Clinical Approach to Sudden Cardiac Death Syndromes

Clinical cardiologists are encountering an important challenge in the care of families with inherited cardiac diseases. The majority of the inherited cardiac diseases causing sudden death express themselves at variable ages in the form of altered muscle f

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Ramon Brugada (Ed.)

Clinical Approach to Sudden Cardiac Death Syndromes

Ramon Brugada Dean, School of Medicine Director, Cardiovascular Genetics Center UdG-IDIBGi Universitat de Girona Pic de Peguera 11 17003 Girona, Catalonia, Spain

Pedro Brugada Scientific Director, Cardiovascular Division Head, Heart Rhythm Management Centre Free University of Brussels (UZ Brussel) VUB Laarbeeklaan 101 1090 Brussels. Belgium

Josep Brugada Medical Director Hospital Clínic Universitat de Barcelona Villarroel 170 08036 Barcelona, Catalonia, Spain

ISBN: 978-1-84882-926-8     e-ISBN: 978-1-84882-927-5 DOI: 10.1007/978-1-84882-927-5 Springer London Dordrecht Heidelberg New York Library of Congress Control Number: 2009938027 © Springer-Verlag London Limited 2010 This work is subject to copyright. All rights are reserved, whether the whole or part of the material is ­concerned, specifically the rights of translation, reprinting, reuse of illustrations, recitation, broadcasting, reproduction on microfilm or in any other way, and storage in data banks. Duplication of this publication or parts thereof is permitted only under the provisions of the German Copyright Law of September 9, 1965, in its current version, and permission for use must always be obtained from Springer. Violations are liable to prosecution under the German Copyright Law. The use of general descriptive names, registered names, trademarks, etc. in this publication does not imply, even in the absence of a specific statement, that such names are exempt from the relevant ­protective laws and regulations and therefore free for general use. Product liability: The publishers cannot guarantee the accuracy of any information about dosage and application contained in this book. In every individual case the user must check such information by consulting the relevant literature. Cover design: eStudio Calamar, Figueres/Berlin Printed on acid-free paper Springer is part of Springer Science+Business Media (www.springer.com)

Preface

Much has changed in the field of arrhythmias and sudden cardiac death in these last decades. Successful innovative catheter therapies and protective devices have been determinant in enhancing treatment and prevention strategies of individuals at risk. However, despite the advances, sudden cardiac death still remains a major contributor to mortality in our society. While most deaths occur in adult cases and are associated with ischemic heart disease, occasionally the youngest and the fittest, even those who have become our role models for their athletic abilities, may also die suddenly, usually from noncoronary cardiac causes. It has not been until the advent of molecular biology and genetics in cardiology when we have been able to further deepen in our knowledge of these dreadful events in the young. In the last 20 years, genetic research in subjects and families with sudden cardiac death syndromes has brought a vast amount of information on genetic defects responsible for arrhythmogenesis, improving our understanding on how the abnormally codified