brugada R, Gonzalez O, et al. The cloning, genomic organization and tissue expression profile of the human DLG5 geneBMC Genomics 2002 3: 6. 3) Chen YH, Xu SJ, Bendahhou S, et al. KCNQ1 gain-of-function mutation in familial atrial fibrillation. Science 2003; 299:251-254. 4) Matana A, Goldner V, Stanic K, et al. Unmasking effect of propafenone on the concealed form of the Brugada phenomenon. PACE 2000;23:416-418. 5) Karaca M, Dinckal MH.Successful radiofrequency catheter ablation of idiopathic ventricular fibrillation presented as recurrent syncope and diagnosed by an implanted loop recorder. Acta Cardiol. 2006;61:481-484. 6) Ozeke O, Aras D, Celenk MK, Exercise-induced ventricular tachycardia associated with J point ST-segment elevation in inferior leads in a patient without apparent heart disease: a variant form of Brugada syndrome? J Electrocardiol. 2006; 39:409-412. 7) Aksay E, Okan T, Yanturali S.Brugada syndrome, manifested by propafenone induced ST segment elevation. Emerg Med J. 2005;22:748-750. 8) Beldner S, Lin D, Marchlinski FE.Flecainide and propafenone induced ST-segment elevation in patients with atrial fibrillation: clue to specificity of Brugada-type electrocardiographic changes. Am J Cardiol. 2004;94:1184-5. Cordially, Andr?s Ricardo P?rez Riera Jefe del Sector de Electro-Vectocardiologia de la disciplina de Cardiologia del ABC. Facultad de Medicina del ABC (FMABC), Foundaci?n del ABC (FUABC) - Santo Andr? - Sao Paulo - Brasil. Rua Sebastiao Afonso 885 - Zip Code: 044417-100- Jardim Miriam S.P Brazil -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee > > I would like to ask Dr. Andres Perez Riera about a 35-year-old, > male patient, with no structural heart disease or family history of > SCD, baseline ECG with incomplete right bundle branch block with > superior axis, who presents for the first time with Paroxysmal > Atrial Fibrillation, which after being reverted with oral > propafenone 600 mg (UD), induces ECG with Brugada pattern. > What is the prognosis? Therapeutics? > Does he need an additional screening (genetic, EPS)? > Is he really a Brugada patient or is this just the effect of the > drug, producing Brugada-like pattern? > > Thank you very much. > > Kind regards, > Dr. Silvano Diangelo > Argentina > > -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061021/14ce302a/attachment.html From info at scd-symposium.org Sun Oct 22 00:19:16 2006 From: info at scd-symposium.org (SCD Symposium) Date: Sun, 22 Oct 2006 00:19:16 -0300 Subject: [SCD-FORUM] 81C Case: Male, 29yrs. Dr. Zhu Message-ID: <5D20FB2D-014F-4979-8DDF-90928BDE6386@scd-symposium.org> Case: Male, 29yrs Illness history: Viral myocarditis was diagnosed based on premature ventricular contractions (PVCs) at age 8 after an influenza attack. Since then, PVBs never disappeared and right ventricle enlargement had been noticed. ECG revealed VT lasting 3 minutes at age 19, accompanied with faintness. After 2-3 years, no VT reoccurred. The second VT episode was recorded at age 21 lasting 2-3 hrs accompanied with syncope. Intravenous propafenon worked. Oral administration of propafenon could not prevent the patient from VT attack. RFCA for VT had been delivered twice at age 22~23 by Pro. Yang at the first affiliated Hospital of Dalian medical university. During RFCA, VT could not be induced and cardiomyopathy was diagnosed by endocardiac biopsy. VTs occurred intermediately lasting several seconds to a few minutes. VT restored to sinuous rhythm either spontaneously or by intravenous propafenon administration. VT usually occurred at midnight or early morning. At the following years, Sometimes VT occurred quite often during a certain period (for example: twice attacks in Winter), and then no occurrence for several months or even longer time. The patient enjoys doing exercise. No VT attacked during exercise. No restriction to exercise. Since 27 yrs old (2004), propafenon has been administrated in the doses of 150mg bid or tid. Drug quitted after 1-2 months administrations, keeping no VT attack for 0.5-1.5 yrs. Since last year, the drug cannot be quitted, otherwise VT reoccurred. So far, the doses of the drug are 200mg at early morning and 150mg at night. Lower dosages may not prevent the VTs. Any suggestion about the treatment? Zhu -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061022/43a9c60e/attachment.html From info at scd-symposium.org Sun Oct 22 08:40:55 2006 From: info at scd-symposium.org (SCD Symposium) Date: Sun, 22 Oct 2006 08:40:55 -0300 Subject: [SCD-FORUM] 82E RE: Case: Male, 29yrs. Dr. Fontaine to Dr. Zhu In-Reply-To: <5D20FB2D-014F-4979-8DDF-90928BDE6386@scd-symposium.org> References: <5D20FB2D-014F-4979-8DDF-90928BDE6386@scd-symposium.org> Message-ID: <365DAACC-66A0-4FE1-A2DE-03CDC2F2381F@scd-symposium.org> Dear Dr Zhu Interesting case. In such a case it is important what is the probable site of origin of VT ? Is the VT always of the same morphology ? If VT originates in the left ventricle it is probably the result of myocarditis If VT originates in the RV it is probably a case of ARVD or IDCM with preserved cardiac function. Therefore it is also important to have a better description of the clinical expression of myocarditis. How severe it was? It is also important to know if regular echo showed a decrease in cardiac function. Guy Fontaine MD PhD -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee > > Case: Male, 29yrs > > Illness history: Viral myocarditis was diagnosed based on premature > ventricular contractions (PVCs) at age 8 after an influenza attack. > Since then, PVBs never disappeared and right ventricle enlargement > had been noticed. > > ECG revealed VT lasting 3 minutes at age 19, accompanied with > faintness. After 2-3 years, no VT reoccurred. > > The second VT episode was recorded at age 21 lasting 2-3 hrs > accompanied with syncope. Intravenous propafenon worked. Oral > administration of propafenon could not prevent the patient from VT > attack. > > RFCA for VT had been delivered twice at age 22~23 by Pro. Yang at > the first affiliated Hospital of Dalian medical university. During > RFCA, VT could not be induced and cardiomyopathy was diagnosed by > endocardiac biopsy. > > > VTs occurred intermediately lasting several seconds to a few > minutes. VT restored to sinuous rhythm either spontaneously or by > intravenous propafenon administration. VT usually occurred at > midnight or early morning. At the following years, Sometimes VT > occurred quite often during a certain period (for example: twice > attacks in Winter), and then no occurrence for several months or > even longer time. The patient enjoys doing exercise. No VT attacked > during exercise. No restriction to exercise. > > Since 27 yrs old (2004), propafenon has been administrated in the > doses of 150mg bid or tid. Drug quitted after 1-2 months > administrations, keeping no VT attack for 0.5-1.5 yrs. > Since last year, the drug cannot be quitted, otherwise VT > reoccurred. So far, the doses of the drug are 200mg at early > morning and 150mg at night. Lower dosages may not prevent the VTs. > > Any suggestion about the treatment? > > Zhu -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061022/3c4359d3/attachment.html From info at scd-symposium.org Sun Oct 22 08:47:52 2006 From: info at scd-symposium.org (SCD Symposium) Date: Sun, 22 Oct 2006 08:47:52 -0300 Subject: [SCD-FORUM] 83E RE: ICD implantation in children. Dr. Peter Scwartz to Dr. Didier Klug In-Reply-To: References: Message-ID: <9571608A-4661-42EB-A145-319596BF6C92@scd-symposium.org> I could not agree more. The complexities and rate of complications with ICD implants in children are such to make not justifiable this approach, for LQTS patients, unless there has been a cardiac arrest or very special circumstances. It should not be forgotten that the combined incidence of CA/SD for LQT1 patients (mean follow-up 12 years) is 1.2%. LQT1 pts with syncope don't need an ICD. And LQT2 pts have an incidence of 7% (all CA, in our 2 series). What is necessary is to explain carefully to the families the pro and cons and the actual risks, and then help them to decide. This, however, takes time. And this is what a good physician should do. Peter J. Schwartz -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee > > ICD implantation in children is a major problem. The first problem > is the implantation of the ICD lead inside the right ventricle with > coil unfitted for the size of the cavity and development of > adherences between the coil and the tricuspid valve. In this > population we will have to extract these leads during the FU of > these patients and in our experience this extraction is challenging > even on recent leads. Hence, we have recently used the technique > describe by GASPARINI et al (J Cardiovasc Electrophysiol, Vol. 16, > pp. 1381-1383) with a coil in the inferior vena-cava. What is your > experience with this technique? > > Didier Klug, MD, PhD > H?pital cardiologique de Lille > University of Lille > France > -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061022/354470b8/attachment.html From info at scd-symposium.org Sun Oct 22 08:56:43 2006 From: info at scd-symposium.org (SCD Symposium) Date: Sun, 22 Oct 2006 08:56:43 -0300 Subject: [SCD-FORUM] 84E. LQTS in pregnancy. Dr. Zaklyazminskaya Message-ID: <0565EF9E-D5A3-40F5-ABF4-2800B625D405@scd-symposium.org> Dear organizers and colleagues! Thank you for interesting and useful educational materials and remarkable discussion! I'd like to ask your advise about my patients applied genetic counselling last week. Patient is young woman (24 years old) with LQTS, QTc-480-495 ms, with pre-syncope events in your adolescence but not at present time. She was treated by beta-blockers before 19 years old and had been canceled medication self-dependent. She is in her 18 weeks of pregnancy without any symptoms or specific discomfort. I have some doubt about absence of medication in this situation. In 4 known generation we have information about 4 additional relatives with mild course of disease and none SCD events. Now we verified KCNQ1 mutation in all affected family members and going to diagnose LQTS for baby synchronous with blood sampling for newborn metabolic screening (4 day old in Russia). My questions are: 1/ Is it advisable to recommence beta-blockers now? 2/ Do you know any side effects due to beta-blockers further to possible A-V block for fetus? 3/ What are yours recommendations for therapy in postpartum and lactation ? 4/ Not only for this patient. Is the strategy of mother's therapy differ against child status (affected/non-affected, mutation carrier/non- carrier) during lactation taking into account extraction of beta-blockers with the milk? Thank you in advance for all opinions and consultations! Dr. Elena Zaklyazminskaya, M.D., Ph.D. Russian Research Center for Medical Genetics, 1, Moskvorechie, Moscow, Russia, 115478 dnalab at rol.ru www.dnalab.ru -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061022/27ebde18/attachment.html From info at scd-symposium.org Sun Oct 22 09:26:19 2006 From: info at scd-symposium.org (SCD Symposium) Date: Sun, 22 Oct 2006 09:26:19 -0300 Subject: [SCD-FORUM] 85E RE: HCM in 49-year-old man. Dr. Skeberis In-Reply-To: References: Message-ID: <398B56FB-1E9D-4F73-A488-7FA6627C7796@scd-symposium.org> Sharing in this excellent symposium, I would like to suggest that a surgical solution must be given, from the hemodynamic point of view. As for an ICD implantation, I would be very sustained because the patient had no syncope and no information about the familiar history is written down. Finally, I would like to ask if a Holter monitoring had been performed, in order to diagnose the nature of the palpitation? =20 Vassilis Skeberis MD, FESC Cardiology Deapartment and EP Lab of Hippokration University Hospital, Thessaloniki Greece -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee > > First of all, greetings. Congratulations for such an interesting > event. > I have a case I would like to share. It is a young man (49 years > old) with hypertrophic cardiomyopathy diagnosed years ago. > Now he is very symptomatic, with angina in rest and crises of > palpitations accompanied by dizziness, and we could not document > which type they are. In echocardiogram I have found besides septal > hypertrophy of 23 mm, double gradient, one in LVOT, peak of 68 > mmHg, and another intraventricular, of 145 mmHg. He is being > treated with propranolol 40 mgs per 8 hours. > My question is: would he benefit with a dual chamber pacemaker? > Would it be essential to implant ICD? Which of the options is more > appropriate (pacemaker, myectomy, or septal ablation)? > Once again, congratulations, > Dr. Rafael S. Le?n de la Torre > Cardi?logo-Ecocardiografista > Centro de atenci?n cardiovascular de Camaguey > Cuba > > -- > Dr. Sergio Dubner > President of Scientific Committee > > Dr. Edgardo Schapachnik > President of Steering Committee > > > > > _______________________________________________ > Scd-forum mailing list > Scd-forum at scd-symposium.org > http://www.grupoakros.com.ar/mailman/listinfo/scd-forum -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061022/5527ccbf/attachment.html From info at scd-symposium.org Sun Oct 22 13:30:39 2006 From: info at scd-symposium.org (SCD INFO) Date: Sun, 22 Oct 2006 13:30:39 -0300 Subject: [SCD-FORUM] 87E RE: Alleged apical hypertrophic cardiomyopathy. Dr. Jamil Message-ID: <453B9CAF.4040904@scd-symposium.org> Dear Luis Wolman Thank you very much for presenting such an interesting case history. Apical hypertrophic cardiomyopathy relatively common in Asia including Bangladesh. Regarding management of your patient, he doesn't require any active treatment at present. But as his father died suddenly in premature age, he needs farther evaluation to detect potential risk of malignant vetricular arrhythmia and SCD. So, EPS would be a good choice for farther evalation and to decide for profilactic ICD for prevention of SCD. Thank you very much. Abdullah Al Jamil, MBBS, FCPS, MD Electrophysiologist Associate Professor of Cardiology Bangabandhu Sheikh Mujib Medical University Shabag, Dhaka-1000, Bangladesh > Hello, friends from the cyberspace, > Congratulations and thank you for this new great event. I would like > to ask about the best management to follow in the following clinical > case: this is a 56-year-old patient, who consulted 10 years ago as a > routine. In that moment he had a very pathological ECG, with negative > and huge T waves in all the anterior side. We conducted ergometer > test, 2D echo, Holter and coronary angiography, and all of them > resulted normal. Since then studies have been repeated annually, and > he remains completely asymptomatic. The ECG persists with no changes > and the two last echoes report: apical ventricular hypertrophy. A > spect gamma camera does not report the alleged apical hypertrophy. We > have scheduled a cardiac resonance. The question is: should this > patient receive any treatment? For instance, beta blockers? > At first I had thought that this was a case of genetic hypertrophic > cardiomyopathy (troponin?) without phenotype. Now, I'm not sure that's > correct. I would like to have your opinion about what management to > follow. The father of the patient died when he was 56 years old, > suddenly, but he ignores if the father was a coronary patient, and the > sons of the patient do not show any pathology to this date. Thank you > very much, > Luis Wolman From info at scd-symposium.org Sun Oct 22 13:09:44 2006 From: info at scd-symposium.org (SCD INFO) Date: Sun, 22 Oct 2006 13:09:44 -0300 Subject: [SCD-FORUM] 86E RE: Alleged apical hypertrophic cardiomyopathy. Dr. Perez Riera Message-ID: <453B97C8.9010407@scd-symposium.org> Dear Luis Wolman: I'm Andres Ricardo Perez Riera from Sao Paulo Brazil, and I'll answer your question. Hypertrophic cardiomyopathy (HCM) is a myocardial disease with variable phenotpye and genotype. Non-obstructive hypertrophy localized to the cardiac apex (wall thickening is confined to the most distal region at the apex,) or apical hypertrophic cardiomyopathy (ApHCM) is a specific variant of HCM. This disease has been first described in Japan where the prevalence is much higher than in the western world. ApHCM, occurs in only 1 to 2% of the non-Japanese population. Only a limited number of sarcomere gene defects (eg, cardiac actin Glu101Lys) consistently produce ApHCM (1). A single amino acid substitution in actin causes either CHF or maladaptive cardiac hypertrophy, depending on its effect on actin structure and function. De novo mutations in cardiac actin gene were identified in two patients with sporadic HCM who presented with syncope in early childhood. Patients were heterozygous for missense mutations resulting in Pro164Ala and Ala331Pro amino acid substitutions, adjacent to regions of actin-actin and actin-myosin interaction, respectively. A mutation that cosegregated with familial HCM was also found, causing a Glu99Lys substitution in a weak actomyosin binding domain. The cardiac phenotype in many affected patients was characterized by an ApHCM (2). The typical features of AHC include: 1) Giant negative T waves in the precordial ECG leads Giant negative T waves negativity mayor or equal 1.0 mV (10 mm). Giant negative T waves are more common in Japanese patients than American patients: 15% in Japan vs 3% in US (3); 2) Sometimes R-wave voltage and T-wave negativity progressively decreased in magnitude at serial electrocardiograms; 3) Non-sustained or sustained VT in patients that developed apical aneurysm with normal coronary arteries; 4) A spade-like configuration of the left ventricle at end-systole in the right anterior oblique projection. Non-spade ApHCM was newly identified on cardiac magnetic resonance (CMR) short-axis images, and this could be an additional, important underlying cause of moderately to severely inverted T waves. The area of hypertrophied myocardium is confined to a narrow region of the septum or the anterior or lateral wall at the apical level (non-spade apical hypertrophic cardiomyopathy)(4); 5) The absence of an outflow tract pressure gradient; 6) Mild symptoms; 7) The prognosis of ApHCM with regard to SCD is believed to be better than that of common HCM. Patients with the ApHCM had a benign clinical course. However, the mutation Arg719Trp in the cardiac beta-myosin heavy chain (beta MHC) gene is a high risk factor for sudden death and can be associated with an unusual ApHCM(5); 8) Progressing to myocardial necrosis and aneurysm formation because of the chronic myocardial ischemia at the apex eventually is observed (6) 9) 123I-MIBG imaging revealed regional sympathetic denervation in the inferior and lateral regions. Recent observations suggest that the risk of SCD might be increased not only in common HCM, but also in Japanese-type ApHCM (7). PES demonstrated reproducible induction of VF in aborted SD and presyncopal patients, resulting in the need for an ICD and amiodarone. Patients with refractory atrial fibrillation with a rapid ventricular response suffered from serious congestive heart failure. A prudent assessment and strategy in patients with this disease would be indispensable in avoiding a disastrous outcome. To clarify the mechanisms of ECG abnormalities in hypertrophic cardiomyopathy, 102 patients were examined with CMR. Distribution and magnitude of hypertrophy and late-enhancement were correlated with ECG abnormalities: 1) Abnormal Q waves reflect the interrelation between upper anterior septal thickness and other regions of the left and right ventricles, and wider Q waves are associated with late-enhancement; 2) Conduction disturbances and absent septal Q waves are associated with late-enhancement; 3) The depth of negative T waves is related to craniocaudal asymmetry and apical late-enhancement (8) As many as 25% of Japanese patients with HCM have predominately apical involvement. Despite its low incidence, physicians caring for patients with chest pain need to consider ApHCM, in their differential diagnosis(9). In ApHCM, sustained cavity obliteration is an important pathophysiologic condition as well as hypertrophy, ischemia, and prolonged QTc, which are considered jointly related to the development of aneurysm through interactions(10). References 1) Arad M, Penas-Lado M, Monserrat L, et al. Gene mutations in apical hypertrophic cardiomyopathy. Circulation. 2005; 112:2805-2811 2) Olson TM, Doan TO, Kishimoto NY, et al. Inherited and de novo mutations in the cardiac actin gene cause hypertrophic cardiomyopathy. J Mol Cell Cardiol. 2000;32:1687-1694. 3) Kitaoka H, Doi Y, Casey SA, Comparison of prevalence of apical hypertrophic cardiomyopathy in Japan and the United States. Am J Cardiol. 2003;92:1183-1186. 4) Suzuki J, Watanabe F, Takenaka K, et al. New subtype of apical hypertrophic cardiomyopathy identified with nuclear magnetic resonance imaging as an underlying cause of markedly inverted T waves J Am Coll Cardiol. 1993;22:1175-1181; 5) Dohlemann C, Hebe J, Meitinger T, Apical hypertrophic cardiomyopathy due to a de novo mutation Arg719Trp of the beta-myosin heavy chain gene and cardiac arrest in childhood. A case report and family study. 6) Marcu CB, Kapoor A, Donohue TJ Apical aneurysm in a patient with apical hypertrophic cardiomyopathy. Conn Med. 2006; 70:297-300. 7) Ridjab D, Koch M, Zabel M, Schultheiss HP, Morguet AJ.Cardiac Arrest and Ventricular Tachycardia in Japanese-Type Apical Hypertrophic Cardiomyopathy. Cardiology. 2006; 107:81-86. 8) Dumont CA, Monserrat L, Soler R, et al .Interpretation of electrocardiographic abnormalities in hypertrophic cardiomyopathy with cardiac magnetic resonance. Eur Heart J. 2006; 27:1725-1731. 9) Iskandar SB, Dittus K, Merrick D. Uncommon cause of a common disease. South Med J. 2003;96:828-831 10) Matsubara K, Nakamura T, Kuribayashi T, et al.Sustained cavity obliteration and apical aneurysm formation in apical hypertrophic cardiomyopathy. Am Coll Cardiol. 