J Wave Syndromes Brugada and Early Repolarization Syndromes Charles Antzelevitch Gan-Xin Yan Editors 123 J Wave Syndromes Charles Antzelevitch (cid:129) Gan-Xin Yan Editors J Wave Syndromes Brugada and Early Repolarization Syndromes Editors Charles Antzelevitch Gan-Xin Yan Cardiovascular Research Lankenau Institute for Medical Research Lankenau Institute for Medical Research Lankenau Medical Center Wynnewood Wynnewood Pennsylvania Pennsylvania USA USA ISBN 978-3-319-31576-8 ISBN 978-3-319-31578-2 (eBook) DOI 10.1007/978-3-319-31578-2 Library of Congress Control Number: 2016942096 © Springer International Publishing Switzerland 2016 T his work is subject to copyright. All rights are reserved by the Publisher, whether the whole or part of the material is concerned, specifi cally the rights of translation, reprinting, reuse of illustrations, recitation, broadcasting, reproduction on microfi lms or in any other physical way, and transmission or information storage and retrieval, electronic adaptation, computer software, or by similar or dissimilar methodology now known or hereafter developed. T he use of general descriptive names, registered names, trademarks, service marks, etc. in this publication does not imply, even in the absence of a specifi c statement, that such names are exempt from the relevant protective laws and regulations and therefore free for general use. T he publisher, the authors and the editors are safe to assume that the advice and information in this book are believed to be true and accurate at the date of publication. Neither the publisher nor the authors or the editors give a warranty, express or implied, with respect to the material contained herein or for any errors or omissions that may have been made. Printed on acid-free paper This Springer imprint is published by Springer Nature The registered company is Springer International Publishing AG Switzerland We dedicate this book to the members of the Heart Rhythm Society (HRS), the European Heart Rhythm Association (EHRA), and the Asian-Pacifi c Heart Rhythm Society (APHRS) who met in Shanghai, China, in April 2015 to generate an expert consensus document and formulate this text focused on the J wave syndromes. We are also proud to dedicate this compendium to the many investigators whose collective works have advanced us to this exciting juncture in the history of cardiac electrophysiology and arrhythmias, on whose shoulders we stand, as well as to our mentors, collaborators, and fellows who have assisted us in advancing the fi eld. Last but certainly not least, we dedicate this book to our families, whose understanding and support permitted us to dedicate the time and effort needed to formulate this text. Charles Antzelevitch Gan-Xin Yan Foreword S udden cardiac death remains the most important public health issue on the planet. Despite intense research efforts and remarkable progress in heart disease popula- tions, we have not been able to impact its overall incidence in the community where the vast majority of victims have not been previously identifi ed as being at risk. Particularly tragic is its continued occurrence in young and apparently healthy peo- ple. Although sudden death is far less common in this population, it is an unspeak- able calamity, truncating productive lives, widowing spouses, and orphaning children around the world. It is now clear that a particularly important mechanism for sudden death in the young is some form of the newly defi ned J wave syndromes. Though knowledge regarding these entities has advanced rapidly, many challenges remain. Drs. Antzelevitch and Yan, outstanding leaders in this area of research, convened an expert panel to review the state of the art in order to identify those issues requiring better defi nition. These include ionic mechanisms, genetics, and, most importantly, methods of diagnosis, risk stratifi cation, and treatment that can be applied on a global basis. The aim of the exercise was to mark those areas in which research and educational methods can be most effi ciently and expeditiously directed. I t is fair to say that this elite group of scientists succeeded beyond expectations. Not only have they produced an extremely valuable consensus document, but they have also put forth this book that provides amplifi cation of the most complex issues. Most importantly, the information contained herein will help resolve confusion that exists in the clinical community and has led to inappropriate, ineffective, and fre- quently over-aggressive treatment. A s they have perennially, my colleagues Drs. Antzelevitch and Yan lead the way in helping us understand how we can translate learnings in the basic laboratory into the clinic, with the ultimate goal of mitigating, if not preventing, sudden cardiac death. For their stewardship of this Herculean effort, we are most grateful. Wynnewood, PA, USA Peter R. Kowey , MD, FACC, FAHA, FHRS January 15, 2016 vii Pref ace T he J wave syndromes, consisting of the Brugada (BrS) and early repolarization syn- dromes (ERS), have captured the interest of the cardiology community for over two decades, following the identifi cation of BrS as a new clinical entity in 1992. Although experimental evidence for a heightened risk for sudden cardiac death (SCD) associated with early repolarization was reported at the turn of the century, the clinical impact of ERS was not fully appreciated until 2008 with seminal reports on the subject from the groups of Haissaguerre, Viskin, and Nam. A book dedicated to the Brugada syndrome was published by us and the Brugada brothers in 2005, emanating from the expert consensus conference dedicated to BrS in 2004. A book specifi cally focused on ERS has not previously been published other than the expert consensus document dealing with terminology of early repolarization by MacFarlane and coworkers. This book stemmed from an expert consensus conference forum organized in 2015 to evaluate new information and highlight emerging concepts with respect to differential diagnosis, prognosis, and cellular and ionic mechanisms and approaches to the therapy of the J wave syndromes. Leading experts, including members of the Heart Rhythm Society (HRS), the European Heart Rhythm Association (EHRA), and the Asian-Pacifi c Heart Rhythm Society (APHRS) met in Shanghai, China, in April 2015. The task force was charged with a review of emerging concepts and assessment of new evidence for or against particular diagnostic procedures and treatments. Members of this Task Force were selected to represent professionals involved with the medical care of patients with the J wave syndromes as well as those involved in research into the mechanisms underlying these syndromes. Selected experts in the fi eld undertook a comprehensive review of the literature. Critical eval- uation of methods of diagnosis, risk stratifi cation, approaches to therapy, and mech- anistic insights was performed, including assessment of the risk–benefi t ratio. T his text is designed to supplement and complement the expert consensus docu- ment that will be simultaneously published dealing with the J wave Syndromes: Emerging Concepts and Gaps of Knowledge. Wynnewood, PA, USA Charles Antzelevitch Wynnewood, PA, USA Gan-Xin Yan ix