Arrhythmogenic Right Ventricular Cardiomyopathy Dysplasia: Recent Advances

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This book covers all aspects (biological, pathological, genetics, clinical and therapeutical) of arrhythmogenic right ventricular cardiomyopathy/dysplasia, a recent cardiomyopathy which represents a very high risk of sudden death in the young and in athletes. The monograph presents the results of 5-year research program on ARVC/D supported by grants of both the European Commission and the NIH, which enabled the discovery of seven disease-causing genes, thus opening new avenues for the early identification of affected patients and for sudden death prevention. A panel of top scientists, both European and Americans, contributed to this volume, which will be an essential reference for professionals and residents in cardiology, radiology, human genetics, and sport medicine.

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Arrhythmogenic RV Cardiomyopathy/Dysplasia Recent Advances FRANK I. MARKUS ANDREA NAVA GAETANO THIENE Indice III FRANK I. MARCUS • ANDREA NAVA • GAETANO THIENE (Eds) Arrhythmogenic RV Cardiomyopathy/ Dysplasia Recent Advances IV Indice FRANK I. MARCUS Section of Cardiology Department of Medicine Sarver Heart Center University of Arizona Tucson (AZ), USA ANDREA NAVA Department of Cardiological, Thoracic and Vascular Sciences University of Padua, Italy GAETANO THIENE Department of Medical Diagnostic Sciences University of Padua, Italy Library of Congress Control Number: 2007922495 ISBN 978-88-470-0489-4 Springer Milan Berlin Heidelberg New York Springer is a part of Springer Science+Business Media springer.com © Springer-Verlag Italia 2007 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, re-use of illustrations, recitation, broadcasting, reproduction on microfilms or in other ways, and storage in data banks. Duplication of this publication or parts thereof is only permitted under the provisions of the Italian Copyright Law in its current version, and permission for use must always be obtained from Springer. Violations are liable for prosecution under the Italian 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 publisher 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: Simona Colombo, Milan, Italy Typesetting: C & G di Cerri e Galassi, Cremona, Italy Printer: Arti Grafiche Nidasio, Assago, Italy Printed in Italy Springer-Verlag Italia S.r.l.,Via Decembrio 28, I-20137 Milan Indice V PREFACE This book covers all the recent research highlights of arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D), a recently discovered heart muscle disease which is a major threat to the life of affected young people. It summarizes nearly 25 years of investigation on the etiology, genetics, pathology, clinical features, diagnosis, and treatment of ARVC/D. In particular, a 5-year research program supported by grants from both the European Community and the National Heart, Lung and Blood Institutes has contributed to the discovery of seven disease-causing genes, thus opening new avenues for the early identification of affected patients and prevention of sudden death. A Workshop was held in Venice, Italy, October 3, 2005, as part of the Venice Arrhythmia Meeting, where the European and American investigators presented and discussed several major achievements which are now reported in this book. As a result of thes