2003;42:1338. All the best Andr?s Ricardo P?rez Riera Chief of Electro-Vectocardiology Sector of the Discipline of Cardiology, ABC Faculty of Medicine (FMABC), Foundation of ABC (FUABC) - Santo Andr? - Sao Paulo - Brazil. Rua Sebastiao Afonso 885 - Zip Code: 044417-100- Jardim Miriam S.P Brazil El 21/10/2006, a las 20:53, SCD Symposium escribi?: > Hello, friends from the cyberspace, > Congratulations and thank you for this new great event. I would like > to ask about the best management to follow in the following clinical > case: this is a 56-year-old patient, who consulted 10 years ago as a > routine. In that moment he had a very pathological ECG, with negative > and huge T waves in all the anterior side. We conducted ergometer > test, 2D echo, Holter and coronary angiography, and all of them > resulted normal. Since then studies have been repeated annually, and > he remains completely asymptomatic. The ECG persists with no changes > and the two last echoes report: apical ventricular hypertrophy. A > spect gamma camera does not report the alleged apical hypertrophy. We > have scheduled a cardiac resonance. The question is: should this > patient receive any treatment? For instance, beta blockers? > At first I had thought that this was a case of genetic hypertrophic > cardiomyopathy (troponin?) without phenotype. Now, I'm not sure that's > correct. I would like to have your opinion about what management to > follow. The father of the patient died when he was 56 years old, > suddenly, but he ignores if the father was a coronary patient, and the > sons of the patient do not show any pathology to this date. Thank you > very much, > Luis Wolman -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee From info at scd-symposium.org Sun Oct 22 13:39:53 2006 From: info at scd-symposium.org (SCD INFO) Date: Sun, 22 Oct 2006 13:39:53 -0300 Subject: [SCD-FORUM] 88E RE: HCM in 49-year-old man. Dr. Rigopoulos Message-ID: <453B9ED9.2000406@scd-symposium.org> Dear Dr. de la Torre, This is really an interesting case. After excluding coronary artery disease in this patient, an option before any intervention would be to change his medication to verapamil or add disopyramide to the propranolol. If the patient still remains symptomatic, he should either have myectomy or a septal ablation procedure, based mainly on the morphologic appearance of the septum. Palpitations should, however, be further clarified. Has the patient had any Holter recording? It would be very heplful to know if he has VT bursts or only supraventricular runs. Dr. Angelos Rigopoulos 2nd Department of Cardiology University of Athens Medical School Athens, Greece > First of all, greetings. Congratulations for such an interesting event. > I have a case I would like to share. It is a young man (49 years old) > with hypertrophic cardiomyopathy diagnosed years ago. > Now he is very symptomatic, with angina in rest and crises of > palpitations accompanied by dizziness, and we could not document which > type they are. In echocardiogram I have found besides septal > hypertrophy of 23 mm, double gradient, one in LVOT, peak of 68 mmHg, > and another intraventricular, of 145 mmHg. He is being treated with > propranolol 40 mgs per 8 hours. > My question is: would he benefit with a dual chamber pacemaker? Would > it be essential to implant ICD? Which of the options is more > appropriate (pacemaker, myectomy, or septal ablation)? > Once again, congratulations, > Dr. Rafael S. Le?n de la Torre > Cardi?logo-Ecocardiografista > Centro de atenci?n cardiovascular de Camaguey > Cuba -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee From miayala at gigared.com Sun Oct 22 14:43:23 2006 From: miayala at gigared.com (Maria Isabel Ayala) Date: Sun, 22 Oct 2006 14:43:23 -0300 Subject: [SCD-FORUM] EXPERTS ASK, EXPERTS ANSWER Message-ID: <20061022174043.BDFDF5AFA@n5.gigared.com> Dr. Leonid Makarov from Russia asks - What patients have indication for molecular genetic studies? In the case of detection of genetic mutations, characteristic for short or long QT syndrome, Brugada syndrome, Arrhythmogenic Right Ventricular Dysplasia, would it be ethical to inform all patients about these findings? Dr. Alejandra Guerchicoff, Dr. Guido D. Pollevick and Dr. Charles Antzelevitch answer - Genetic tests are done to confirm a suspected diagnosis in a proband with clinical signs and symptoms of the disease, to predict the possibility of future illness in family members, to detect the presence of a carrier state in unaffected individuals (whose children may be at risk), and to predict the response to therapy or qualify a patient for gene-specific therapy, particularly in syndromes such as long QT syndrome. Genetic testing is also critically important to advancing research aimed at better diagnosis and treatment of the syndrome. Genetic tests involving the channelopathies you mention are best done under the supervision of an electrophysiologist, ideally with the assistance and support of a certified genetic counselor. Receiving test results can be an emotional experience and the collaboration of the physician with a genetic counselor can help guide the patient and his/her family through a difficult time and assist with decisions as to whether extend the test to other family members. Privacy issues are paramount in any discussion involving notification of patients of the results of genetic testing. All patients tested must give informed consent before the test can be carried out. In so doing, they can elect to be notified of the results or not. Their individual rights must be respected. Most countries have laws that protect the privacy of individuals undergoing genetic testing, preventing release of the results to anyone without specific, written consent from the patient. From info at scd-symposium.org Sun Oct 22 23:20:17 2006 From: info at scd-symposium.org (SCD Symposium) Date: Sun, 22 Oct 2006 23:20:17 -0300 Subject: [SCD-FORUM] Bibliographical update Message-ID: <2F147D8D-331F-41FD-8758-9EB4632C6EC6@scd-symposium.org> Dear colleagues, We include instructions to access the bibliography of this last week. We hope this will be of your interest. To access the bibliography http://www.scd-symposium.org/files/2006_10_21.html Kind regards, Dr. Edgardo Schapachnik - Dr. Sergio Dubner -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061022/aca7d86d/attachment.html From info at scd-symposium.org Mon Oct 23 16:03:09 2006 From: info at scd-symposium.org (SCD Symposium) Date: Mon, 23 Oct 2006 16:03:09 -0300 Subject: [SCD-FORUM] 80S RE: HCM in 49-year-old man. Dr. Rondon In-Reply-To: References: Message-ID: <1E1E838A-68A9-4B39-9094-0D8A39B3622E@scd-symposium.org> Dear Dr. Rafael S Leon de la Torre: Interesting case and I dare to give my viewpoint, because in our Hospital we have had a casuistics (> 40 cases) of HOCM with cases of significant gradient (up to 130 mmHg) that have remarkably improved with pacemaker implantation in the RV tip with short AV delay to prevent entry of its own rhythm, and if is possible, to inhibit its triggering with oral beta blockers of the Atenolol type (1 or 2 intakes a day for convenience). Nowadays, our echocardiography specialists do not allow us to implant pacemakers in these patients, since with septal ablation with absolute alcohol with echocardiographic sectorization with intracoronary Acuson, we have an excellent response of decrease and even disappearance of gradients. As regards myomectomy, we do not have any experience, even when we know that it is the most appropriate management, when by using other methods we have had no results, but in our hospital, as far as I know, there are only two cases performed over the last 20 years ? therefore we cannot speak of experience. The complication in your case is the presence of double gradient, which delimits the presence of nonlocated septal hypertrophy towards the LVOT but midseptal plus the one from the outflow that keep the major basal intermediary gradient? I think that the characterization with Acuson may be of great help in locations that could be ablated and have a lower degree of gradient in this patient. We are very near and we can share this case study with you in our University Hospital of Caracas, for which I'm at your disposal to help your patient. Regards, Dr Mauricio Rond?n -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee > > First of all, greetings. Congratulations for such an interesting > event. > I have a case I would like to share. It is a young man (49 years > old) with hypertrophic cardiomyopathy diagnosed years ago. > Now he is very symptomatic, with angina in rest and crises of > palpitations accompanied by dizziness, and we could not document > which type they are. In echocardiogram I have found besides septal > hypertrophy of 23 mm, double gradient, one in LVOT, peak of 68 > mmHg, and another intraventricular, of 145 mmHg. He is being > treated with propranolol 40 mgs per 8 hours. > My question is: would he benefit with a dual chamber pacemaker? > Would it be essential to implant ICD? Which of the options is more > appropriate (pacemaker, myectomy, or septal ablation)? > Once again, congratulations, > Dr. Rafael S. Le?n de la Torre > Cardi?logo-Ecocardiografista > Centro de atenci?n cardiovascular de Camaguey > Cuba > > -- > Dr. Sergio Dubner > President of Scientific Committee > > Dr. Edgardo Schapachnik > President of Steering Committee > > > > > _______________________________________________ > Scd-forum mailing list > Scd-forum at scd-symposium.org > http://www.grupoakros.com.ar/mailman/listinfo/scd-forum -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061023/4040f78a/attachment.html From info at scd-symposium.org Mon Oct 23 21:58:06 2006 From: info at scd-symposium.org (SCD Symposium) Date: Mon, 23 Oct 2006 21:58:06 -0300 Subject: [SCD-FORUM] 92E RE: Brugada's pattern detection in a son of SCD's patient. Dr. Silvia Priori In-Reply-To: References: Message-ID: Dear Sergio, dear Dr Cagnolatti, I do not think that it is possible to conlcude that the index case has Brugada Syndrome. Based on the description the 54 year old man has no ECG pattern suggestive for Brugada Syndrome, he has no typical arrhythmic event (monomorphic VT is no typical of the syndrome) . Obviously the pharmacological challenge (at least with procainamide if in the country of Dr Cagnolatti ajmaline or flecainide are not available) should be performed. I presume that Dr Cagnolatti has excluded short QT Syndrome, long QT, CPVT and the other inherited arrhythmogenic syndromes. I would like to know the trigger for the first cardiac arrest, if the patient had experienced syncopal events or palpitations before the cardiac arrest: such information may help assess the probability that the patient has Brugada Syndrome. I would also try to get an ECG other family members (does the patient have brothers and sisters?) to evaluate if in the family there are other individuals with an ECG suggestive for Brugada Sydnrome. Obviously the management of the son is the fundamental clinical challenge. If the boy is asymptomatic, even in the presence of a spontaneous pattern, I would do nothing else than monitoring his clinical course. I would consider the implant of a Reveal subcutaneous events monitor just to be sure that there are no arrhythmic events. I think that data from several labs concur nowadays to dicourage programmed electrical stimulation for risk stratification, but of course Dr Cagnolatti should decide whether he wants to propose this option to his patient: I would not. Silvia G Priori, MD;PhD > > Dear Sergio, > About a case of reanimation of sudden death in a 54-year-old > patient, with no structural heart disease, with all the studies > made: coronary angiography, MNR, Holter, EPS. > Everything appears to be normal. Monomorphic arrhythmic storm is > detected at the moment the patient is admitted. The patient is > provided life support and he recovers in 24 hs. ICD implantation is > decided. After three months he displays three shocks by arrhythmic > storm, incessant ventricular tachycardia. Mapping and focus > ablation is decided, in outflow tract with mismatch. > To this moment, the patient has not displayed any event. It's been > three months, and in a Holter register in one of his sons, typical > Brugada pattern is observed. The patient is 17 years old, > asymptomatic. I cannot perform pharmacological test. What should I > do next? > > Dr. Cagnolatti > > -- > Dr. Sergio Dubner > President of Scientific Committee > > Dr. Edgardo Schapachnik > President of Steering Committee > > > > > _______________________________________________ > Scd-forum mailing list > Scd-forum at scd-symposium.org > http://www.grupoakros.com.ar/mailman/listinfo/scd-forum -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061023/fee96ba8/attachment.html From info at scd-symposium.org Mon Oct 23 22:20:20 2006 From: info at scd-symposium.org (SCD Symposium) Date: Mon, 23 Oct 2006 22:20:20 -0300 Subject: [SCD-FORUM] 93C RE: Comments to clinical case Cuilan Li et al. Clinical patient with catecholamine sensitive polymorphic VT. Dr. Cuilan Li to Dr. Makarov In-Reply-To: References: Message-ID: Dear Dr. Makarov: About the essentiality to perform diagnostic studies in family members of a victim of SD by noncoronary origin and had no background of cardiological disease, from my point of view, they really need it. We saw a 28-y-o young man last year who asked for help to make sure if he has the risk to die from heart disease. The reason is that his mother had SD when she was riding bicycle on the going to work road and his younger sister had SD when she was doing physical exercise in Junior High School. The young man and his father were scared by these events. Fortunately, after doing examinations such as 12 lead ECG, Holter, Exercise test, Echocardiogram, no abnormality was found for him and his father, suggesting that the carriers of disease-causing gene (whatever they suffer from) might all gone and the left people may not be a carrier. The first three useful diagnostic studies should be ECG (sometimes including Holter recording), Exercise test, Echocardiogram. Based on the indication from these data, further study such as EPS may be considered. Cuilan Li Cuilan Li, Ph. D Department of Cardiology, Peking University People?s Hosp., Beijing 100044, China Tel: 86-10-68314422 Ext. 5940 licuilan at gmail.com -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee > > Dear collegues! I want to add my comments to clinical case thad > presented Dr. Cuilan Li et al. ?Clinical presentation and > management of a patient with catecholamine sensitive polymorphic > ventricular tachycardia (CPVT)?. By my mention it is typical > clinical and ECG pattern for children and young patients with some > of kind of catecholaminergic VT (CVT) that we described before > (Makarov L. et al. Short PR interval, high circadian index and > bradycardia ? pattern with high risk of syncope and sudden death in > children with catecholaminergic ventricular tachycardia. Europ > Heart J 2004; 25: 222, Suppl. Abstract) and during this symposium: > bidirectional VT, bradycardia, short PR interval, and double > tachycardia (VT and SVT). Some of this patients also demonstrated > of the shortening of the QT interval (possibly as result of calcium > overload of cardiomyocites). I am sure in arrythmogenic origin of > syncope in present patient and useful of the active anthyarrhythmic > therapy for it. From drugs more effective Beta-blockers (1-2 mg/ > kg) or/and calcium antagonists (verapamil), but authors had > detected proarrhythmia from verapamil, that not typical for our > experience. In the some patients the propafenone can be effective. > Most of our patients with CVT showed improvement in their clinical > symptoms: syncope events and ventricular tachycardia did not recur. > But in a numerous patients that tolerant to therapy with recurrent > syncope implantation of the ICD could be useful. I am not sure in > useful of the EPI study for this patient, repetitive of Holter, > stress test and/or IV infusion of isoproterenol more frequently > detected typical bidirectional VT. Concern authors question # 3 > (The catecholamine concentration in blood was normal for this > patient. What do we expect of the catecholamine concentrations in > CPVT patients in general?)- Fisher J.et al. (JACC1999; 34 (7): > 2015-22) showed normal or lower level of plasma catecholapimes in > rest in this patients, but parid increasing of it during exercise, > to compare with normal range. > > Dr. Leonid Makarov M.D., Ph.D. professor (Pediatry) > > Moscow Institute pediatry and children surgery. > > 125412 Taldomskaya str.2 , Moscow, Russia. > > leo at oss.ru > > > -- > Dr. Sergio Dubner > President of Scientific Committee > > Dr. Edgardo Schapachnik > President of Steering Committee > > > > > _______________________________________________ > Scd-forum mailing list > Scd-forum at scd-symposium.org > http://www.grupoakros.com.ar/mailman/listinfo/scd-forum -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061023/9907e219/attachment.html From info at scd-symposium.org Tue Oct 24 07:43:50 2006 From: info at scd-symposium.org (SCD Symposium) Date: Tue, 24 Oct 2006 07:43:50 -0300 Subject: [SCD-FORUM] 95C RE: Case: Male, 29yrs. Dr. Xueshan In-Reply-To: <5D20FB2D-014F-4979-8DDF-90928BDE6386@scd-symposium.org> References: <5D20FB2D-014F-4979-8DDF-90928BDE6386@scd-symposium.org> Message-ID: <5B5BCBFF-7E2B-4D87-BAB0-3CFF871E6942@scd-symposium.org> Try Radiofrequency Catheter Ablation again, and noursih the myocardium. Dr. Xueshan ?? ?? -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee > > Case: Male, 29yrs > > Illness history: Viral myocarditis was diagnosed based on premature > ventricular contractions (PVCs) at age 8 after an influenza attack. > Since then, PVBs never disappeared and right ventricle enlargement > had been noticed. > > ECG revealed VT lasting 3 minutes at age 19, accompanied with > faintness. After 2-3 years, no VT reoccurred. > > The second VT episode was recorded at age 21 lasting 2-3 hrs > accompanied with syncope. Intravenous propafenon worked. Oral > administration of propafenon could not prevent the patient from VT > attack. > > RFCA for VT had been delivered twice at age 22~23 by Pro. Yang at > the first affiliated Hospital of Dalian medical university. During > RFCA, VT could not be induced and cardiomyopathy was diagnosed by > endocardiac biopsy. > > > VTs occurred intermediately lasting several seconds to a few > minutes. VT restored to sinuous rhythm either spontaneously or by > intravenous propafenon administration. VT usually occurred at > midnight or early morning. At the following years, Sometimes VT > occurred quite often during a certain period (for example: twice > attacks in Winter), and then no occurrence for several months or > even longer time. The patient enjoys doing exercise. No VT attacked > during exercise. No restriction to exercise. > > Since 27 yrs old (2004), propafenon has been administrated in the > doses of 150mg bid or tid. Drug quitted after 1-2 months > administrations, keeping no VT attack for 0.5-1.5 yrs. > Since last year, the drug cannot be quitted, otherwise VT > reoccurred. So far, the doses of the drug are 200mg at early > morning and 150mg at night. Lower dosages may not prevent the VTs. > > Any suggestion about the treatment? > > Zhu > -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061024/b955c275/attachment.html From info at scd-symposium.org Tue Oct 24 07:16:35 2006 From: info at scd-symposium.org (SCD Symposium) Date: Tue, 24 Oct 2006 07:16:35 -0300 Subject: [SCD-FORUM] 89S Prophylactic ICD in nonobstructive asymmetrical Idiopathic Hypertrophic Cardiomyopathy? Dr. Landaeta In-Reply-To: Message-ID: Dear attendants to this great symposium on the Internet, I wish to pose the case of a 50-year-old female patient with first episode (3 years ago) of paroxysmal atrial fibrillation with rapid ventricular response (170 b/min), which was accompanied by precordial oppression and prolonged presyncope. She has been receiving treatment with verapamil, atenolol, separately or combined, inconstantly. In a recurrent way, she presents clinics of ?palpitations? related to paroxysmal AF in several ECG recordings, now with preserved ventricular response. The resting ECG shows a sinus rhythm pattern, and a normal one. TT echocardiogram shows baseline Septum Hypertrophy of 15 mm (asymmetrical) without Intraventricular or transvalvular gradients; the left atrium slightly dilated, the LVEF is preserved. The functional class is I according to the NYHA. She denies family history of ?sudden? death due to any cause. I am thinking of starting treatment with amiodarone to prevent recurrences of AF. However, what role may the indication of an ICD have to prevent SCD on this patient? Which would be the other risk stratification tests for SD applicable in this situation? Thank you very much, Dr. C?sar Landaeta T. Cardiologo Electrofisi?logo Venezuela -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061024/4bc07eca/attachment.html From info at scd-symposium.org Tue Oct 24 10:15:51 2006 From: info at scd-symposium.org (SCD Symposium) Date: Tue, 24 Oct 2006 10:15:51 -0300 Subject: [SCD-FORUM] =?utf-8?q?96C_RE=3A_Case=3A_Male=2C_29yrs=2E_Dr=2E__?= =?utf-8?b?5ZGo6KOU5b+g?= In-Reply-To: <5D20FB2D-014F-4979-8DDF-90928BDE6386@scd-symposium.org> References: <5D20FB2D-014F-4979-8DDF-90928BDE6386@scd-symposium.org> Message-ID: <14C2F2C3-DA2E-4190-A474-C3962E871DE2@scd-symposium.org> The patient might be ARVD, and ICD implantation might be required. -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee > > Case: Male, 29yrs > > Illness history: Viral myocarditis was diagnosed based on premature > ventricular contractions (PVCs) at age 8 after an influenza attack. > Since then, PVBs never disappeared and right ventricle enlargement > had been noticed. > > ECG revealed VT lasting 3 minutes at age 19, accompanied with > faintness. After 2-3 years, no VT reoccurred. > > The second VT episode was recorded at age 21 lasting 2-3 hrs > accompanied with syncope. Intravenous propafenon worked. Oral > administration of propafenon could not prevent the patient from VT > attack. > > RFCA for VT had been delivered twice at age 22~23 by Pro. Yang at > the first affiliated Hospital of Dalian medical university. During > RFCA, VT could not be induced and cardiomyopathy was diagnosed by > endocardiac biopsy. > > > VTs occurred intermediately lasting several seconds to a few > minutes. VT restored to sinuous rhythm either spontaneously or by > intravenous propafenon administration. VT usually occurred at > midnight or early morning. At the following years, Sometimes VT > occurred quite often during a certain period (for example: twice > attacks in Winter), and then no occurrence for several months or > even longer time. The patient enjoys doing exercise. No VT attacked > during exercise. No restriction to exercise. > > Since 27 yrs old (2004), propafenon has been administrated in the > doses of 150mg bid or tid. Drug quitted after 1-2 months > administrations, keeping no VT attack for 0.5-1.5 yrs. > Since last year, the drug cannot be quitted, otherwise VT > reoccurred. So far, the doses of the drug are 200mg at early > morning and 150mg at night. Lower dosages may not prevent the VTs. > > Any suggestion about the treatment? > > Zhu -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061024/b44cc5b2/attachment.html From info at scd-symposium.org Tue Oct 24 07:17:34 2006 From: info at scd-symposium.org (SCD Symposium) Date: Tue, 24 Oct 2006 07:17:34 -0300 Subject: [SCD-FORUM] 90S Hypertrophic cardiomyopathy. Dr. Pellegrini In-Reply-To: <90DEDEC1-3C74-4BC6-B7EB-CAB0B4D821D5@grupoakros.com.ar> Message-ID: <8224B157-F490-4CFC-9960-30AB53A81011@scd-symposium.org> Good afternoon. I would like to read opinions about: what patient with hypertrophic cardiomyopathy should undergo a genetic study? Thank you very much, Dr. Gabriel Pellegrini. La Plata. Argentina. -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061024/be2d3b96/attachment.html From info at scd-symposium.org Tue Oct 24 13:55:45 2006 From: info at scd-symposium.org (SCD Symposium) Date: Tue, 24 Oct 2006 13:55:45 -0300 Subject: [SCD-FORUM] 98E RE: ICD implantation in children. Dr. Saul In-Reply-To: References: Message-ID: Although I do not have any direct experience with a shock coil in the IVC, there are many techniques described for smaller children, many of which have utility because the DFT's in children are generally lower. I think the preference of many peds EP docs in the smaller child is to avoid the intravascular space completely for at least the shock coils. Described options include: - Subcutaneous coils in the back (single lead, array or patch) - An endocardial coil passed thru the inferior pericardium, around the heart and screwed in superiorly near the pulmonary veins. - A patch or coil sewed onto the pleura between lung segments posterior to the heart. - Cans placed in the abdominal wall or between the pericardium and pleura inferiorly. The bottom line is anything that avoids a shock lead in the RV is preferred for patients not near adult size, and may even be better for young patients near adult size due to the issues with long term lead maintainance. J. Philip Saul, M.D. Director, The Children's Heart Program of South Carolina Physician-in-Chief, MUSC Children's Hospital Medical University of South Carolina > > ICD implantation in children is a major problem. The first problem > is the implantation of the ICD lead inside the right ventricle with > coil unfitted for the size of the cavity and development of > adherences between the coil and the tricuspid valve. In this > population we will have to extract these leads during the FU of > these patients and in our experience this extraction is challenging > even on recent leads. Hence, we have recently used the technique > describe by GASPARINI et al (J Cardiovasc Electrophysiol, Vol. 16, > pp. 1381-1383) with a coil in the inferior vena-cava. What is your > experience with this technique? > > Didier Klug, MD, PhD > H?pital cardiologique de Lille > University of Lille > France -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061024/ce65bb2d/attachment.html From info at scd-symposium.org Tue Oct 24 16:15:49 2006 From: info at scd-symposium.org (SCD Symposium) Date: Tue, 24 Oct 2006 16:15:49 -0300 Subject: [SCD-FORUM] 91S RE: Prophylactic ICD in nonobstructive asymmetrical Idiopathic Hypertrophic Cardiomyopathy? Dr. Rondon In-Reply-To: References: Message-ID: Cesar, As you well asked, in risk stratification for patients with nonobstructive hypertrophic cardiomyopathy: absence of familial sudden death absence of evidence of malignant ventricular arrhythmia in Holter or EP lab absence of late potentials in signal-averaged ECG preserved LV function absence of IV gradient FC I Rules out to a certain extent SCD risk in this population? now, what you have to try is for the patient to understand that he or she must have his/her treatment in a methodical way. A significant attempt is to find fractionated potentials and verify the possibility of pulmonary veins isolation, by ablation with Ensite system, to decrease even more the risk of rapid events of AF ? Regards, Mauricio Rond?n -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee > > Dear attendants to this great symposium on the Internet, > I wish to pose the case of a 50-year-old female patient with first > episode (3 years ago) of paroxysmal atrial fibrillation with rapid > ventricular response (170 b/min), which was accompanied by > precordial oppression and prolonged presyncope. She has been > receiving treatment with verapamil, atenolol, separately or > combined, inconstantly. In a recurrent way, she presents clinics of > ?palpitations? related to paroxysmal AF in several ECG recordings, > now with preserved ventricular response. The resting ECG shows a > sinus rhythm pattern, and a normal one. TT echocardiogram shows > baseline Septum Hypertrophy of 15 mm (asymmetrical) without > Intraventricular or transvalvular gradients; the left atrium > slightly dilated, the LVEF is preserved. The functional class is I > according to the NYHA. She denies family history of ?sudden? death > due to any cause. I am thinking of starting treatment with > amiodarone to prevent recurrences of AF. However, what role may the > indication of an ICD have to prevent SCD on this patient? Which > would be the other risk stratification tests for SD applicable in > this situation? > > Thank you very much, > Dr. C?sar Landaeta T. > Cardiologo Electrofisi?logo > Venezuela > > -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061024/f4671634/attachment.html From info at scd-symposium.org Tue Oct 24 16:25:44 2006 From: info at scd-symposium.org (SCD Symposium) Date: Tue, 24 Oct 2006 16:25:44 -0300 Subject: [SCD-FORUM] 99C RE: Case: Male, 29yrs. Dr. Zhu's ECGs In-Reply-To: <5D20FB2D-014F-4979-8DDF-90928BDE6386@scd-symposium.org> References: <5D20FB2D-014F-4979-8DDF-90928BDE6386@scd-symposium.org> Message-ID: <4D683A05-2B30-4285-A1B7-F732C77916D1@scd-symposium.org> SEE THE ECGs http://www.scd-symposium.org/files/zhu1.jpg (1.6 Mb) http://www.scd-symposium.org/files/zhu2.jpg (4.2 Mb) -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee > > Case: Male, 29yrs > > Illness history: Viral myocarditis was diagnosed based on premature > ventricular contractions (PVCs) at age 8 after an influenza attack. > Since then, PVBs never disappeared and right ventricle enlargement > had been noticed. > > ECG revealed VT lasting 3 minutes at age 19, accompanied with > faintness. After 2-3 years, no VT reoccurred. > > The second VT episode was recorded at age 21 lasting 2-3 hrs > accompanied with syncope. Intravenous propafenon worked. Oral > administration of propafenon could not prevent the patient from VT > attack. > > RFCA for VT had been delivered twice at age 22~23 by Pro. Yang at > the first affiliated Hospital of Dalian medical university. During > RFCA, VT could not be induced and cardiomyopathy was diagnosed by > endocardiac biopsy. > > > VTs occurred intermediately lasting several seconds to a few > minutes. VT restored to sinuous rhythm either spontaneously or by > intravenous propafenon administration. VT usually occurred at > midnight or early morning. At the following years, Sometimes VT > occurred quite often during a certain period (for example: twice > attacks in Winter), and then no occurrence for several months or > even longer time. The patient enjoys doing exercise. No VT attacked > during exercise. No restriction to exercise. > > Since 27 yrs old (2004), propafenon has been administrated in the > doses of 150mg bid or tid. Drug quitted after 1-2 months > administrations, keeping no VT attack for 0.5-1.5 yrs. > Since last year, the drug cannot be quitted, otherwise VT > reoccurred. So far, the doses of the drug are 200mg at early > morning and 150mg at night. Lower dosages may not prevent the VTs. > > Any suggestion about the treatment? > > Zhu -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061024/8177b194/attachment.html From info at scd-symposium.org Tue Oct 24 17:27:22 2006 From: info at scd-symposium.org (SCD Symposium) Date: Tue, 24 Oct 2006 17:27:22 -0300 Subject: [SCD-FORUM] 94C Molecular genetic studies. Dr. Liu In-Reply-To: <20061022174043.BDFDF5AFA@n5.gigared.com> References: <20061022174043.BDFDF5AFA@n5.gigared.com> Message-ID: <962F2C1D-B267-4F28-926E-918A231E4DC2@scd-symposium.org> Dear Dr. Alejandra Guerchicoff, Dr. Guido D. Pollevick, Dr. Charles Antzelevitch: I have a question, this is the problem I met in clinical practice last year. If the one who was found having mutation, but he or she did not want to tell his or her spouse, how shall we do? All the family members have been screened, and every one want know the result. Sincerely Yours Wenling Liu from Beijing Univ.People's Hosp. -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee > > Dr. Leonid Makarov from Russia asks > > - What patients have indication for molecular genetic studies? In > the case of detection of genetic mutations, characteristic for > short or long QT syndrome, Brugada syndrome, Arrhythmogenic Right > Ventricular Dysplasia, would it be ethical to inform all patients > about these findings? > > Dr. Alejandra Guerchicoff, Dr. Guido D. Pollevick and Dr. Charles > Antzelevitch answer > - Genetic tests are done to confirm a suspected diagnosis in a > proband with clinical signs and symptoms of the disease, to predict > the possibility of future illness in family members, to detect the > presence of a carrier state in unaffected individuals (whose > children may be at risk), and to predict the response to therapy or > qualify a patient for gene-specific therapy, particularly in > syndromes such as long QT syndrome. Genetic testing is also > critically important to advancing research aimed at better > diagnosis and treatment of the syndrome. Genetic tests involving > the channelopathies you mention are best done under the supervision > of an electrophysiologist, ideally with the assistance and support > of a certified genetic counselor. > Receiving test results can be an emotional experience and the > collaboration of the physician with a genetic counselor can help > guide the patient and his/her family through a difficult time and > assist with decisions as to whether extend the test to other family > members. > Privacy issues are paramount in any discussion involving > notification of patients of the results of genetic testing. All > patients tested must give informed consent before the test can be > carried out. In so doing, they can elect to be notified of the > results or not. Their individual rights must be respected. Most > countries have laws that protect the privacy of individuals > undergoing genetic testing, preventing release of the results to > anyone without specific, written consent from the patient. -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061024/400493c0/attachment.html From info at scd-symposium.org Tue Oct 24 17:21:50 2006 From: info at scd-symposium.org (SCD Symposium) Date: Tue, 24 Oct 2006 17:21:50 -0300 Subject: [SCD-FORUM] 71S HCM in 49-year-old man. Dr. Madias In-Reply-To: References: Message-ID: Dear Dr. Rafael S Leon de la Torre: It is possible that your very interesting patient with symptomatic HOCM may be benefited from a comprehensive tierred management approach, which could include: 1) Continuation of the current drug regimen. 2) Echo-based assessment of the stability and location of the gradients at rest, and following a stress test. 3) Insertion of a dual chamber temorary pacing system, with echo-based assessment of the gradients at rest and after exercise, employing a range of AV delays. 4) If unsuccessful with the above, consider alcohol septal ablation, with post-ablation echo-based assessment of the gradient(s), at rest and after stress testing. 5) If still there is a persistence of gradient(s), you should refer your patient to a center experienced in myectomy. Warmest Regards, John E. Madias, MD, FACC, FAHA, Professor of Medicine (Cardiology) Mount Sinai School of Medicine of the New York University, Cardiology Division, Elmhurst Hospital Center Tel:(718) 334-5005 FAX:(718) 334-5990 email:madias at nychhc.org -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee > > First of all, greetings. Congratulations for such an interesting > event. > I have a case I would like to share. It is a young man (49 years > old) with hypertrophic cardiomyopathy diagnosed years ago. > Now he is very symptomatic, with angina in rest and crises of > palpitations accompanied by dizziness, and we could not document > which type they are. In echocardiogram I have found besides septal > hypertrophy of 23 mm, double gradient, one in LVOT, peak of 68 > mmHg, and another intraventricular, of 145 mmHg. He is being > treated with propranolol 40 mgs per 8 hours. > My question is: would he benefit with a dual chamber pacemaker? > Would it be essential to implant ICD? Which of the options is more > appropriate (pacemaker, myectomy, or septal ablation)? > Once again, congratulations, > Dr. Rafael S. Le?n de la Torre > Cardi?logo-Ecocardiografista > Centro de atenci?n cardiovascular de Camaguey > Cuba > -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061024/f079aafe/attachment.html From info at scd-symposium.org Tue Oct 24 17:34:20 2006 From: info at scd-symposium.org (SCD Symposium) Date: Tue, 24 Oct 2006 17:34:20 -0300 Subject: [SCD-FORUM] 101E RE: Case: Male, 29yrs. Dr. Zhu In-Reply-To: <14C2F2C3-DA2E-4190-A474-C3962E871DE2@scd-symposium.org> References: <5D20FB2D-014F-4979-8DDF-90928BDE6386@scd-symposium.org> <14C2F2C3-DA2E-4190-A474-C3962E871DE2@scd-symposium.org> Message-ID: Except ICD we will have no other way to help him, right? I mean if he doesn?t have enough money, then what we can do for him? He is only 29, Dr. Zhu -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committe > > The patient might be ARVD, and ICD implantation might be required. > > > > >> >> Case: Male, 29yrs >> >> Illness history: Viral myocarditis was diagnosed based on >> premature ventricular contractions (PVCs) at age 8 after an >> influenza attack. Since then, PVBs never disappeared and right >> ventricle enlargement had been noticed. >> >> ECG revealed VT lasting 3 minutes at age 19, accompanied with >> faintness. After 2-3 years, no VT reoccurred. >> >> The second VT episode was recorded at age 21 lasting 2-3 hrs >> accompanied with syncope. Intravenous propafenon worked. Oral >> administration of propafenon could not prevent the patient from VT >> attack. >> >> RFCA for VT had been delivered twice at age 22~23 by Pro. Yang at >> the first affiliated Hospital of Dalian medical university. During >> RFCA, VT could not be induced and cardiomyopathy was diagnosed by >> endocardiac biopsy. >> >> >> VTs occurred intermediately lasting several seconds to a few >> minutes. VT restored to sinuous rhythm either spontaneously or by >> intravenous propafenon administration. VT usually occurred at >> midnight or early morning. At the following years, Sometimes VT >> occurred quite often during a certain period (for example: twice >> attacks in Winter), and then no occurrence for several months or >> even longer time. The patient enjoys doing exercise. No VT >> attacked during exercise. No restriction to exercise. >> >> Since 27 yrs old (2004), propafenon has been administrated in the >> doses of 150mg bid or tid. Drug quitted after 1-2 months >> administrations, keeping no VT attack for 0.5-1.5 yrs. >> Since last year, the drug cannot be quitted, otherwise VT >> reoccurred. So far, the doses of the drug are 200mg at early >> morning and 150mg at night. Lower dosages may not prevent the VTs. >> >> Any suggestion about the treatment? >> >> Zhu > > -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061024/fbff9180/attachment.html From info at scd-symposium.org Tue Oct 24 20:24:19 2006 From: info at scd-symposium.org (SCD Symposium) Date: Tue, 24 Oct 2006 20:24:19 -0300 Subject: [SCD-FORUM] 102E RE: Hypertrophic cardiomyopathy. Dr. Perez Riera In-Reply-To: <8224B157-F490-4CFC-9960-30AB53A81011@scd-symposium.org> References: <8224B157-F490-4CFC-9960-30AB53A81011@scd-symposium.org> Message-ID: <0533D02D-B9D4-415A-A8CD-51F4CB208844@scd-symposium.org> Dr. Gabriel Pellegrini from La Plata. Argentina. I?m Andr?s Ricardo P?rez Riera from Sao Paulo Brazil. Risk stratification in HCM should include a complete clinical- cardiological evaluation that should also consider new diagnostic features, e. g. MRI. Risk stratification should also include genetic testing, since some gene mutations seem to be associated with a higher risk for SCD than others. However, genetic testing in HCM in not yet available on a routine basis. Major risk factors for SCD include (1): 1) A SURVIVED CARDIAC ARREST (VF): The implantation of a ICD is first-line therapy in patients with documented VT/VF or patients who have survived SCD(Secondary prevention of SD). SCD is the most devastating presenting manifestation of HCM. It has the highest incidence in preadolescent and adolescent children and is particularly related to extreme exertion. The risk of SD in children is as high as 6% per year. In more than 80% of cases, the arrhythmia that causes sudden death is VF. Many of these cases degenerate into VF from rapid atrial arrhythmias, such as fibrillation, supraventricular tachycardia, or WPW syndrome, while others result from VT and low cardiac output hemodynamic collapse. 2) NON-SUSTAINED AND SUSTAINED VT IN HOLTER MONITORING; 3) FAMILIAL HISTORY OF SD AT A YOUNG AGE: SCD at children, preadolescent and adolescent is not infrequently the first symptom of an inherited cardiac disease. Because these diseases usually inherit as an autosomal dominant trait, first-degree family members have a 50% chance of carrying the same genetic defect. Besides clinical cardiologic examination of the remaining family members, post-mortem molecular genetic investigation can be of value in reaching a diagnosis and in determining the subsequent therapeutic options for immediate relatives(2); 4) UNEXPLAINED SYNCOPE:It occurs more commonly in children and young adults with small LV chamber size and evidence of VT upon ambulatory monitoring. Syncope resulting from inadequate cardiac output upon exertion or from cardiac arrhythmia; 5) AN ABNORMAL BLOOD PRESSURE RESPONSE ON EXERCISE; 6) LEFT VENTRICULAR THICKNESS GREATER OR EQUAL THAN 30mm: Surgical myectomy is effective in reducing symptoms in children with LV obstruction who are unresponsive to drugs. The percutaneous septal ablation can also be regarded as a feasible alternative; 7) THE PRESENCE OF "MALIGNANT" MUTATIONS: mutations (R403Q, R453C, G716R and R719W) are highly malignant defects in the beta- myosin heavy chain (MYH7). In the cardiac troponin T gene (TNNT2), a specific mutation (R92W) has been associated with high risk of SD (3). Genetic testing is not widely available at this time but is becoming increasingly available in this disease setting. In research situations or in larger pedigrees, genotyping is informative for the identification of additional family members once the proband's genotype has been determined. Genetic studies have defined HCM as a disease of the sarcomere caused by mutations in any of 11 genes that encode different elements of the contractile apparatus in cardiac myocytes. Over 200 individual mutations have been identified. With a prevalence estimated to be ~1/500-1/1000 in the general population, HCM is the most common monogenic cardiac disorder. Each factor has a low positive predictive accuracy, but patients having two or more of these risk factors have high risk. The presence of two or more risk factors is associated with a 6-year SCD survival rate of 72%, justifying the consideration of prophylactic therapy. Invasive EPS appear to carry little advantage over non-invasive risk stratification. Other uses for EPS include the investigation and treatment of individuals with conduction disease and/or WPW syndrome, atrial flutter and fibrillation and MVT. Appropriate management may then involve radiofrequency ablation(4). References 1) Pellnitz C, Geier C, Perrot A, et al. Sudden cardiac death in familial hypertrophic cardiomyopathy. Identification of high-risk patients Dtsch Med Wochenschr. 2005;130:1150-1154. 2) Wilde AA, van Langen IM, Mannens MM, Sudden death at young age and the importance of molecular-pathologic investigation Ned Tijdschr Geneeskd. 2005;149:1601-1604; 3) Ackerman MJ, Van Driest SL, Ommen SR, et al. Prevalence and age-dependence of malignant mutations in the beta-myosin heavy chain and troponin T genes in hypertrophic cardiomyopathy: a comprehensive outpatient perspective. J Am Coll Cardiol. 2002; 39:2042-2048. 4) Behr ER, Elliott P, McKenna WJ. et al. Role of invasive EP testing in the evaluation and management of hypertrophic cardiomyopathy. Card Electrophysiol Rev. 2002; 6:482-486. All the best Andr?s Ricardo P?rez Riera Chief of Electro-Vectocardiology Sector of the Discipline of Cardiology, ABC Faculty of Medicine (FMABC), Foundation of ABC (FUABC) - Santo Andr? - Sao Paulo - Brazil. Rua Sebastiao Afonso 885 - Zip Code: 044417-100- Jardim Miriam S.P Brazil- -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee > > > Good afternoon. > I would like to read opinions about: what patient with hypertrophic > cardiomyopathy should undergo a genetic study? > > Thank you very much, > Dr. Gabriel Pellegrini. La Plata. Argentina. -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061024/dd0cab46/attachment.html From info at scd-symposium.org Tue Oct 24 22:13:00 2006 From: info at scd-symposium.org (SCD Symposium) Date: Tue, 24 Oct 2006 22:13:00 -0300 Subject: [SCD-FORUM] EXPERTS ASK, EXPERTS ANSWER Message-ID: <59DF293B-05A6-4082-85CB-C9240659D7D7@scd-symposium.org> Dr. Ariel Szyszko from Argentina asks - Is it necessary to conduct a defibrillation threshold test as a routine in cardioverter defibrillator implants, or are there patients in whom the test's risk/benefit would not be justified? Dr. Alfred Buxton from U.S.A. answers - The risk of performing defibrillation threshold testing is extremely low, and we perform it routinely. We defer performing the test in patients in atrial fibrillation who have not been adequately anticoagulated. One situation in which we do consider deferring the test is in patients with nonischemic dilated cardiomyopathy in whom we have questions about their hemodynamic stability, but in general ICD implants should not be performed in unstable individuals. We repeat the defibrillation test yearly in patients whose threshold at previous tests was >20 joules. We also repast the defibrillation test in patients whose receiving new antiarrhythmic medication such as amiodarone, that can elevate the DFT. Alfred E. Buxton, M.D. Ruth and Paul Levinger Professor of Medicine Director, Division of Cardiology Director, Arrhythmia Services and Electrophysiology Laboratory Brown Medical School Cardiology Division 2 Dudley Street, Suite 360 Providence, RI 02905 Alfred_Buxton at Brown.edu -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061024/6dda178b/attachment.html From info at scd-symposium.org Wed Oct 25 12:22:17 2006 From: info at scd-symposium.org (SCD Symposium) Date: Wed, 25 Oct 2006 12:22:17 -0300 Subject: [SCD-FORUM] 97S Valvular Dilated Cardiomyopathy. Dr. Fernandez In-Reply-To: <95B97B0A-D837-4B56-9BC6-70F1C2713303@grupoakros.com.ar> Message-ID: <708D3A38-09A5-4C01-B299-F17604475A6D@scd-symposium.org> Before anything, I would like to congratulate you for such an achievement. Case: 64-year-old male, with dyslipaemia, HBP, smoker, sedentary, obese, carrier of Valvular Dilated Cardiomyopathy with history of Bentall surgery of Bono N27 plus Venous Bridge to Posterior Diagonal (severe LV), September, 2004. In November, 2004, he presents episode of sudden death, he is implanted an ICD. In January 2005 syncope episode, a SPECT is carried out reporting: medial/apical ischemia, EF 32%, CAG and PTCA are carried out with a STENT to RCA. In June 2005: infection of ICD wires: Exchange of wires and generator and corresponding treatment. In October 2005 he is admitted due to multiple VT episodes and one spurious shock due to AF, in relation to amiodarone decrease from 400 to 200 mg according to the patient; non-dialytic CRF, hyperthyroidism with TSH 0.06 that is why three months ago amiodarone is interrupted and danantizol deltisone is started. In September 2006 he presents electrical storm, treatment is optimized with danantizol 20 mg/12 hs elevating TSH to 0.159, flecainide is interrupted, propafenone and lidocaine are started. He evolves with multiple episodes of NSVT. He presents sustained VT that required shocks. Presently he is on carvedilol 25 mg/12 hs, EV lidocaine. A new CAG is carried out to assess progression of coronary artery disease, which does not account for the triggering of arrhythmia. He is evaluated by the Electrophysiology service staff, who say that there are access difficulties for mapping of ectopic focus and possible ablation. The patient is referred to us to evaluate cardiac transplantation. Dr Carlos Fernandez -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061025/6992fe7c/attachment.html From info at scd-symposium.org Wed Oct 25 15:06:30 2006 From: info at scd-symposium.org (SCD Symposium) Date: Wed, 25 Oct 2006 15:06:30 -0300 Subject: [SCD-FORUM] 103E RE: Prophylactic ICD in nonobstructive asymmetrical Idiopathic Hypertrophic Cardiomyopathy? Dr. Brembilla-Perrot In-Reply-To: References: Message-ID: Dear Dr Dubner and Dr Schapachnick I would like to answer to Dr Landeata. AF is a frequent cause of syncope in HCM especially after 40 years. SD occurring in patients with HCM and AF is generally related to cardiac collapse and rarely due to VF. Therefore the indication of ICD is very debatable in a patient with history of familial SD, no VT on Holter monitoring, 50 years old and with presyncope proved as related to AF. The main problem here is to avoid a rapid AF : Amiodarone and beta blockers are the first choice drugs. Sometimes the association of beta blockers with cibenzoline or disopyramide (shown several years ago to reduce LV obstruction) are useful. More, in patients with refractory AF, curative treatment of atrial arrhythmia by RF catheter ablation is actually indicated. Sincerely yours B Brembilla-Perrot B. BREMBILLA-PERROT, A. JACQUOT, D. BEURRIER, L. JACQUEMIN. Hypertrophic cardiomyopathy : value of atrial programmed electrical stimulation in patients with or without syncope with special reference to the role of atrial arrhythmias. Int J Cardiol 1997 ; 59 : 47-56 -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee > > Dear attendants to this great symposium on the Internet, > I wish to pose the case of a 50-year-old female patient with first > episode (3 years ago) of paroxysmal atrial fibrillation with rapid > ventricular response (170 b/min), which was accompanied by > precordial oppression and prolonged presyncope. She has been > receiving treatment with verapamil, atenolol, separately or > combined, inconstantly. In a recurrent way, she presents clinics of > ?palpitations? related to paroxysmal AF in several ECG recordings, > now with preserved ventricular response. The resting ECG shows a > sinus rhythm pattern, and a normal one. TT echocardiogram shows > baseline Septum Hypertrophy of 15 mm (asymmetrical) without > Intraventricular or transvalvular gradients; the left atrium > slightly dilated, the LVEF is preserved. The functional class is I > according to the NYHA. She denies family history of ?sudden? death > due to any cause. I am thinking of starting treatment with > amiodarone to prevent recurrences of AF. However, what role may the > indication of an ICD have to prevent SCD on this patient? Which > would be the other risk stratification tests for SD applicable in > this situation? > > Thank you very much, > Dr. C?sar Landaeta T. > Cardiologo Electrofisi?logo > Venezuela > > -- > Dr. Sergio Dubner > President of Scientific Committee > > Dr. Edgardo Schapachnik > President of Steering Committee > > > > > _______________________________________________ > Scd-forum mailing list > Scd-forum at scd-symposium.org > http://www.grupoakros.com.ar/mailman/listinfo/scd-forum -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061025/23d06ede/attachment.html From info at scd-symposium.org Wed Oct 25 18:06:38 2006 From: info at scd-symposium.org (SCD Symposium) Date: Wed, 25 Oct 2006 18:06:38 -0300 Subject: [SCD-FORUM] 105E Female patient, 30 years old. Dr. Yunlong Xia Message-ID: Dear Dr. Perez Riera and other colleagues Last week I have met a special case, and here I want to get help from you. Female, 30 years old. She came into my hospital because of black spell and syncope for 2 weeks. The ECG shows atrial standstill and junctional/ventricular escape rhythm of 36, with atrium suspected to be retrograde excited (Fig 1***). After injection of 1 mg atropine for 10-20 min, the escape rate was only slightly increased to 50-55 bpm with standstill atrium (Fig 2***). After that isoprotorenol was intravenous infused to keep escape rate 50-55 bpm. Occasionally, paroxysmal atrial tachycardia/fibrillation and ventricular premature complex could be observed during monitoring, and AV was found not to be 1:1 conducted (Fig. 3***, P wave is clear especially on Lead V1). The routine blood test, heart enzyme, thyroid test and autoimmune system test are all inside normal range. Echocardiography reports the significant tricuspid annulus dilation, with tricuspid insufficiency, medium pulmonary artery hypertension, intra vena cava dilation (IVC 20), right system dilation (RA 52 X 61, RV 21), left atrial dilation (LA 43 X 57) and normal ventricular wall motion. After observation for 2 days, the bradycardia continued and a DDDR pacemaker was implanted, with atrium lead in right appendage. During implantation the atrium was demonstrated to be retrograde activated, but no further EP test was performed. After implantation, the P wave could be only seen positively on lead V1, whereas in other leads it can?t be clearly observed (Fig 4***). Later echocardiography showed relatively weak right atrium motion, and left atrium standstill. Warfarin was administered to her with INR 1.8-2.5. SEE ECGs http://www.scd-symposium.org/files/yunlong1.jpg http://www.scd-symposium.org/files/yunlong2.jpg http://www.scd-symposium.org/files/yunlong3.jpg http://www.scd-symposium.org/files/yunlong4.jpg Family histories: Two years ago the lady got pregnant (during that period her ECG is normal) and then had a baby boy with normal ECG. She has a younger brother and a younger sister with normal ECG now. The lady?s father has 7 brothers and sisters, in which 4 of them died before 40 years old due to heart attack without clear diagnosis, because of the limited medical conditions in their hometown. Her father and one of her aunt have received pacemaker therapy around 20 years ago, and the patient did not know their clear diagnosis. Her father died from cerebral embolism later and her aunt also died later with uncertain reasons. She don?t know more about her family history now because most of her other relatives are scattering in China and living far away from her, and cannot be reached so easily. We are still encouraging her to find more of her family members. My question is: 1. Have you met such kind of familial bradycardia with atrial standstill and tricuspid annulus dilation before? Progressive Cardiac Conduction Defects (PCCD) or the Lev-Len?gre disease usually causes RBBB/LBBB or even complete AVB with sclerosis of the left "cardiac skeleton" (Mitral annulus abnormal). However, besides the sinus and/ or atrial conduction defect, in this case right ?cardiac skeleton? seems to be sclerosis. 2. The patient did not accept the cardiac Computed Tomography due to the cost. She doesn?t want EP test and myocardial biopsy now. In my hospital we cannot have a genetic test, either. What should we do now? If necessary we can encourage the patient to do more test. 3. After pacemaker implantation, her left atrium is standstill. Is it possible that her left atrium function could recover later? Thanks for your time. All the best Yunlong Xia Department of Cardiology First Affiliate Hospital of Dalian Medical University Dalian, China. 116011 Email: yunlong.xia at gmail.com -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061025/c5d9ebd5/attachment.html From info at scd-symposium.org Wed Oct 25 15:11:34 2006 From: info at scd-symposium.org (SCD Symposium) Date: Wed, 25 Oct 2006 15:11:34 -0300 Subject: [SCD-FORUM] 104E RE: Case: Male, 29yrs. Dr. Brembilla-Perrot In-Reply-To: References: <5D20FB2D-014F-4979-8DDF-90928BDE6386@scd-symposium.org> <14C2F2C3-DA2E-4190-A474-C3962E871DE2@scd-symposium.org> Message-ID: <3C4D7535-A3A1-46F0-9FE3-0C838E2DDC07@scd-symposium.org> Dear Dr Dubner and Dr Schapachnick I would like to answer to Dr Zhu. ICD is certainly the best means to prevent SD in this young patient with syncopal VT and suspected of ARVD. But if it not possible it is at least necessary to introduce beta blockers in association with propafenone and why not to discuss amiodarone with beta blockers. Sincerely yours B Brembilla-Perrot > > > Except ICD we will have no other way to help him, right? I mean if > he doesn?t have enough money, then what we can do for him? He is > only 29, > > Dr. Zhu > > > > >> >> The patient might be ARVD, and ICD implantation might be required. >> >> >> >> >>> >>> Case: Male, 29yrs >>> >>> Illness history: Viral myocarditis was diagnosed based on >>> premature ventricular contractions (PVCs) at age 8 after an >>> influenza attack. Since then, PVBs never disappeared and right >>> ventricle enlargement had been noticed. >>> >>> ECG revealed VT lasting 3 minutes at age 19, accompanied with >>> faintness. After 2-3 years, no VT reoccurred. >>> >>> The second VT episode was recorded at age 21 lasting 2-3 hrs >>> accompanied with syncope. Intravenous propafenon worked. Oral >>> administration of propafenon could not prevent the patient from >>> VT attack. >>> >>> RFCA for VT had been delivered twice at age 22~23 by Pro. Yang at >>> the first affiliated Hospital of Dalian medical university. >>> During RFCA, VT could not be induced and cardiomyopathy was >>> diagnosed by endocardiac biopsy. >>> >>> >>> VTs occurred intermediately lasting several seconds to a few >>> minutes. VT restored to sinuous rhythm either spontaneously or by >>> intravenous propafenon administration. VT usually occurred at >>> midnight or early morning. At the following years, Sometimes VT >>> occurred quite often during a certain period (for example: twice >>> attacks in Winter), and then no occurrence for several months or >>> even longer time. The patient enjoys doing exercise. No VT >>> attacked during exercise. No restriction to exercise. >>> >>> Since 27 yrs old (2004), propafenon has been administrated in the >>> doses of 150mg bid or tid. Drug quitted after 1-2 months >>> administrations, keeping no VT attack for 0.5-1.5 yrs. >>> Since last year, the drug cannot be quitted, otherwise VT >>> reoccurred. So far, the doses of the drug are 200mg at early >>> morning and 150mg at night. Lower dosages may not prevent the VTs. >>> >>> Any suggestion about the treatment? >>> >>> Zhu -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061025/b9498a24/attachment.html From INFO at scd-symposium.org Wed Oct 25 21:29:23 2006 From: INFO at scd-symposium.org (SCD Symposium) Date: Wed, 25 Oct 2006 21:29:23 -0300 Subject: [SCD-FORUM] 107E Female patient, 30 years old. Dr. Perez Riera Message-ID: <20061026002955.91681E4872B@mail.gigared.com> Dear Dr Yunlong Xia from China Very interesting case. Your young woman has or had: 1) Strong familiar positive background for sudden cardiac death in first degree relatives( four members!!!! ) The family have disease of the conduction system because her father and one of her aunt have received pacemaker implantation 2) Clearly she has a diffuse disease of intra atrial (bradycardia, atrial standstill, after pacemaker implantation, her left atrium is standstill., paroxysmal atrial tachycardia/fibrillation) and intraventricular conduction system; 3) Clinical class III: Syncope; 4) Pulmonary arterial hypertension with dilatation of right and left atrium and right ventricle. 5) Typical familial Atrial standstill picture. Atrial standstill is a rare arrhythmogenic condition characterized by the absence of electrical and mechanical activity in the atria, transient or persistent, and complete or partial. It can be Secondary to (1) 1) Primary or idiopathic 2) Sporadic; 3) Familial: autosomal dominant. A few cases of familial forms of primary atrial standstill have been described in the literature. Mutation in the cardiac Na+ channel gene SCN5A associated with relatively rare genotypes for two connexin 40 polymorphisms has been reported. Rarely is associated with Brugada syndrome(BrS): Takehara et al, reported a case of BrS with ST elevation in the right precordial and inferior leads accompanied by atrial standstill and VF. Atrial standstill and J wave elevation were provoked by procainamide. Genetic analysis revealed a missense mutation (R367H) in SCN5A. The resultant mutant Na+ channel was nonfunctional when expressed heterologously in Xenopus oocytes. The study suggests that genetic defects in SCN5A may be associated with atrial standstill in combination with BrS (2). My dearest friend Dr Hanno Tan wrote: ?various gating changes that ultimately result in reduced Na current may elicit BrS, conduction disease, atrial standstill, and sinus node disease. Emerging insights now also link these gating defects to enhanced arrhythmia susceptibility in common, acquired, disease. For instance, AP prolongation in CHF may be explained by increased persistent Na current. Of note, recent studies have also linked Na current reduction to structural cardiac defects, notably cardiac fibrosis, dilated cardiomyopathy and, possibly, ARVD/C. Clearly, these observations highlight the cardiac Na channel as an interesting target for novel therapy strategies?(3). 4) Secondary to 4-1) Ebstein's anomaly: atrial standstill, atrial paralysis and atrio-ventricular block are an uncommon association with Ebstein's anomaly (4) In this case is possible problem in right cameras. 4-2) Emery-Dreifuss muscular dystrophy (X-linked); 4-3) Kugelberg-Welander syndrome (autosomal recessive); 4-4) Myocardial infarction; 4-5) Amyloidosis. Diagnosis of atrial standstill include: 1) Bradycardia on ECG 2) Absence of P waves in any lead of the standard ECG 3) Atrial paralysis; 4) Atrial dilatation; 5) Eventually atrial fibrillation; 6) Eventually atrial flutter; 7) Slow junctional escape rhythm; 8) Syncope or Morgagni-Adams-Stokes episodes( near 50% of cases); 9) High prevalence of thrombotic complications ( as her father) 10) Cardiac enlargement due primarily to atrial enlargement seems to be a common feature of both the complete and partial form of the familial persistent atrial standstill syndrome (5). 11) Atrial standstill associated with syncope, dilated cardiomyopathy, and SCD (6). Your young patient and some family members seem have all of this before elements. Treatment 1) Anticoagulation; 2) IECA 3) Diuretic 4) Pacemaker implantation; 5) Atrial flutter: treated by His-bundle ablation(7) You ask: Is it possible that her left atrium function could recover later? Answer: No because it is a progressive familial disease. You wrote: If necessary we can encourage the patient to do more test. Yes you must encourage the patient and family members to do the genetic test. References 1) Marini M, Arbustini E, Disertori M.Atrial standstill: a paralysis of cardiological relevance Ital Heart J Suppl. 2004;5:681-686. 2) Takehara N, Makita Nm Kawabe J et al. A cardiac sodium channel mutation identified in Brugada syndrome associated with atrial standstill. J Intern Med. 2004; 255:137-142. 3) Tan H. Sodium channel variants in heart disease: expanding horizons. J Cardiovasc Electrophysiol. 2006;17 Suppl 1:S151-S157. 4) Carballal J, Asensio E, Hernandez R. et al. Ebstein's anomaly, atrial paralysis and atrio-ventricular block: an uncommon association. Europace. 2002; 4:451-454. 5) Disertori M, Guarneiro M, Vergara G, et al.Familial endemic persistent atrial standstill in a small mountain community: review of eight cases. Eur Heart J. 1983;4:354-361 6) Fazelifar AF, Arya A, Haghjoo M, et al. Familial atrial standstill in association with dilated cardiomyopathy. Pacing Clin Electrophysiol. 2005;28:1005-1008. 7) Balaji S, Till J, Shinebourne EA, Familial atrial standstill with coexistent atrial flutter. Pacing Clin Electrophysiol. 1998;21:1841-1842. All the best Andr?s Ricardo P?rez Riera Chief of Electro-Vectocardiology Sector of the Discipline of Cardiology, ABC Faculty of Medicine (FMABC), Foundation of ABC (FUABC) - Santo Andr? - Sao Paulo - Brazil. Rua Sebastiao Afonso 885 - Zip Code: 044417-100- Jardim Miriam S.P Brazil- Phone: 5504-6243 Fax: 5506-0398. ________________________________________ Dear Dr. Perez Riera and other colleagues Last week I have met a special case, and here I want to get help from you. Female, 30 years old. She came into my hospital because of black spell and syncope for 2 weeks. The ECG shows atrial standstill and junctional/ventricular escape rhythm of 36, with atrium suspected to be retrograde excited (Fig 1***). After injection of 1 mg atropine for 10-20 min, the escape rate was only slightly increased to 50-55 bpm with standstill atrium (Fig 2***). After that isoprotorenol was intravenous infused to keep escape rate 50-55 bpm. Occasionally, paroxysmal atrial tachycardia/fibrillation and ventricular premature complex could be observed during monitoring, and AV was found not to be 1:1 conducted (Fig. 3***, P wave is clear especially on Lead V1). The routine blood test, heart enzyme, thyroid test and autoimmune system test are all inside normal range. Echocardiography reports the significant tricuspid annulus dilation, with tricuspid insufficiency, medium pulmonary artery hypertension, intra vena cava dilation (IVC 20), right system dilation (RA 52 X 61, RV 21), left atrial dilation (LA 43 X 57) and normal ventricular wall motion. After observation for 2 days, the bradycardia continued and a DDDR pacemaker was implanted, with atrium lead in right appendage. During implantation the atrium was demonstrated to be retrograde activated, but no further EP test was performed. After implantation, the P wave could be only seen positively on lead V1, whereas in other leads it can?t be clearly observed (Fig 4***). Later echocardiography showed relatively weak right atrium motion, and left atrium standstill. Warfarin was administered to her with INR 1.8-2.5. SEE ECGs http://www.scd-symposium.org/files/yunlong1.jpg http://www.scd-symposium.org/files/yunlong2.jpg http://www.scd-symposium.org/files/yunlong3.jpg http://www.scd-symposium.org/files/yunlong4.jpg Family histories: Two years ago the lady got pregnant (during that period her ECG is normal) and then had a baby boy with normal ECG. She has a younger brother and a younger sister with normal ECG now. The lady?s father has 7 brothers and sisters, in which 4 of them died before 40 years old due to heart attack without clear diagnosis, because of the limited medical conditions in their hometown. Her father and one of her aunt have received pacemaker therapy around 20 years ago, and the patient did not know their clear diagnosis. Her father died from cerebral embolism later and her aunt also died later with uncertain reasons. She don?t know more about her family history now because most of her other relatives are scattering in China and living far away from her, and cannot be reached so easily. We are still encouraging her to find more of her family members. My question is: 1. Have you met such kind of familial bradycardia with atrial standstill and tricuspid annulus dilation before? Progressive Cardiac Conduction Defects (PCCD) or the Lev-Len?gre disease usually causes RBBB/LBBB or even complete AVB with sclerosis of the left "cardiac skeleton" (Mitral annulus abnormal). However, besides the sinus and/or atrial conduction defect, in this case right ?cardiac skeleton? seems to be sclerosis. 2. The patient did not accept the cardiac Computed Tomography due to the cost. She doesn?t want EP test and myocardial biopsy now. In my hospital we cannot have a genetic test, either. What should we do now? If necessary we can encourage the patient to do more test. 3. After pacemaker implantation, her left atrium is standstill. Is it possible that her left atrium function could recover later? Thanks for your time. All the best Yunlong Xia Department of Cardiology First Affiliate Hospital of Dalian Medical University Dalian, China. 116011 Email: yunlong.xia at gmail.com From info at scd-symposium.org Thu Oct 26 00:01:15 2006 From: info at scd-symposium.org (SCD Symposium) Date: Thu, 26 Oct 2006 00:01:15 -0300 Subject: [SCD-FORUM] 106S RE: EXPERTS, ASK. EXPERTS ANSWER. Dr. Valero In-Reply-To: <2B414C65-A753-4E2C-AB17-CFC472EF7439@grupoakros.com.ar> Message-ID: <8256A020-C721-4CE0-AB37-007A64CA536E@scd-symposium.org> In general, I agree with the answer from Dr. Buxton to Dr. Szyszko. In the LABOR (LatinAmerican Biotronik Ongoing Registry) registry, which has 1,100 patients included to date with a mean 30-day follow- up and in 97% of them the rate of reversion after implantation is documented. We could observe, just as it has been presented in the Inter-American Congress of Cardiology, that most doctors do not seek the true threshold, but they arbitrarily evaluate 15 Joules. We have not seen problems in the follow-up and we believe that, if the therapy was successful with that energy, it is enough, especially with patients with low ejection fraction in whom looking for the threshold repeatedly to determine the real one, might mean a complication in the procedure. Dra Elina M Valero FACC, RM HRS, MT SAC Buenos Aires Argentina -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061026/d49a5ed4/attachment.html From INFO at scd-symposium.org Thu Oct 26 09:46:20 2006 From: INFO at scd-symposium.org (SCD Symposium) Date: Thu, 26 Oct 2006 09:46:20 -0300 Subject: [SCD-FORUM] 111E Can we use diazepam as a regular drug to help stoping PSVT attack? Message-ID: <20061026124721.E42D6184C078@mail.gigared.com> Congratulations to the SCD symposium in internet! I am a doctor in emergency department,and i often deal with patients with PSVT attacks.Some patients don't like to have RFCA,and they suffer from PSVT from time to time.Now a case:male,42.He sufftered from PSVT 2-3 times one year for more than 5 years.He didn't use any drugs after attacks.This time the ECG shows classical PSVT.HR 150bpm.After using 210mg propafenon,HR decreased to 136bpm,but PSVT still existed.Then we use ATP 20mg,6 minutes passed,it didn't work well either.The patient was so worrying,so we gave him diazepam 10mg iv,1minute after using diazepam,ECG shows sinus rhythm,HR 76bpm. My question is:Whether or not we can use diazepam as a regular drug to help stoping PSVT attack if there is no restrictions to the patient? Thanks for your answering! Xiuling Tan dezhou people's hospital shandong province China From INFO at scd-symposium.org Thu Oct 26 11:03:30 2006 From: INFO at scd-symposium.org (SCD Symposium) Date: Thu, 26 Oct 2006 11:03:30 -0300 Subject: [SCD-FORUM] 112E Female patient, 30 years old. Dr. Yunlong Xia Message-ID: <20061026140415.30567E489B2@mail.gigared.com> Dear Dr. Xia, This is an interesting case. There is an original article published in Circulation. 2004;110:2163-2167. This paper reported 21 patients with same features as you reported in the your index case and her family. The genetic analysis of these patients revealed that there is a SCN5A mutation ( a heterozygous G-to-A mutation at position 3823 that changed an aspartic acid to asparagine (D1275N) in a highly conserved residue of exon 21) in all affected family members compared to no such mutations in 300 control chromosomes. This syndrome has an autosomal dominant pattern. In this syndrome, t here is a strong correlation between the penetrance of a conduction disorder and the manifestation of dilatation in this pedigree. This correlation is 100% for affected individuals beyond the youngest generation. This disroder is characterized by sinus node dysfunction, arrhythmia, and right and occasionally left ventricular dilatation and dysfunction. These patients showed enlarged right ventricle at early stages of the disease and occasionally biventricular enlargement and clinical features of DCM in later stages. I think genetic analysis of your patient would reveal the same mutation in the SCN5A gene and there is no chance for recover of LA stadstill. For more information, you can see this article. Best regrads, Majid Haghjoo,MD Department of Pacemaker and Electrophysiology Rajaie Cardiovascular Medical and Research Center Tel: +98 21 2392 2931 Fax:+98 21 2204 8174 Tehran, Iran From INFO at scd-symposium.org Thu Oct 26 11:20:38 2006 From: INFO at scd-symposium.org (SCD Symposium) Date: Thu, 26 Oct 2006 11:20:38 -0300 Subject: [SCD-FORUM] 108S T wave alternans. Dr. Komoliatova Message-ID: <20061026142116.AFD5DE487C2@mail.gigared.com> Dear Colleagues, First of all, I want to thank the people who organized the symposium. My question is about microvolt T wave alternans. Working with our Holter monitoring system, we realized that the system evaluates ST segment variability, between two adjoining QRS complexes. This ST segment variability could be associated to microvolt T wave alternans or are they two ECG-phenomenons totally different? Thanks, Dr. Vera Komoliatova From INFO at scd-symposium.org Thu Oct 26 11:48:36 2006 From: INFO at scd-symposium.org (SCD Symposium) Date: Thu, 26 Oct 2006 11:48:36 -0300 Subject: [SCD-FORUM] 108E T wave alternans. Dr. Komoliatova Message-ID: <20061026144923.8DC1AE48A1C@mail.gigared.com> Dear Colleagues, First of all, I want to thank the people who organized the symposium. My question is about microvolt T wave alternans. Working with our Holter monitoring system, we realized that the system evaluates ST segment variability, between two adjoining QRS complexes. This ST segment variability could be associated to microvolt T wave alternans or are they two ECG-phenomenons totally different? Thanks, Dr. Vera Komoliatova From INFO at scd-symposium.org Thu Oct 26 11:49:58 2006 From: INFO at scd-symposium.org (SCD Symposium) Date: Thu, 26 Oct 2006 11:49:58 -0300 Subject: [SCD-FORUM] 113E Case: Male, 29yrs. Dr. Zhu Message-ID: <20061026145048.573ACE48A29@mail.gigared.com> Dear Dr. Brembilla-Perrot: Sorry that I didn't present very clear. This young patients has already tried most of the medicines we have in China for VT, whick include four different kind of beta blockers, amiodarone and mexiletine. Also we have tried use beta blocker together with amiodarone, or others. Atenolol did work for a while and then failed. At last, only propafenone alone works. Thank you for concerning our case. Sincerely yours, Ning Zhu From INFO at scd-symposium.org Thu Oct 26 11:49:13 2006 From: INFO at scd-symposium.org (SCD Symposium) Date: Thu, 26 Oct 2006 11:49:13 -0300 Subject: [SCD-FORUM] 109E Parasystole and syncope. Dr. Pervova. Message-ID: <20061026144952.9D78EE4870B@mail.gigared.com> Dear Colleagues: I would like to ask about the relationship between syncope and parasystole. Supraventricular parasystole diagnosis, usually, is favorable. On the other hand, its ventricular form can be complicated with ventricular tachycardia. Therefore, risk stratification of a patient with parasystole is very important. In these cases, the treatment may vary in a very wide range: from no treatment at all to antiarrhythmic drug treatment, even RF ablation of arrhythmogenic foci. I understand that the syndromes discussed in this symposium are more current. However, parasystoles have a higher incidence than these syndromes. Or, maybe parasystoles are no longer mentioned and were ?merged? into another new syndrome? I would like to know your opinions. My regards to all the participants, Dr. Ekaterina Pervova Rusia, Mosc? From INFO at scd-symposium.org Thu Oct 26 12:31:12 2006 From: INFO at scd-symposium.org (SCD Symposium) Date: Thu, 26 Oct 2006 12:31:12 -0300 Subject: [SCD-FORUM] =?utf-8?q?110E_Threshold_tests=2E_Dr=2E_Rond=C3=B3n?= Message-ID: <20061026153200.DC4AD184C06C@mail.gigared.com> Answer to Dr. Ariel Szyszko (Argentina) As Dr. Buxton well said, the risk of threshold tests in any patient, even with dilated cardiomyopathy and reduced EF, is very low and the possibility of a catastrophic event is pretty unimportant, even when I'm sure that in many schools, in patients with EF lower than 15%, do not administer a test shock but use maximum power in the first shock. In our hospital, although most of our patients have chagasic cardiomyopathy with viral origin with FC III-IV (NYHA), we predominantly perform threshold tests with values of >15 J in order to make an effective first shock always, and we do not insist in looking for a minor threshold since anyway, we are interested in the patient recovering with the first shock. We also perform threshold tests when the management plan has changed (predominantly if Amiodarone is included) since we know that threshold is quite increased; therefore we perform the test, usually, with 5 to 10 J above the first threshold with no drug. Why is it important to measure the threshold test and why we do not use the maximal load from the beginning as first shock? 1.- How do we know that the site where we implant the electrode is of good quality to defibrillate and to stimulate post-shock? 2.- How do we know that the patient is able to recover even with maximum load? 3.- If we use maximum load from the beginning, the longevity of the system is much lower and we spend power that in many cases is unnecessary and very painful 4.- It is advisable in many cases with sustained VT, the possibility of administering ATP (anti-tachycardia pacing) in order to achieve a reversion with overdrive mechanisms without using high energy loads. For all these reasons, the threshold test is advisable? Regards, Dr Mauricio Rond?n Secci?n de Electrofisiolog?a y Marcapasos Hospital Universitario de Caracas Caracas- Venezuela From INFO at scd-symposium.org Thu Oct 26 12:32:01 2006 From: INFO at scd-symposium.org (SCD Symposium) Date: Thu, 26 Oct 2006 12:32:01 -0300 Subject: [SCD-FORUM] =?utf-8?q?110E_Threshold_tests=2E_Dr=2E_Rond=C3=B3n?= Message-ID: <20061026153251.9C5D7E48A10@mail.gigared.com> Answer to Dr. Ariel Szyszko (Argentina) As Dr. Buxton well said, the risk of threshold tests in any patient, even with dilated cardiomyopathy and reduced EF, is very low and the possibility of a catastrophic event is pretty unimportant, even when I'm sure that in many schools, in patients with EF lower than 15%, do not administer a test shock but use maximum power in the first shock. In our hospital, although most of our patients have chagasic cardiomyopathy with viral origin with FC III-IV (NYHA), we predominantly perform threshold tests with values of >15 J in order to make an effective first shock always, and we do not insist in looking for a minor threshold since anyway, we are interested in the patient recovering with the first shock. We also perform threshold tests when the management plan has changed (predominantly if Amiodarone is included) since we know that threshold is quite increased; therefore we perform the test, usually, with 5 to 10 J above the first threshold with no drug. Why is it important to measure the threshold test and why we do not use the maximal load from the beginning as first shock? 1.- How do we know that the site where we implant the electrode is of good quality to defibrillate and to stimulate post-shock? 2.- How do we know that the patient is able to recover even with maximum load? 3.- If we use maximum load from the beginning, the longevity of the system is much lower and we spend power that in many cases is unnecessary and very painful 4.- It is advisable in many cases with sustained VT, the possibility of administering ATP (anti-tachycardia pacing) in order to achieve a reversion with overdrive mechanisms without using high energy loads. For all these reasons, the threshold test is advisable? Regards, Dr Mauricio Rond?n Secci?n de Electrofisiolog?a y Marcapasos Hospital Universitario de Caracas Caracas- Venezuela From INFO at scd-symposium.org Thu Oct 26 17:55:43 2006 From: INFO at scd-symposium.org (SCD Symposium) Date: Thu, 26 Oct 2006 17:55:43 -0300 Subject: [SCD-FORUM] 114E RE: Case: Male, 29yrs. Dr. Belhassen Message-ID: <20061026205610.C99A5E48702@mail.gigared.com> Dear Dr Dubner and Dr Schapachnick I would like to answer to Dr Zhu. It is obvious that economic reasons will not enable this patient to get an ICD; therefore, and as suggested by my friend Dr Brembilla-Perrot, we need to find some effective medication. Instead of the combination amiodarone + betablocker, I personnally prefer Sotalol in this young patient. Several good papers (especially from Germany) have showed the efficacy of that medication in this type of patient. Prof. Bernard Belhassen Director, Cardiac Electrophysiology Laboratory Tel-Aviv Medical Center Tel-Aviv 64239, Israel Fax: 00.972.3.697.4418 From INFO at scd-symposium.org Thu Oct 26 18:05:28 2006 From: INFO at scd-symposium.org (SCD Symposium) Date: Thu, 26 Oct 2006 18:05:28 -0300 Subject: [SCD-FORUM] 115E Female patient, 30 years old. Dr. Towbin Message-ID: <20061026210555.BCAB5E487D5@mail.gigared.com> Probably the most common autosomal dominant form would be due to mutations in lamin A/C (LMNA), the gene responsible for autosomal dominant Emery-Dreifuss muscular dystrophy, the CDDC disorder (Conduction Disease associated with Dilated Cardiomyopathy),and a form of Limb Girdle Muscular Dystrophy, as well as a variety of other disorders (Progeria, Dunnigan lipodystrophy, etc). This is available as a fee-for-service test in a variety labs including our own. Jeff Towbin,MD From INFO at scd-symposium.org Thu Oct 26 21:29:21 2006 From: INFO at scd-symposium.org (SCD Symposium) Date: Thu, 26 Oct 2006 21:29:21 -0300 Subject: [SCD-FORUM] 116E T wave alternans. Dr. Klingenheben Message-ID: <20061027002949.26E1FE486A4@mail.gigared.com> These are different ECG phenomenons. Microvolt TWA is assessed using a spectral methodology during (exercise-induced) increasing heart rate. The key literature can be obtained at www.alternans.org A new but different methodology determining TWA from Holter recordings is currently under clinical investigation. Thomas Klingenheben, Bonn Germany From INFO at scd-symposium.org Fri Oct 27 19:22:28 2006 From: INFO at scd-symposium.org (SCD Symposium) Date: Fri, 27 Oct 2006 19:22:28 -0300 Subject: [SCD-FORUM] 117E Parasystole and syncope. Dr. Perez Riera. Message-ID: <20061027222254.936EEE48727@mail.gigared.com> Dear Dr. Ekaterina Pervova from Russia Moscow. Eric Andr?s Ricardo P?rez Riera from Sao Paulo Brasil answer. Parasystole is usually an extrasystolic rhythm which can occur at SA node (1), atrium, junction and particularly in the ventricles. The dysrhythmia, resulting from the co-existence of two pacemakers, the sinus node and an ectopic focus, presents distinctive regular patterns, with transitions from one pattern to another occurring abruptly. Parasystole is the expression of a pacemaker that is protected from, and thus independent of, the dominant rhythm. Parasystole is an arrhythmia characterized by a second automatic rhythm existing simultaneously with normal SA rhythm. It is admitted that the parasystolic focus is protected from the environing myocardium by an entry block but can manifest itself. Parasystolic pacemakers are protected from depolarization by the SA node by some kind of entrance block. This block can be complete or incomplete. Actually, a pure unidirectional block does not exist and the environing myocardium affects the parasystolic rhythm by an electrotonic current which modulates the output. The classic criteria for the diagnosis of parasystole are: 1) Variable coupling intervals: Fixed coupling refers to a fixed interval between the sinus QRS complex and the VPC; this indicates reentry or a triggered focus as the possible cause. Variable coupling could be due to parasystole or multifocal ectopy; 2) Constant shortest interectopic intervals. Vagal stimulation causing temporary sinus arrest is the optimal method for differentiation between parasystole and extrasystoles in cases without spontaneous pure ectopic cycles (2). 3) Mathematically related interectopic intervals; 4) Fusion beats: Simultaneous activation of the ventricle by 2 sources can lead to a beat with characteristics between the conducted sinus beat and the ectopic beat. Three quantitative indices as necessary conditions have been used as diagnostic criteria for parasystole with high sensitivity and high specificity (3): 1) Take the earliest recorded eight interectopic intervals in which at least four intervals containing sinus beats or other beats having activated to the area within the ectopic focus. When in case of deficiency, it will fill up a vacancy in order. The ratios of the shortest coupling interval to the shortest ectopic cycle length are all less than 80%; 2) The coefficients of variation of the eight ectopic cycle length are all less than 6%; 3) The maximal differences of coupling intervals are equal to or more than 110ms. A non-parasystolic complex which occurs prematurely in the parasystolic cycle delays it. Conversely it accelerates the cycle when it occurs late. By this fact, a parasystole pacing is possible and can lead to a fixed coupling. This arrhythmia is frequently unknown and can be experimentally, reproduced by a sucrose gap preparation. A large amount of experimental and clinical data has pointed out several atypical phenomena that make the recognition of parasystole difficult. This especially occurs in the presence of influence exerted from sinus impulses upon the parasystolic rhythm. Several different expressions of parasystolic rhythm may be present within the same tracing. Supernormal modulation is responsible for the occurrence of couplets. In the case of a heart-transplant patient (4) with a small heart-rate variability as a result of heart denervation, the model predicts the RR intervals with an error of less than 6% for an 80-beat sequence. From a physiological point of view, the results imply that the interaction between the two pacemakers in the heart is fairly weak, and hence the parasystole observed in the heart-transplant patient can be modelled as pure parasystole. A minimal or absent modulation results in the classical picture of parasystole; when a mild modulating influence is present, the typical pattern of modulated parasystole ensues, whereas a strong modulation leads to disappearance of the typical features of parasystole and manifestation of concealed bigeminy.(5) In two cases, studied by Itoh et al (6). ventricular parasystole was associated with VT and in one patient, catheter ablation was successful. In patient 1, with dilated cardiomyopathy, ventricular parasystole led to VT, which converted to VF. Both ventricular parasystole and VT disappeared immediately, and no recurrence has been observed during a follow-up of 8 months. Catheter ablation to the parasystolic focus was effective and a relationship between VP and VT was strongly suggested. The ventricular parasystole revealed a new depressive effect of adenosine on ventricular parasystolic activity. Disappearance of "true" parasystole with adenosine was related (7). A case of VF during Holter monitoring was described by Szpotz from Poland. Tape analysis doesn't shows any significant changes of QT intervals, increasing in HR and ventricular ectopic activity. It was attempt possibility that modulated parasystole may lead to VF (8). Referentes 1) Satullo G, Oreto G, Luzza F, Sinus parasystole. Am Heart J. 1991; 121:1507-1512. 2) Kinoshita S, Okada F, Konishi G et al. Differentiation between parasystole and extrasystoles. Influence of vagal stimulation on parasystolic impulse formation. J Electrocardiol. 1994;27:169-174. 3) Ren Z, Zhou J, Xu G. et al., The diagnostic criteria for classic parasystole. Chin Med J (Engl). 1999; 112:992-4. 4) Costa M, Pimentel IR, Santiago T. et al.Modelling a parasystolic rhythm in a heart-transplant patient. Med Biol Eng Comput. 1999;37:492-496. 5) Satullo G, Oreto G, Cavallaro L. The many faces of parasystolic rhythm G Ital Cardiol. 1993;23:699-712. 6) Itoh E, Aizawa Y, Washizuka T et al. Two cases of ventricular parasystole associated with ventricular tachycardia. Pacing Clin Electrophysiol. 1996;19:370-373 7) Tomcsanvi J, Tenczer J, Horvath L. Effect of adenosine on ventricular parasystole. J Electrocardiol. 1996;29:61-63. 8) Szpotz M. Case of ventricular fibrillation recorded during Holter monitoring and initiated by ventricular parasystole. Przegl Lek. 1994; 51:319-321. All the best Andr?s Ricardo P?rez Riera Chief of Electro-Vectocardiology Sector of the Discipline of Cardiology, ABC Faculty of Medicine (FMABC), Foundation of ABC (FUABC) - Santo Andr? - Sao Paulo - Brazil. Rua Sebastiao Afonso 885 - Zip Code: 044417-100- Jardim Miriam S.P Brazil- Phone: 5504-6243 Fax: 5506-0398. From INFO at scd-symposium.org Fri Oct 27 19:26:39 2006 From: INFO at scd-symposium.org (SCD Symposium) Date: Fri, 27 Oct 2006 19:26:39 -0300 Subject: [SCD-FORUM] 119E microvolt T- wave alternans Dr. Roy Message-ID: <20061027222705.67B4FE48793@mail.gigared.com> Dear Dr. Vera Komoliatova Micro wave T wave alternans and ST segment variability are not the same. A fluctuation in morphology of the T- wave during alternating beats is called T-wave alternans (TWA). These subtle alternations are usually not apparent on the surface ECG, but can be measured by several signal processing techniques which amplifies the signals in microvolt level (Microvolt TWA). The alternations in T wave morphology are reflective of physiologically important abnormalities in repolarisation present on microvolt level. MTWA are linked to electrical instability of the heart and they are predictive of ventricular arrhythmias. Spectral analysis during controlled heart rate acceleration is the most widely applied technique to measure TWA. The test is performed with seven standard stress test electrodes and seven electrodes which have multisegment microvolt alternans sensors on an exercise stress test machine. We use Cambridge Heart Inc CH 2000 for this purpose which uses FFT spectral analysis. The magnitude of TWA is strongly dependent on heart rate and optimal rate for measuring MTWA is between 100 to 120 beats per minute. T-wave alternans is simpler and briefer than a standard exercise test done to detect myocardial ischemia. Recently studies of microvolt T-wave alternans have shown great promise as a second tier of risk stratification in patients with left ventricular dysfunction. This test has very high negative predictive accuracy. T-wave alternans tests are non-invasive, inexpensive, safe, work equally well in ischemic and non-ischemic left ventricular dysfunction, and have very high negative predictive accuracy and the automated reading gives excellent results. Measurements of MTWA may be inaccurate in patients who have not withheld beta blockers and who have persistent atrial fibrillation or flutter and the contraindications are same as for a standard exercise stress test. Dr. Sunil Roy T.N E mail: sunilroytn at calicutmedicalcollege.ac.in From info at scd-symposium.org Sat Oct 28 00:21:45 2006 From: info at scd-symposium.org (SCD Symposium) Date: Sat, 28 Oct 2006 00:21:45 -0300 Subject: [SCD-FORUM] EXPERTS ASK, EXPERTS ANSWER Message-ID: Dr. Serge Boveda from France asks - Is EP study (programmed ventricular pacing) still valuable for risk stratification in sudden cardiac death? Dr. Alfred Buxton from U.S.A. answers - Yes - Programmed stimulation can add valuable information in patients with coronary disease for whom ICD implant is considered in the following situation: Patients with ejection fraction >30%, that do not have symptomatic heart failure, left bundle branch block, or non-specific intraventricular conduction delay are at very low risk for sudden death (<3% over 2 years) if they do not have inducible VT at EP testing. The presence of inducible VT elevates that risk considerably. The model referred to in reference 2 below also suggests that even in patients whose EF is <= 30%, if there is no symptomatic heart failure, left bundle branch block, or non-specific intraventricular conduction delay the risk of sudden death is 3.4% over 2 years. This combination of findings was observed in approximately 25% of patients enrolled in the MUSTT study. In these patients, finding inducible VT would significantly elevate risk of sudden death. The model referred to in reference 2 will require prospective testing before it can be advocated for general use. References: 1. Buxton AE, Hafley GE, Lee KL, Gold MR, Packer DL, Lehmann MH, Josephson ME, Wyse DG, Fisher JD, Prystowsky EN, Talajic MR, Pires LA, for the MUSTT Investigators. Relation of Ejection Fraction and Inducible Ventricular Tachycardia to Mode of Death in Patients with Coronary Artery Disease. An Analysis of Patients Enrolled in the Multicenter Unsustained Tachycardia Trial. Circulation 2002;106:2466-2472. 2. Buxton AE, Lee KL, Hafley GE, Prystowsky EN, Josephson ME, Fisher JD, Gold MR, The MUSTT Investigators. A Simple Model Using the MUSTT Database Can Stratify Total Mortality and Sudden Death Risk of Coronary Disease Patients. J Am Coll Cardiol 43:Suppl-425A, 2004. Alfred E. Buxton, M.D. Ruth and Paul Levinger Professor of Medicine Director, Division of Cardiology Director, Arrhythmia Services and Electrophysiology Laboratory Brown Medical School Cardiology Division 2 Dudley Street, Suite 360 Providence, RI 02905 401 444-5328 (phone) Alfred_Buxton at Brown.edu -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061028/aee89b28/attachment.html From INFO at scd-symposium.org Sat Oct 28 19:06:56 2006 From: INFO at scd-symposium.org (SCD Symposium) Date: Sat, 28 Oct 2006 19:06:56 -0300 Subject: [SCD-FORUM] 118S RE Post-PAF reversion Brugada pattern. Dr. Diangelo Message-ID: <20061028220747.47A1CE487EB@mail.gigared.com> Dear Dr. Andres Perez Riera, I appreciate your answer related to the patient presenting Brugada type I pattern post-reversion of paroxysmal AF after using propafenone orally. Regarding this, I still have some doubts I would like you to clarify for me. According to what was published on the 2nd Consensus on Brugada Syndrome (Circulation 2005;111:000-000), to an asymptomatic patient, without structural heart disease, without familiar history of SCD, with drug-induced (non-spontaneous) Brugada I pattern ECG, close follow up is advised, with no need of further stratification (with no need of EPS). Looking forward to reading your answer soon, I appreciate your collaboration. DR. Silvano Diangelo - DR. Marcelo Lanzotti Argentina From INFO at scd-symposium.org Sat Oct 28 21:10:51 2006 From: INFO at scd-symposium.org (SCD Symposium) Date: Sat, 28 Oct 2006 21:10:51 -0300 Subject: [SCD-FORUM] 121E RE: Case: Male, 29yrs. Dr. Zhu Message-ID: <20061029001147.87DC9E486BC@mail.gigared.com> Dear Dr Dubner and Dr Schapachnick I would like to answer to Prof.Belhassen: Thank you for your suggestion. This patient had been given sotalol for short time when he was 22-23. It didn't work well with him at that time. We may try again now. Thank you again. Prof.NingZhu Cardiovascular Department The second affiliated hospital of Dalian Medical University From INFO at scd-symposium.org Sat Oct 28 21:20:17 2006 From: INFO at scd-symposium.org (SCD Symposium) Date: Sat, 28 Oct 2006 21:20:17 -0300 Subject: [SCD-FORUM] 122E RE: Case: Male, 29yrs. Dr. Zhu's ECGs. Dr. Zhang Message-ID: <20061029002102.01BF6E486A1@mail.gigared.com> Dear Dr. Zhu: Since I'm interested in using a series ECG markers for ARVD prediction, following I would like to provide my point of view about ECGs of your case. 1. For the ECG without PVCs, the QRSD in V1+V2+V3/V4+V5+V6 >25 ms and we see pathologic Q waves in V1-2 although the T wave is upright in RV leads and inverted in LV precordial leads. 2. For the ECGs with one PVC in precordial ECGs, QRS silons (notified by my ARVD mentor Dr. Guy Fontaine in many ARVD patients recently) and the classic epsilon waves are the most prominent in the RV leads. The PVC is probably originated from RV apex. By ECG evaluation alone, we see a strong evidence of RV cardiomyopathy. Syncope due to RV ventricular arrhtymias is the primary complain. With RV enlargement and the histological result, this patient meets the Task Force Criteria for ARVD/C diagnosis. The QRS silons and epsilon waves suggest the significant myocardial damage due to the disease progression. Thus I'm not supprised that his ventricular arrhythmia is hard to treat. Although he has no arrhythmias during and post exercise, extremely physical activities should be limited because it may speed up the disease progression. I wish he could have an ICD for sudden arrhythmic death prevention in addition to the drug therapy. Thank you very much for sharing those ECGs with our SCD-forum participants. Li Zhang, MD Assistant Professor, University of Utah School of Medicine Dept. of Medicine, LDS Hospital 324 10th Avenue, Suite 130 Salt Lake City, UT 84103 U.S.A. Tel: 1-801-408-5015 Fax: 1-801-408-2361 ldlzhang at gmail.com From INFO at scd-symposium.org Sat Oct 28 21:27:37 2006 From: INFO at scd-symposium.org (SCD Symposium) Date: Sat, 28 Oct 2006 21:27:37 -0300 Subject: [SCD-FORUM] 123E Genetic testing Dr. Zaklyazminskaya Message-ID: <20061029002828.66227E486BC@mail.gigared.com> Dear Dr. Wenling Liu! This problem to be not uncommon in general genetic councelling, not only in cardiogenetics. Usually we know about such position of the patient before genetic testing from special form. Informed Consent Form in our Genetic Center contain the questions: - Do you agree to communicate to your relatives about genetic testing results? Yes or No - Do you agree to genetist to contact with your relatives about this disease? Yes or No If patient disagree we could only discuss with him/her potential benefits of genetic counselling for the other member of the family. Particularly frequent we meet with such difficulties in some ethnic group practicing Islam. If this decision is invariable we can nothing unfortunately. Sincerely Dr. Elena Zaklyazminskaya, M.D., Ph.D. Russian Research Center for Medical Genetics, 1, Moskvorechie, Moscow, Russia, 115478 +7 495 5043166 +7 495 3248110 dnalab at rol.ru www.dnalab.ru >Message: 5 >Dear Dr. Alejandra Guerchicoff, Dr. Guido D. Pollevick, Dr. Charles Antzelevitch: >I have a question, this is the problem I met in clinical practice last year. If the one who was found having mutation, but he or she did not want to tell his or her spouse, >how shall we do? All the family members have been screened, and every one want know the result. >Sincerely Yours >Wenling Liu from Beijing Univ.People's Hosp. From INFO at scd-symposium.org Sat Oct 28 21:35:52 2006 From: INFO at scd-symposium.org (SCD Symposium) Date: Sat, 28 Oct 2006 21:35:52 -0300 Subject: [SCD-FORUM] 124E Question to Dr. Andres Peres Riera about atrial standstill. Dr. Zaklyazminskaya Message-ID: <20061029003642.C6DCCE4869E@mail.gigared.com> Dear Dr. Andres Riera! We have 1 female patient, 43 years old, with isolated right atrial standstill, now pacemaker was implanted (Ebstein's anomaly, Emery-Dreifuss MD, Kugelberg-Welander and amiloidosis were excluded). Proband's mother died suddenly at 38 y.o. We screened some promotors fragments, coding sequences and adjacent intronic area of Cx40, SCN5A, LMNA. Rare polymorphisms described previously by your citations was founded but none mutations in SCN5A or LMNA genes. What do you think about predictive value of identification this Cx40 without SCN5A mutations? Proband insist upon doing testing to her two daughters (dizygotic twins 16 years old) but I have some doubts. Have you information about any preventive medication for this condition? Sincerely, Dr. Elena Zaklyazminskaya, M.D., Ph.D. Russian Research Center for Medical Genetics, 1, Moskvorechie, Moscow, Russia, 115478 +7 495 5043166 +7 495 3248110 dnalab at rol.ru www.dnalab.ru From INFO at scd-symposium.org Sat Oct 28 22:42:58 2006 From: INFO at scd-symposium.org (SCD Symposium) Date: Sat, 28 Oct 2006 22:42:58 -0300 Subject: [SCD-FORUM] 120S PSVT Dr. Rodriguez Martorell Message-ID: <20061029014354.DBE7BE4869D@mail.gigared.com> Dear experts, mainly Dr. Perez Riera, I apologize for replying the questions by our colleague. Nevertheless, I would like to learn about your concepts. Dr. Xiuling Tan, I think that in your case, it would be useful before starting any therapeutic management, to know what type of PSVT we are facing: (orthodromic tachycardia using concealed accessory pathway in its circuit, which in fact respond well to adenosine and verapamil, or antidromic tachycardia with anterograde conduction and maximal preexcitation, which due to its high rate and hemodynamic involvement, will need electrical cardioversion and/or drugs (class I-C or III), during the acute phase). This case seems to correspond to the first one, but you do not state whether there is poor hemodynamic tolerance. Nevertheless, you doubt about whether Diazepam was the cause of the reversion of symptoms. According to our modest experience, I don't think so, since the crisis was interrupted by the action of the drugs that were initially used, and not by the sedative. On the other hand, we do not advocate the use of more than one drug in the acute phase, by deleterious and proarrhythmogenic electrophysiological effects. We do use electric cardioversion, which is a harmless and rapid method, when clinical symptoms appear, or we do not have the appropriate drugs. Finally, I think that if he is a carrier of an accessory pathway, with any of the mentioned tachyarrhythmias and the patient has a risky profession, explanations should be given to him about the best therapeutic option that is radiofrequency ablation, which will improve his quality of life. Kind regards, Dr. Francisco Rodriguez Martorell. (Cardi?logo Intensivista). Unidad de Cuidados Intensivos Coronarios. Hospital ``Calixto Garc?a?. Habana. Cuba. From info at scd-symposium.org Sun Oct 29 15:03:06 2006 From: info at scd-symposium.org (SCD Symposium) Date: Sun, 29 Oct 2006 15:03:06 -0300 Subject: [SCD-FORUM] Bibliographical update Message-ID: Dear colleagues, We include instructions to access the bibliography of this last week. We hope this will be of your interest. To access the bibliography http://www.scd-symposium.org/files/2006_10_28.html Kind regards, Dr. Edgardo Schapachnik - Dr. Sergio Dubner -- Dr. Sergio Dubner President of Scientific Committee Dr. Edgardo Schapachnik President of Steering Committee -------------- next part -------------- An HTML attachment was scrubbed... URL: http://www.grupoakros.com.ar/pipermail/scd-forum/attachments/20061029/d49536a9/attachment.html From INFO at scd-symposium.org Sun Oct 29 17:01:57 2006 From: INFO at scd-symposium.org (SCD Symposium) Date: Sun, 29 Oct 2006 17:01:57 -0300 Subject: [SCD-FORUM] 127C Hemodynamics. Dr. Chen Message-ID: <20061029200244.69A9FE489D5@mail.gigared.com> Dear Chairman, Douglas P. Zipes Sudden cardiac death is mainly resulted from the disturbance of cardiac electrophysioloy which can cause severe deterioration of hemodynamics. There is about 400 thousand SCD cases in the USA annually, and one person is died from SCD in every minute. 20%-40% of them were in youth or middle age. About 75% of the dead had no aura of heart disease. It was reported that SCD with organic heart disease occurred mainly in youth or middle age. 70% was healthy man under 40 years old. The underlying diseases are LQTS, Brugada syndrome, myocardial ischemia or infarction, and consequent ventricular fibrillation, that deteriorated hemodynamics, and finally heart stopped beating and brain death. My question is about hemodynamics. Whether the theory or the study method in hemodynamics are reasonably in human heart? Almost all of the study or research about hemodynamics were established in an ideal model in physics, such as ideal fluid in ideal tube with constant pressure. However, the blood flow in arterial vessel is different from the ideal model, that is the velocity of the blood flow in the middle of the vessel is faster than that at the edge of the vessel wall. In Poiseuille's law: Q =K?r4(P1?P2)/L =p r4(P1?P2)/ 8hL. Q?blood flow, is the amount of blood passes a cross section area, also called volume speed. R, resistance of blood flow, is determined by the friction in blood and between the blood and the vessel wall. R= 8hL /p r4. According to this formula, if the radius of an artery is 1 cm and the arteriole is 20?m, the ratio of R in artery and in arteriole is 1:6.25?10(exp 10). This means that if the R in artery is 1g, there is a force of 6.25?10(exp 10)g to push the blood cells in arteriole. This is unbelievable and impossible. Because the human cardiovascular system are not ideal tube in physics. Arteries are different in diameter, have branches and elastic wall. Blood is a kind of inhomogenous, viscous fluid. Blood ejected from the heart intermittently, not in a constant pressure. The diameter of red blood cell is greater than capillary vessel, what push RBC change their shape and through capillary vessel. It is axis flowing in artery, and how about small artery and arteriole? I am expecting a soon response from Douglas P. Zipes, a top rank scientist. Hezhong Chen, Associated professor , the cardiology department in the affiliated hospital of Leihe medical school, Henan province, China chinachenhezhong at 126.com From INFO at scd-symposium.org Sun Oct 29 17:08:41 2006 From: INFO at scd-symposium.org (SCD Symposium) Date: Sun, 29 Oct 2006 17:08:41 -0300 Subject: [SCD-FORUM] 128E Question on SCD studies. Dr. Sosnowski Message-ID: <20061029200937.46CB1E487CD@mail.gigared.com> Dear Professors, I would like to know what size of study population is required in studies on SCD? M.Sosnowski MD,PhD Katowice, Poland From INFO at scd-symposium.org Sun Oct 29 21:44:27 2006 From: INFO at scd-symposium.org (SCD Symposium) Date: Sun, 29 Oct 2006 21:44:27 -0300 Subject: [SCD-FORUM] 125R Thank you very much for a great Symposium. Dr. Leonid Macarov. Moscow. Message-ID: <20061030004514.024C0E48F50@mail.gigared.com> Dear Drs. Dubner, Schapachnik, Vorotniak, and other colleagues, Thank you very much for this great event, which gave us the chance to all the participants to exchange opinions and obtain new scientific and clinical knowledge. I'm certain that all participants were enriched by the clinical experiences, which will influence in a very positive way, the care for our patients. Thank you very much for a great work, so necessary for all of us! Dr. Leonid Makarov M.D., Ph.D, Professor of medicilne (Pediatry). Moscow Institute Pediatry and Children Surgery. Center for children arrhythmia. Dpt. Diagnostic of Arrhythmia. 127412 Moscow, Taldomskaya str.2 E-mail: leo at oss.ru From INFO at scd-symposium.org Sun Oct 29 21:46:54 2006 From: INFO at scd-symposium.org (SCD Symposium) Date: Sun, 29 Oct 2006 21:46:54 -0300 Subject: [SCD-FORUM] 126R T wave alternans. Dr.Iabluchanski Message-ID: <20061030004732.997C0E492F4@mail.gigared.com> I would like to thank Dr. Thomas Klingenheben for the Web site he mentioned: www.alternans.org I carefully reviewed the information presented in this site; however, I did not find the answer to my concern: which is the mechanism of T wave alternans. Thank you very much, Dr. Nikolai Iabluchansli From INFO at scd-symposium.org Sun Oct 29 23:15:42 2006 From: INFO at scd-symposium.org (SCD Symposium) Date: Sun, 29 Oct 2006 23:15:42 -0300 Subject: [SCD-FORUM] 129E Post-PAF reversion Brugada pattern. Dr. Perez Riera Message-ID: <20061030021622.CD294E48CC5@mail.gigared.com> Dear friends Drs Silvano Diangelo and Marcelo Lanzotti from Argentina: You say "I still have some doubts" I answer: I have a lot of doubts. Recently the fantastic ?myth? alive Prof Guy Fontain from France wrote to us: There are two aspects in Medicine. Drs who want to treat patients correctly and Drs (academics) who want to UNDERSTAND the diseases. The goal is different at the beginning but will lead to better identification and treatment at the end? Please read in attechment http://www.scd-symposium.org/files/spectrum.doc All the best Andr?s Ricardo P?rez Riera Chief of Electro-Vectocardiology Sector of the Discipline of Cardiology, ABC Faculty of Medicine (FMABC), Foundation of ABC (FUABC) - Santo Andr? - Sao Paulo - Brazil. Rua Sebastiao Afonso 885 - Zip Code: 044417-100- Jardim Miriam S.P Brazil- Phone: 5504-6243 Fax: 5506-0398. From INFO at scd-symposium.org Sun Oct 29 23:29:45 2006 From: INFO at scd-symposium.org (SCD Symposium) Date: Sun, 29 Oct 2006 23:29:45 -0300 Subject: [SCD-FORUM] message about attachments. Drs. Schapachnik and Dubner Message-ID: <20061030023041.77CA2E48B08@mail.gigared.com> Dear colleagues and friends, Since the Symposium is close to finishing on October 31st, after this message is distributed, no messages with attached files (ECG tracings, documents, etc.) will be admitted. Regrettably, those that do contain attached files, will not be distributed in the Forums. Thank you for your understanding. Edgardo and Sergio From INFO at scd-symposium.org Sun Oct 29 23:35:49 2006 From: INFO at scd-symposium.org (SCD Symposium) Date: Sun, 29 Oct 2006 23:35:49 -0300 Subject: [SCD-FORUM] 130E Dr. Xia's case (No. 105) Dr. Li Zhang's comments Message-ID: <20061030023640.6FE7BE48A3B@mail.gigared.com> Dear Dr. Yunlong Xia: Following I would like to provide my comments re. the ECGs of your patients, hoping to get some feedback from you, and from the world ECG master Dr. Perez Riera and from any of the experts and colleagues. >From the ECGs you provided, I see three major problems in this case. 1. Severe sinus node, intra-atrial and AV conduction abnormalities as you described in the case report. 2. Several ECG signs suggest the presence of the RV cardiomyopathy in addition to the severe conduction defects. If the inverted deflection 150 ms after QRS is a retrograded P wave in V1, the small deflection 30-40 ms after QRS in D II, III, aVL, aVF and V3-6 could be the epsilon wave or isolated AF post QRS. SAECG will help to determine whether late potential is positive. 3. A marked QT prolongation (QTc 540 ms) during atropine test is seen in ECG tracing named Yunlong 2. With family hx of SCD of several members in the paternal side, recurrent syncopal episodes and evidence of multi-chamber involvement, I wish she could have an ICD for SCD prevention. Thank you so much for sharing this interesting case with us, Li Zhang, MD From INFO at scd-symposium.org Sun Oct 29 23:47:05 2006 From: INFO at scd-symposium.org (SCD Symposium) Date: Sun, 29 Oct 2006 23:47:05 -0300 Subject: [SCD-FORUM] 131E Gene screening in HCM Dr. Liu Message-ID: <20061030024802.E66F5E48CE8@mail.gigared.com> Dear Drs Gabriel Pellegrini. La Plata. Argentina: I think genes screening should be down in patients with Familial Hypertrophic Cardiomyopathy It is estimated that over one million of patients suffer from hypertrophic cardiomyopathy (HCM) in China. HCM is the common cause of sudden death in young Chinese, especially in sportsmen.Therefore it should be pay more attention,especially in the familial HCM,because the familial patients are associated with a higher risk of sudden death.In our cohort patients there were 4 /11 families which at least one patient suffered SCD,and only 1/56 of sporadic patient had SCD. The genes? mutatons screening should be done in familial patients ,and then the same mutation should be screened in the other members of the family in order to find the patients who have not been fonud yet.The genomic typing of familial HCM is more worth than the sporadic patients,because the rate of mutation of familial HCM is higher .In our Cohort patients,there were 5/10 patients identified mutations in ??myosin heavy chain gene (MYH7), or cardiac myosin-binding protein C (MYBPC3) ,and there was only 1/50 patient identified a mutation in MYH7. 4 other members in these families idenfied the mutations.Therefore,The all members of the HCM family especially for youth should be tested, in order to find new patients and take measures to prevent SCD. Wenling Liu. Cardiology Division,Peking University People?Hospital,Beijing 100044, China., From INFO at scd-symposium.org Sun Oct 29 23:53:03 2006 From: INFO at scd-symposium.org (SCD Symposium) Date: Sun, 29 Oct 2006 23:53:03 -0300 Subject: [SCD-FORUM] 132E Atrial Standstill. Dr. Perez Riera to Zaklyazminskaya Message-ID: <20061030025349.7077DE48D09@mail.gigared.com> Dear colleague Dr. Elena Zaklyazminskaya, M.D., Ph.D.from Moscow, Russia. Here Andr?s Ricardo P?rez Riera answering from S?o Paulo Brazil. On the cellular level, AF leads to a strong shortening and an impaired rate adaptation of the AP as well as to changes in AP morphology. AF is associated with an altered gene expression of the L-type calcium channel and of K+ channels (Ito, IK1 and IKACh). The molecular mechanisms of intra-atrial conduction slowing are less well understood; changes in the expression or distribution of gap junction proteins or a decrease of the fast Na+ inward channel have been reported. A trigger of initiation for electrical remodeling is an overload of the cytoplasm with Ca2+ and a consecutive decrease of the systolic Ca2+ gradient, furthermore changes in calcium-handling proteins are detectable in AF. Gap junctions, assembled from connexins, form the cell-to-cell pathways for propagation of the precisely orchestrated patterns of current flow that govern the regular rhythm of the healthy heart. As in most tissues and organs, multiple connexin types are expressed in the heart; connexin43, connexin40 (Cx40) and connexin45 (Cx45) are found in distinctive combinations and relative quantities in different, functionally specialized subsets of cardiomyocyte. Gap junctional connexin proteins Cx40, Cx43 are a determinant of myocardial conduction and are implicated in the development of AF. An abnormal distribution of the gap junction occurs in chronic AF 1. Alterations of gap junction organization and connexin expression are now well established as a consistent feature of human heart disease in which there is an arrhythmic tendency. These alterations may take the form of structural remodeling, involving disturbances in the distribution of gap junctions and/or alteration of the amount or type of connexins expressed 2. Downregulation of Cx40 and abnormal phosphorylation of Cx40 may result in abnormal cell-to-cell communication and alteration in the electrophysiologic properties of the atrium, leading to the initiation and/or perpetuation of AF. Two linked polymorphisms within regulatory regions of the gene for Cx40, at nucleotides -44 (G --> A) and +71 (A --> G) occur in about 7% of the general population. Cx40 is abundant in the atrium, and homozygosity for the linked polymorphisms combined with an SCN5A mutation appeared to be responsible for familial atrial standstill. The rare linked Cx40 polymorphisms are associated with enhanced coefficient of dispersion and thus with the substrate for reentry in AF. Reentry is promoted by spatial dispersion of refractoriness that can be expressed as a coefficient of dispersion3. In neonatal hearts, the relative abundance of Cx43 and Cx40 is an important determinant of atrial impulse propagation through which dominance of Cx40 decreases and dominance of Cx43 increases local propagation velocity4. Wetzel et al. tested the hypothesis that AF is associated with changes in the expression of Cx40 and 43 in the left atrium with more pronounced changes in mitral valve disease than in lone AF. Protein concentrations of Cx40 and Cx43 were analyzed in left atrial tissue of patients undergoing cardiac surgery. One group of patients had lone AF (n = 41), one group had AF and mitral valve repair (n = 36), and one group in sinus rhythm served as controls (n = 15). Western blot analysis of Cx 40 and Cx43 expression showed an increase of both gap junctional proteins (connexin 43 > connexin 40) in patients with AF of all forms compared with patients in sinus rhythm. Subgroup analysis showed increased concentrations of connexin 40 in lone AF and AF with mitral valve disease compared with sinus rhythm, whereas the same analysis for connexin 43 reached significance only in the mitral valve disease group. No differences in Cx40 and Cx43 expressions were detectable between lone AF and AF with mitral valve disease. Within the groups Cx40 and Cx43 expression did not differ between patients with paroxysmal AF and patients with chronic In this paper the authors concluded that AF can induce changes in the left atrium with increased connexin expression. Furthermore, no systematic differences between patients with paroxysmal and chronic AF were detected5. In canine animal model enalapril suppressed atrial pacing-induced AF with tachycardia-mediated cardiomyopathy by suppressing interstitial fibrosis, Cx43 over-expression and conduction delay6;7 In the atria, features of gap organization and connexin expression have been implicated in the initiation of AF and, once the condition becomes chronic, gap junction alterations associated with remodelling may contribute to persistence of the condition. By correlating data from studies on the human patient with those from animal and cell models, alterations in gap junctions and connexins have emerged as important factors to be considered in understanding the pro-arrhythmic substrate found in a variety of forms of heart disease. Atrial activation pattern during AF is related to connexin expression and this relationship is altered by AF-induced remodeling in the fibrillating atria of chronic AF. Intercellular coupling and pattern of atrial activation are interrelated, but only in conjunction with the remodeling of atrial electrophysiology that occurs in chronic AF8. The cardiac gap-junction protein Cx40 is expressed selectively in atrial myocytes and mediates the coordinated electrical activation of the atria. The two single nucleotide polymorphisms in the promoter region of the Cx40 gene were significantly associated with AF. The Cx40 (-44A +71alleleG) haplotype was associated with a higher risk for AF. This haplotype also had significantly lower promoter activity in atrial myocytes9. References 1) Nao T, Ohkusa T, Hisamatsu Y, et al. Comparison of expression of connexin in right atrial myocardium in patients with chronic atrial fibrillation versus those in sinus rhythm. Am J Cardiol. 2003; 91:678-683. 2) Severs NJ, Coppen SR, Dupont E, et al. Gap junction alterations in human cardiac disease. Cardiovasc Res. 2004; 62:368-377. 3) Hauer RN, Groenewegen WA, Firouzi M, et al. Cx40 polymorphism in human atrial fibrillation. Adv Cardiol. 2006;42:284-91 4) Beauchamp P, Yamada KA, Baertschi AJ, et al. Relative Contributions of Connexins 40 and 43 to Atrial Impulse Propagation in Synthetic Strands of Neonatal and Fetal Murine Cardiomyocytes. Circ Res. 2006 Oct 19; [Epub ahead of print] 5) Wetzel U, Boldt A, Lauschke J, Weigl J, et al. Expression of connexins 40 and 43 in human left atrium in atrial fibrillation of different aetiologies. Heart. 2005; 91:166-170. 6) Sakabe M, Fujiki A, Nishida K, et al. Enalapril prevents perpetuation of atrial fibrillation by suppressing atrial fibrosis and over-expression of connexin43 in a canine model of atrial pacing-induced left ventricular dysfunction. J Cardiovasc Pharmacol. 2004; 43:851-859. 7) Schultz R, Heusch G. Pathophysiology of atrial fibrillation: importance of the renin-angiotensin system] Dtsch Med Wochenschr. 2006;131:S95-98. 8) Kanagaratnam P, Cherian A, Stanbridge RD, et al. Relationship between connexins and atrial activation during human atrial fibrillation. J Cardiovasc Electrophysiol. 2004;15:206-216., 9) Juabg JM, Chern YR, Tsai CT, The association of human connexin 40 genetic polymorphisms with atrial fibrillation. Int J Cardiol. 2006 Jun 27; [Epub ahead of print] All the best Andr?s Ricardo P?rez Riera Chief of Electro-Vectocardiology Sector of the Discipline of Cardiology, ABC Faculty of Medicine (FMABC), Foundation of ABC (FUABC) - Santo Andr? - Sao Paulo - Brazil. Rua Sebastiao Afonso 885 - Zip Code: 044417-100- Jardim Miriam S.P Brazil- Phone: 5504-6243 Fax: 5506-0398. From INFO at scd-symposium.org Mon Oct 30 00:01:58 2006 From: INFO at scd-symposium.org (SCD Symposium) Date: Mon, 30 Oct 2006 00:01:58 -0300 Subject: [SCD-FORUM] 133E microvolt T- wave alternans Dr. Bigger Message-ID: <20061030030254.C14F0E48D6A@mail.gigared.com> Dr. Roy, I thought your essay on microvolt TWA was elegant and I agreed with all of your statements except the last sentence. "Measurements of MTWA may be inaccurate in patients who have not withheld beta blockers and who have persistent atrial fibrillation or flutter and the contraindications are same as for a standard exercise stress test." The multi-center study by Bloomfield et al. (J Am Coll Cardiol 2006; 47:456-463) did not discontinue beta-blockers (81% of the 549 patients were on beta-blockers for the MTWA test) and yet the MTWA test had excellent negative predictive accuracy. Also, the 2-tiered strategy works in atrial fibrillation as well, the high-risk patients identified by LVEF in the first tier is not removed from the second tier in patients who have atrial fibrillation; thus, they get ICD prophylaxis ( the 2-tiered strategy works in this situation because AF doubles the risk of death in this high-risk subgroup (LVEF <0.31 and AF compared with LVEF <0.31 and no AFas shown by MADIT II investigators). There are fewer "contraindications for a MTWA test and a standard exercise test because the level of effort is much lower for the MTWA test, just walking at a normal pace. Happily, the 2-tiered stratgy using LVEF (tier 1) and MTWA (tier 2) works in almost all situations. -- J Thomas Bigger, MD Professor of Medicine and of Pharmacology Columbia University 630 West 168th Street, PH 9-103D New York, NY 10032 USA 212-305-5058 (phone) 212-305-7141 (fax) jtb2 at columbia.edu From INFO at scd-symposium.org Mon Oct 30 00:18:10 2006 From: INFO at scd-symposium.org (SCD Symposium) Date: Mon, 30 Oct 2006 00:18:10 -0300 Subject: [SCD-FORUM] 134E Genetic testing Dr. Schwartz Message-ID: <20061030031855.87CF3E48958@mail.gigared.com> My view is that the rules should be changed. Once we identify someone with an LQTS disease-causing mutation that implies - unless we are dealing with a de novo mutation - that other relatives (probably 50% of the siblings with their descendants) will be affected. To prevent the communication means that people at risk for sudden death (either as a direct evolution of LQTS or because the patients receive even non cardiovascular QT prolonging drugs) are not placed in a postion to avoid their death. This should be regarded as a criminal act, and not part of the right to privacy. Some proper action should be prepared by our scientific community providing the evidence to the legislators that withholding this information may cause many deaths. I don't think that we should remain passive when we know that information exists that could save the life of several individuals and that this vital information is denied to innocent people simply because a relative decides in this way. I find it incredible that soemone considers more important the so-called privacy of some single person than the life of many individuals. Just talk to the parents of children who died suddenly because an uncle decided to keep the information on the disease for himself! Some discussion on this point would be useful. Peter J. Schwartz From INFO at scd-symposium.org Mon Oct 30 00:20:42 2006 From: INFO at scd-symposium.org (SCD Symposium) Date: Mon, 30 Oct 2006 00:20:42 -0300 Subject: [SCD-FORUM] 135E Question on SCD studies. Dr. Buxton Message-ID: <20061030032124.375A9E48C82@mail.gigared.com> In order to answer this question, one must know the following: 1. What is the expected event rate in the population being studied over whatever follow-up time period is expected. 2. If one is performing a study evaluating the effect of a therapeutic intervention, one must have an idea of the magnitude of reduction in the event being studied expected as a result of the intervention. Alfred E. Buxton, M.D. Ruth and Paul Levinger Professor of Medicine Director, Division of Cardiology Director, Arrhythmia Services and Electrophysiology Laboratory Brown Medical School Cardiology Division 2 Dudley Street, Suite 360 Providence, RI 02905 401 444-5328 (phone) Alfred_Buxton at Brown.edu From INFO at scd-symposium.org Mon Oct 30 00:30:04 2006 From: INFO at scd-symposium.org (SCD Symposium) Date: Mon, 30 Oct 2006 00:30:04 -0300 Subject: [SCD-FORUM] 136S RE: Brugada's pattern detection in a son of SCD's patient. Dr. Cagnolatti Message-ID: <20061030033103.3450DE48A23@mail.gigared.com> The patient did not ever present prodromes or had any syncope episode. Short and long QT were ruled out, and we do not have procainamide. I haven't seen any ECG of his brother yet. I will call him to that end. dr alfredo cagnolatti From INFO at scd-symposium.org Mon Oct 30 11:54:22 2006 From: INFO at scd-symposium.org (SCD Symposium) Date: Mon, 30 Oct 2006 11:54:22 -0300 Subject: [SCD-FORUM] 139E TWA and beta blockers. Dr. Roy Message-ID: <20061030145510.75CA7E48CA2@mail.gigared.com> Prof. Thomas Bigger Thank you very much for your comments and bringing to my notice a very good article. As a protocol we stop beta blockers prior to the test to avoid patients from reaching TWA threshed heart rate and hence an indeterminate test result. Beta blockers in fact reduce the magnitude of MTWA. (Rashba EJ et al Circulation 2002; 105: 837-42, Murata M et al Circ J 2003; 67: 821-5, Komiya N et al Clinic Electrophysiol 2005; 28: 680-4). Beta blocker induced reduction in MTWA is one of the mechanism by which they may reduce the risk of SCD. I do agree with you that there are only few ?contraindications? for TWA. If the patient can not exercise on a treadmill like heart failure with very poor effort tolerance, we use dobutamine infusion for heart rate acceleration. Atrial pacing is also another option accelerate heart rate for TWA test. Dr. Sunil Roy T.N E mail: sunilroytn at calicutmedicalcollege.ac.in From INFO at scd-symposium.org Mon Oct 30 12:25:20 2006 From: INFO at scd-symposium.org (SCD Symposium) Date: Mon, 30 Oct 2006 12:25:20 -0300 Subject: [SCD-FORUM] 137S Brugada syndrome and LQTS. Dr. Cowan Message-ID: <20061030152617.BC278E48DEC@mail.gigared.com> Congratulations on this event, where we can learn more and more with the professors and I admire their efforts. I seize this opportunity to thank you especially. My question is: since Brugada Syndrome is a channelopathy caused by mutation in Na+ channels in the SCN5A gene, behaving as a primary disease of the electric system and framed within J wave and Na+ channel syndromes: this does not have QT or QTc interval alteration or if it does, please explain to me exactly in which situations. Thank you very much. Dr. Jaime Ricardo Cowan Panam? -America Central From INFO at scd-symposium.org Mon Oct 30 12:26:06 2006 From: INFO at scd-symposium.org (SCD Symposium) Date: Mon, 30 Oct 2006 12:26:06 -0300 Subject: [SCD-FORUM] 138S Intoxication by Diethylene Glycol. Dr. Cowan Message-ID: <20061030152658.3A96FE48E16@mail.gigared.com> Dear experts, I request information if possible, about intoxication with Diethylene Glycol: does it cause a direct myocardiotoxic effect or does it alter the QT/QTc interval? We are facing an epidemics of intoxication with this substance by contamination of cough syrup and topical-use drugs, commonly used in the community, with severe effects, even mortal. Thank you, Dr. Jaime Ricardo Cowan Panam? - Am?rca Central From INFO at scd-symposium.org Mon Oct 30 12:26:58 2006 From: INFO at scd-symposium.org (SCD Symposium) Date: Mon, 30 Oct 2006 12:26:58 -0300 Subject: [SCD-FORUM] 140E Concern about His bundle LBBB. Dr. Ruiz Alais Message-ID: <20061030152741.1042BE487AB@mail.gigared.com> Dear colleagues, I will be very grateful if you could clarify a doubt I have about complete left bundle branch block of the His bundle: is it possible that this entity may produce sudden nocturnal death caused by ventricular fibrillation? I have this doubt since over the last two years I have had 2 patients who have died in such circumstances. Thank you very much in advance, considering we are about to end such interesting symposium. JORGE RUIZ ALAIS M.D. From INFO at scd-symposium.org Mon Oct 30 18:30:24 2006 From: INFO at scd-symposium.org (SCD Symposium) Date: Mon, 30 Oct 2006 18:30:24 -0300 Subject: [SCD-FORUM] 141E gratitude and special thanks. Dr. Zaklyazminskaya Message-ID: <20061030213105.D6689E48C37@mail.gigared.com> Dear organizers and all colleagues! It was remarkable and very useful symposium. Thank you very mach for the lectures, clinical cases, discussion and comments! I hope, the splendid tradition of annual internet symposiums will be go on. My special thanks for Dr. Andres Peres Riera for permanent kindness and irrefragable answers. And one consideration about the last message (134E) from Dr. Peter J. Schwartz. I take off one's head to you, dear Doctor! Now we have a lot of fears to touch every confines of individual. Sometimes this primacy of personality oversteps the limits of common sense. But choice founded by common sense could cause the proceedings...:( Dr. Elena Zaklyazminskaya, M.D., Ph.D. Russian Research Center for Medical Genetics, 1, Moskvorechie, Moscow, Russia, 115478 +7 495 5043166 +7 495 3248110 dnalab at rol.ru www.dnalab.ru From INFO at scd-symposium.org Tue Oct 31 01:20:48 2006 From: INFO at scd-symposium.org (SCD Symposium) Date: Tue, 31 Oct 2006 01:20:48 -0300 Subject: [SCD-FORUM] 143E Seven year old boy with suspected LQT3 Dr. Costa Message-ID: <20061031042142.B4159E48F96@mail.gigared.com> Dear collegues: A seven year old boy was admitted 1 week ago because he had two episodes of syncope at rest, last one with seizures; after a neurological evaluation, a ECG was performed , and showed a QTc prolongation of 520 msec, with late onset peacked/biphasic T wave pattern, resembling a LQT3. His father and two sisters, of 12 and 14 years old, has long QT >500 msec. All three are still asymptomatic and had a non characteristic pattern of LQT3. His mother, who has a normal ECG, has a history of two brothers with sudden death at ages 25 and 28. Since the first day of hospitalization no ventricular arrhythmia was seen. Treatment with a beta blocker at regular dosage had no effect on QTc duration. Three days ago we began with the administration of oral flecainide, and the QTc measure is now 540 msec. I would like to ask the experts if this patient is at high risk of sudden death, and if it is true, do you suggest an ICD implantantion? Thank you in advance Dr. Gustavo Costa, MD Laboratorio de Electrofisiolog?a Hospital Nacional Alejandro Posadas Buenos Aires - Argentina From INFO at scd-symposium.org Tue Oct 31 01:31:55 2006 From: INFO at scd-symposium.org (SCD Symposium) Date: Tue, 31 Oct 2006 01:31:55 -0300 Subject: [SCD-FORUM] 142S T wave alternans. Dr. Rondon Message-ID: <20061031043247.297FFE489EF@mail.gigared.com> Reply to Dr. Nikolai Iabluchanski: There is no clearly described mechanism as an origin for T wave alternans, but there are several theories. 1. There is no complete depolarization of the tissue, with different refractory periods and the formation of temporally different repolarization. 2. Some regions of the myocardium do not recover completely, since their refractory periods are longer than the cycle? in this they may play a predominant role for M cells (they have refractory periods much longer than those of the endocardium). 3. Alternation of action potential predominant in short cycles and predominant in diseased (ischemic) tissue. 4. In some cases of channelopathies, such as those observed in LQTS, Brugada, etc. I think undoubtedly this may explain your doubt about the origin of T wave alternans. Dr Mauricio Rond?n Caracas - Venezuela From INFO at scd-symposium.org Tue Oct 31 14:43:00 2006 From: INFO at scd-symposium.org (SCD Symposium) Date: Tue, 31 Oct 2006 14:43:00 -0300 Subject: [SCD-FORUM] Credits Message-ID: <20061031174328.A2348E4927F@mail.gigared.com> CREDITS 10 CME credits available for Virtual Sudden Cardiac Death Symposium ? take advantage now! Go to SCD-Symposium homepage, click on CREDITS and follow the instructions or go directly to: http://www.scd-symposium.org/evaluation.php From INFO at scd-symposium.org Tue Oct 31 15:16:11 2006 From: INFO at scd-symposium.org (SCD Symposium) Date: Tue, 31 Oct 2006 15:16:11 -0300 Subject: [SCD-FORUM] 144S Intoxication by diethylene glycol. Dr. Virgini Message-ID: <20061031181639.51C50E488F0@mail.gigared.com> Dear Dr. Cowan, The intoxication with diethylene glycol produces metabolic acidosis with high GAP, with direct myocardial alteration. This is what happened in Argentina with the intoxication with altered propoleum some years ago. Anyway, I wait for the opinion from other colleagues with more experience. This is the only one I have. Dr. Virgini From INFO at scd-symposium.org Tue Oct 31 15:35:53 2006 From: INFO at scd-symposium.org (SCD Symposium) Date: Tue, 31 Oct 2006 15:35:53 -0300 Subject: [SCD-FORUM] 145S Long QT. Dr. Stefani Message-ID: <20061031183631.869D7E4934B@mail.gigared.com> Dear colleagues, My name is Amalia Stefani. I am a pediatric cardiologist and I work with the team of Dr. Claudio de Zuloaga in the Posadas Hospital, from Buenos Aires, Argentina. The reason for this message is to request your evaluation and opinion about a patient with long QT, who has recently come to our service. 7 years old. Male. Long QT (QTc 0.52), type 3 by electrocardiographic pattern (no genetic confirmation). Two syncopes in rest, the second one with tonic-clonic convulsion. With no medication that would prolong QT. Normal lab tests. History of competent bicuspid aortic valve. Monitoring while in hospital and Holter with no detection of arrhythmia. We started treatment with propanolol at 2 mg/kg/day orally, no changes in the QTc with a good dose of beta blockers. We changed to flecainide 2 mg/kg/day and had no satisfactory response. Family history: 2 uncles on the mother side died of sudden death at 25 and 28 years old, mother with normal ECG. Father and 2 sisters with type-3 long QT by electrocardiographic pattern (QTc 0.50 ? 0.53 respectively), asymptomatic. They are not medicated. No history in the father's side of the family. We would be grateful if you could give us your diagnostic impression and management to follow. Kind regards. From INFO at scd-symposium.org Tue Oct 31 15:38:07 2006 From: INFO at scd-symposium.org (SCD Symposium) Date: Tue, 31 Oct 2006 15:38:07 -0300 Subject: [SCD-FORUM] 146E Seven year old boy with suspected LQT3 Dr. Safer Message-ID: <20061031183836.EE6DAE4931D@mail.gigared.com> Dear Dr. Costa, definitely we can expect such a patient to be at high risk of a SCD (syncopes). Though, as of the boy?s age I would have strict reservations to implant an ICD. Maybe, it would be last ratio, if there where no else ways to avoid a SCD. As for what you describe, the image of LQTS3 would fit, and the family history confirms that there is a need for action: (1) Would you be willing to share the boy?s ECGs here, so that we might have a better picture of what the alterations are? The QT-correction might be a little misleading, as with high heart rates, Bazett?s and also Fredericia?s correction will "overcorrect". Anyway, the ECG picture could help us to better understanding and discussion level. (2) Did you take a Holter ECG of the patient? To explore the nature of the syncope this is an ultimate need, as underlying ectopies may happen during sleep phase, with nobody aware that they happened. It would be reasonable to take Holters from the fater, mother and 2 sisters, too. It is reasonable to look for any kind of complex ectopic events, and try to find T-wave alternans, and any kind of T-wave instability (like T-inversions). That ought to bee good markers of SCD risk. (3) Genotyping of the family should be performed, if ever possible. Possibly colleagues here might be willing to help out with this task. (4) As rate reduction by a beta-blocker did not help to shorten QTc, the patient may be assessed for the reaction to QT-shortening drugs. I would suggest to start infusion with low doses of Verapamile (or Lidocaine), then titrate dose stepwise as long as reasonable and to assess the reaction by ECG. Mobitz-2 blocks should be taken care of, and would be a sign to lower dose or stop. Same is true if the QT/QTc would shorten too much. If Verapamile would work, I would suggest to test an oral administration (preferably with a sustained release formulation)until equivalent effect on QT/QTc can be achieved compared to the infusion. Best regards Dr. Anton Safer Weisenheim am Sand, Germany From INFO at scd-symposium.org Tue Oct 31 15:42:54 2006 From: INFO at scd-symposium.org (SCD Symposium) Date: Tue, 31 Oct 2006 15:42:54 -0300 Subject: [SCD-FORUM] =?utf-8?q?147E_Congratulations=2E_Dr=2E_Prof=2EDr=2E_?= =?utf-8?q?Breijo_M=C3=A1rquez_Breijo?= Message-ID: <20061031184322.B1C10E49388@mail.gigared.com> Ours more sincere congratulations to the Organizers of the Symposium on Cardiac Sudden Death. Extraordinarily high level. Thanks to all the Participants for its contributions. Our better desires to all. Prof.Dr. Breijo M?rquez Breijo From INFO at scd-symposium.org Tue Oct 31 16:09:25 2006 From: INFO at scd-symposium.org (SCD Symposium) Date: Tue, 31 Oct 2006 16:09:25 -0300 Subject: [SCD-FORUM] 148S Chagasic female patient. Dr. Perez Solis Message-ID: <20061031190953.C6367E49284@mail.gigared.com> Congratulations for such spectacular symposium. I also would like to thank your unconditional teaching through it. So I dare to ask this question: I have a female patient, 51 years old, chagasic, with permanent pacemaker. Symptomatic bradycardia 5 years ago. She says that since 2 months ago she has dyspnea, FC 2-3. She deemed it unimportant. Today, we casually found AF with moderate ventricular response during the control for her pacemaker, with echo that does not show significant alteration. She is asymptomatic to this date. She consulted with her electrophysiologist, who suggested anticoagulation with acenocumarol, and later electrical cardioversion? My question is: Is there consensus for such management, taking into account her pacemaker, or should we wait, or start a pharmacological treatment? Why? Which would be the most appropriate management to follow? Thanking you in advance, DR. P?REZ SOLIZ agaps_40 at yahoo.com.ar apsoliz at hotmail.com From INFO at scd-symposium.org Tue Oct 31 16:12:13 2006 From: INFO at scd-symposium.org (SCD Symposium) Date: Tue, 31 Oct 2006 16:12:13 -0300 Subject: [SCD-FORUM] 149E Mechanisms of T Wave Alternans Dr. Verrier Message-ID: <20061031191241.8B520E48836@mail.gigared.com> Comment on Mechanisms of T Wave Alternans Whereas T-wave alternans (TWA) is a unitary phenomenon, namely a repeating ABAB pattern in the amplitude and morphology of the T wave, it is likely that the underlying mechanisms differ as a function of pathophysiologic state. This inference is supported by the fact that the clinical presentation of TWA varies markedly. For example, in the classic presentation of the long QT syndrome, the entire T wave may alternate, even bidirectionally, above and below the isoelectric line. In the Brugada syndrome, the terminal portion of the T wave alternates. In the prevalent condition of ischemic heart disease, alternation is concentrated in the ST segment and first half of the T wave. Experimental studies with fluorescent dyes suggest that derangements in intracellular calcium cycling underlie ischemia-induced TWA. Overall, these observations emphasize the need to consider the particular pathophysiologic state in the effort to uncover the pathophysiologic bases for TWA. Increased understanding of this marker of cardiac electrical instability could help to tailor therapeutic interventions aimed at reducing the magnitude of TWA. This is especially important that TWA is not only a marker of arrhythmic risk but also a potential arrhythmic trigger. Dr. Verrier Dr. Richard L. Verrier, F.A.C.C. Associate Professor of Medicine, Harvard Medical School Beth Israel Deaconess Medical Center Harvard-Thorndike Electrophysiology Institute Harvard Institutes of Medicine 77 Avenue Louis Pasteur, Room 223 Boston MA 02115 Phone: 617-667-0730/FAX: 617-975-5270 Email: rverrier at bidmc.harvard.edu From INFO at scd-symposium.org Tue Oct 31 16:18:28 2006 From: INFO at scd-symposium.org (SCD Symposium) Date: Tue, 31 Oct 2006 16:18:28 -0300 Subject: [SCD-FORUM] 150E Genetic testing Dr. Antzelevitch Message-ID: <20061031191855.EE188E49295@mail.gigared.com> Regarding the issue of sharing the results of genetic tests with family members, I wholeheartedly agree with Peter Schwartz and the other responders that we have a moral obligation to prevail upon the proband to divulge the results of a disease-causing mutation to his/her extended family. Please note however that in the United States, HIPPA regulations prohibit sharing of information even within a family. Charlie Antzelevitch _____________________________________ Charles Antzelevitch, PhD, FACC, FAHA, FHRS Executive Director/Director of Research Gordon K. Moe Scholar Professor of Pharmacology Masonic Medical Research Laboratory 2150 Bleecker Street Utica, NY 13501-1787 Tel: (315) 735-2217 ext. 117 (after 5 PM ext. 129) Fax: (315) 735-5648 ca at mmrl.edu www.mmrl.edu From INFO at scd-symposium.org Tue Oct 31 16:19:45 2006 From: INFO at scd-symposium.org (SCD Symposium) Date: Tue, 31 Oct 2006 16:19:45 -0300 Subject: [SCD-FORUM] 151E Brugada syndrome and LQTS Dr. Antzelevitch Message-ID: <20061031192012.DFE80E4920E@mail.gigared.com> In reply to Dr. Cowan: Loss of function of sodium channel activity secondary to SCN5A mutations can accentuate the action potential notch in right ventricular epicardium, thus leading to a prolongation of the RV action potential (RVOT). This results in an accentuation of the J wave (ST segment elevation), inversion of the T wave and prolongation of the QT interval in the right precordial leads (usually most prominent in lead V2). Keep in mind that SCN5A mutations only account for approximately 15% of BrS probands. Other gene mutations can result in more dramatic and more global changes in the QT interval. At the upcoming AHA meeting (late-breaking abstracts) we will present two new genes associated with a Brugada phenotype. Mutations in these genes not only produce an ST segment elevation as well as abbreviation of the QT interval in all leads, in some cases approximating intervals observed in the Short QT Syndrome. The result is a clinical entity that combines BrS and SQTS. I hope this is helpful. Charlie Antzelevitch _____________________________________ Charles Antzelevitch, PhD, FACC, FAHA, FHRS Executive Director/Director of Research Gordon K. Moe Scholar Professor of Pharmacology Masonic Medical Research Laboratory 2150 Bleecker Street Utica, NY 13501-1787 Tel: (315) 735-2217 ext. 117 (after 5 PM ext. 129) Fax: (315) 735-5648 ca at mmrl.edu www.mmrl.edu From INFO at scd-symposium.org Tue Oct 31 16:25:27 2006 From: INFO at scd-symposium.org (SCD Symposium) Date: Tue, 31 Oct 2006 16:25:27 -0300 Subject: [SCD-FORUM] 151E Brugada syndrome and LQTS Dr. Antzelevitch Message-ID: <20061031192555.7D484E48836@mail.gigared.com> In reply to Dr. Cowan: Loss of function of sodium channel activity secondary to SCN5A mutations can accentuate the action potential notch in right ventricular epicardium, thus leading to a prolongation of the RV action potential (RVOT). This results in an accentuation of the J wave (ST segment elevation), inversion of the T wave and prolongation of the QT interval in the right precordial leads (usually most prominent in lead V2). Keep in mind that SCN5A mutations only account for approximately 15% of BrS probands. Other gene mutations can result in more dramatic and more global changes in the QT interval. At the upcoming AHA meeting (late-breaking abstracts) we will present two new genes associated with a Brugada phenotype. Mutations in these genes not only produce an ST segment elevation as well as abbreviation of the QT interval in all leads, in some cases approximating intervals observed in the Short QT Syndrome. The result is a clinical entity that combines BrS and SQTS. I hope this is helpful. Charlie Antzelevitch _____________________________________ Charles Antzelevitch, PhD, FACC, FAHA, FHRS Executive Director/Director of Research Gordon K. Moe Scholar Professor of Pharmacology Masonic Medical Research Laboratory 2150 Bleecker Street Utica, NY 13501-1787 Tel: (315) 735-2217 ext. 117 (after 5 PM ext. 129) Fax: (315) 735-5648 ca at mmrl.edu www.mmrl.edu From INFO at scd-symposium.org Tue Oct 31 17:45:31 2006 From: INFO at scd-symposium.org (SCD Symposium) Date: Tue, 31 Oct 2006 17:45:31 -0300 Subject: [SCD-FORUM] 152S Farewell. Dr. Perez Riera Message-ID: <20061031204613.25D98E49360@mail.gigared.com> Dear colleagues from all the corners of the world, I am writing to you while our event is finishing, to congratulate you for the fantastic result of this magnificent virtual Forum of the ISHNE Sudden Cardiac Death World-Wide Internet Symposium. This has been for me ?a modest student of this topic- a great honor, since I was able to share this with colleagues of great international status, like the Vice-Presidents of the Steering Committee, and illustrious figures such as Drs. Wojciech Zareba, Branco Mautner, and Li Zhang. I would like to thank also, my dear friends at all times, Drs. Edgardo Schapachnik and Sergio Dubner, with whom we have a common feeling of companionship and constant improvement, based on ethics and mutual collaboration. Finally, I would like to thank the great figures of this event, our dear and admired Honorary President, Prof. Dr. Douglas P. Zipes and Co-Chairman, Prof. Silvia Priori, whose profiles need no further comments, due to their gigantic work in the field of sudden death. Finally, I would like to thank the anonymous translators who contributed with their work so that this event could be read in several languages. I am absolutely certain that our expectations were greatly exceeded by the quality of the contributions and the strong interest caused by the discussions. This incomparable result is due to the "miracle" of the Internet, which enables us more and more to democratize information. To all you I embrace you in brotherly way, and until the next event, God willing. All the best for all Andr?s Ricardo P?rez Riera MD Chief of Electro-Vectocardiology Sector of the Discipline of Cardiology, ABC Faculty of Medicine (FMABC), Foundation of ABC (FUABC) - Santo Andr? - Sao Paulo - Brazil. Rua Sebastiao Afonso 885 - Zip Code: 044417-100- Jardim Miriam S.P Brazil- Phone: 5504-6243 Fax: 5506-0398. From INFO at scd-symposium.org Tue Oct 31 17:53:47 2006 From: INFO at scd-symposium.org (SCD Symposium) Date: Tue, 31 Oct 2006 17:53:47 -0300 Subject: [SCD-FORUM] 154E Long QT. Dr. Schwartz Message-ID: <20061031205422.DE879E493AF@mail.gigared.com> Dear Dr. Stefani: Interesting case. The occurrence of sudden deaths in both maternal and paternal side calls for great caution in interpretation and raises the possibility of two independent mutations. I would not trust the ECG pattern to guide my therapeutic decision. Genetic testing is essential and should involve all 9 (perhaps 10) LQTS genes. I am willing to genotype your little boy in my laboratory. Our max screening time is 6 months and our success rate is 70-75%. As to your observations. Never mind if Propranolol does not shorten the QTc, it should still be used at full dose. I would not use Flecainide which has Ikr blocking effect! We use mexiletine and in LQT3 we have approx. 90-100 ms QTc shortening. What information is giving you the Holter recording and exercise? Any unusual pattern nighttime? I will be glad to help, if you wish. Peter J. Schwartz From INFO at scd-symposium.org Tue Oct 31 17:52:41 2006 From: INFO at scd-symposium.org (SCD Symposium) Date: Tue, 31 Oct 2006 17:52:41 -0300 Subject: [SCD-FORUM] 153E Seven year old boy with suspected LQT3 Dr.Makarov, Moscow Message-ID: <20061031205319.689C7E493B2@mail.gigared.com> Dear Dr. Costa, by our experience a beta blockers or Na blockers in pts with LQTS not to influence on the prolongation of QT interval. One of the sign of nonffectivness of the antyarrhythmic drug threapy is repeat syncope or presyncope under therapy. It is also the indication for ICD threapy as well as other proarrhythmogenic signs rest ECG or Holter monitoring - T wave alternans, ventricular arrhythmia by my mention. Of course it will be useful genetic suggesting of the LQT3 for indication to Na blockers therapy. Sincerely yours Dr. Leonid Makarov, Moscow, Russia leo at oss.ru From INFO at scd-symposium.org Tue Oct 31 21:04:41 2006 From: INFO at scd-symposium.org (SCD Symposium) Date: Tue, 31 Oct 2006 21:04:41 -0300 Subject: [SCD-FORUM] 155E Seven year old boy with suspected LQT3 Dr. Schwartz Message-ID: <20061101000513.D5D01E48742@mail.gigared.com> I comment on Dr. Makarov's statement. I am not sure what is his basis to say that Na+ channel blockers (it should be mexiletine!) do not shorten QTc. They are indeed ineffective in LQT1 and LQT2 and should be used only in LQT3 patients. In this group, with very few exceptions, they produce a major shortening of QTc. A statement on this issue requires that only LQT3 patiens are analyzed. On how many LQT3 patients is Dr. Markov's experience based? Thanks for a prompt reply. Peter J. Schwartz From INFO at scd-symposium.org Tue Oct 31 21:11:44 2006 From: INFO at scd-symposium.org (SCD Symposium) Date: Tue, 31 Oct 2006 21:11:44 -0300 Subject: [SCD-FORUM] 156E Mechanisms of T Wave Alternans Dr. Schwartz Message-ID: <20061101001213.BB71AE49351@mail.gigared.com> The comment by Dr. Verrier is absolutely correct and the pathophysiology underlying the different types of TWA should be kept in mind by those interested in this intriguing phenomenon. Peter J. Schwartz From INFO at scd-symposium.org Tue Oct 31 22:52:15 2006 From: INFO at scd-symposium.org (SCD Symposium) Date: Tue, 31 Oct 2006 22:52:15 -0300 Subject: [SCD-FORUM] 157S consultation Dr. Rondon Message-ID: <20061101015257.E481CE49308@mail.gigared.com> Answer for Dr. Perez Soliz: I dare answer you about the case of your 51-year-old chagasic patient, with permanent pacemaker, and current evidence of mild AF with FC II-III and echo that is normal. This population has indication of anticoagulation to prevent, as you know well, the risk of 4 to 1 of stroke. Now, in this population, if EF is normal and there is no evidence of spontaneous echo or thrombi in the left atrium, they have a better prognosis than those with low EF and sign of "smoke" in the atrium or ventricle. The ACUTE protocol demands 4 to 6 weeks of anticoagulation, maintaining INR between 2 to 2.5 with new echo before the cardioversion procedure, and if it is the same as before, programmed cardioversion can be conducted. A single effective shock is preferable. This has been reported in several works: the greater the load, the possibility of the first load to be effective is approximately within the following range of effectiveness: 100 J = 70 - 75 % 200 J = 78 - 80 % 250 J = 85 % 300 J = 92 % If you use defibrillator with biphasic power, you may try 150 to 170 J with a percentage close to 95% of effectiveness in the first shock. Please, remember placing the blades so that the pacemaker is not included within them, to prevent damaging the circuit, so it is suggested to place removable blades with anterior-posterior positioning. I recommend to sedate her with Diprivan (Propofol) 0.3 to 0.5 mg/Kg, slow impregnation and once the sedative effect is achieved, synchronize the load and apply cardioversion. Then, maintain anticoagulation for another 4 weeks, due to the electric stunning effect and atrial remodeling. Electrical activity and poor mechanic activity may be observed, so the left appendage should be seen again, and pulse doppler flow should be measured in the neck of the appendage. This should be greater than 30 ? 35 cm/sec, to know if there is low risk of thrombogenesis. I think that the management is appropriate in this way, and if reversion to SR is not achieved, then you should consider electrophysiological study, check fragmented atrial potentials and with a color 3D mapping system (Ensite System) the possibility of pulmonary vein isolation, if this is the origin of the AF. You still have many options with your patient, even though I don't deny how discouraging AF is in chagasic patients. Dr Mauricio Rond?n Hospital Universitario de Caracas Venezuela From INFO at scd-symposium.org Tue Oct 31 22:54:21 2006 From: INFO at scd-symposium.org (SCD Symposium) Date: Tue, 31 Oct 2006 22:54:21 -0300 Subject: [SCD-FORUM] Closing Speech Message-ID: <20061101015507.41BA3E48D72@mail.gigared.com> We have come to the end of the Symposium, with numerous lectures, clinical cases, and interviews at an extremely excellent level. We also have quite a lot of questions from different places in the world, and answered by experienced guest colleagues. This type of fluid contact about specific topics - the most important of which address real patient issues that are the focus of what we do-- enables the exchange of ideas about individual cases among the investigators and experts, something very difficult to do in many conferences. The discussion of some aspects of guidelines about sudden death recently published by the ACC, AHA, and ESC, coordinated by Dr. D. Zipes and other lecturers from this symposium, has been very useful to understand; we have even been pioneers in their translation into Chinese. As usual, the ISHNE feels proud to be able to organize this unique and distinct academic event, through which we make a contribution to continuing and long distance medical education in the world, updated daily. We would like to thank Dr. Silvia Priori, Co-Chairman of this symposium, Drs. Andres Perez Riera, Li Zhang and Branco Mautner, who participated in the coordination, and all the lecturers that provided content, all the experts who provided questions and answers at a distinguished level, and of course to all the participants, who with their clinical cases, doubts and questions -the essential basis of this symposium- have generated the replies from the experts. We would also like to thank our team of "unknown people", those who are behind the screens, because without them this would have been impossible to fulfill: our audio and video technical coordinators, experts in networks, programmers, translators, secretaries, and maintenance operators. For all of them, thank you very much. Finally, we would also like to thank Medtronic, and especially its managers, Ross Meisner and Hugo Fagioli, who shared this educational idea and provided their unconditional support. To all of them, THANK YOU VERY MUCH, and we share the wait for our next symposia on long QT induced by drugs, chaired by Dr. Wojciech Zareba, and Atrial Fibrillation II, chaired by John Camm. Doug, Sergio and Ed