Current Concepts in Arrhythmogenic Right Ventricular Cardiomyopathy / Dysplasia

166 Pages·2014·3.513 MB·English

by  Corinna Brunckhorst, Corinna Brunckhorst, Firat Duru, Ardan M. Saguner

Current Concepts in
Current Thinking on
Arrhythmogenic
Right Ventricular
Cardiomyopathy/Dysplasia
Editors
CORINNA BRUNCKHORST FIRAT DURU
l
Associate Editor
ARDAN M. SAGUNER
Foreword by
A. JOHN CAMM
Current Concepts in Arrhythmogenic Right Ventricular
Cardiomyopathy/Dysplasia

Current Concepts in Arrhythmogenic Right Ventricular
Cardiomyopathy/Dysplasia
Editors:
Prof. Dr. CORINNA BRUNCKHORST, MD
Co-Director Arrhythmia Service
University Heart Center, Zurich, Switzerland
Prof. Dr. FIRAT DURU, MD
Director Arrhythmia Service
University Heart Center, Zurich, Switzerland
Associate Editor:
Dr. ARDAN M. SAGUNER, MD
Fellow Arrhythmia Service
University Heart Center, Zurich, Switzerland
Minneapolis, Minnesota
© 2014 Corinna Brunckhorst, Firat Duru
Cardiotext Publishing, LLC
3405 W. 44th Street
Minneapolis, Minnesota 55410
USA
www.cardiotextpublishing.com
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This book is intended for educational purposes and to further general scientific and medical knowledge,
research, and understanding of the conditions and associated treatments discussed herein. This book
is not intended to serve as and should not be relied upon as recommending or promoting any specific
diagnosis or method of treatment for a particular condition or a particular patient. It is the reader’s
responsibility to determine the proper steps for diagnosis and the proper course of treatment for any
condition or patient, including suitable and appropriate tests, medications, or medical devices to be used
for or in conjunction with any diagnosis or treatment.
Due to ongoing research, discoveries, modifications to medicines, equipment and devices, and changes
in government regulations, the information contained in this book may not reflect the latest standards,
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Library of Congress Control Number: 2014933127
ISBN: 978-1-935395-92-8
eISBN: 978-1-935395-19-5
Printed in the United States of America
Dedication
We thank our families for their support and understanding.
We dedicate this book to all patients with arrhythmogenic right ventricular
cardiomyopathy/dysplasia. We hope that it serves as a source of knowledge and
inspiration for the physicians and scientists who aim to provide the best medical
care for patients with this disease. We are grateful to the Georg and Bertha
Schwyzer-Winiker Foundation for supporting the Zurich ARVC Program and for
making this book possible.
—The Editors
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Table of Contents
Contributors . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . ix
Foreword . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . xi
Preface . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . xiii
Chapter 1 Discovery of Right Ventricular Cardiomyopathies . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1
Guy H. Fontaine, Robert Frank, Jean-Louis Hébert,
Françoise Hidden-Lucet, Michel Komajda
Chapter 2 Disease Mechanisms in Arrhythmogenic Right Ventricular
Cardiomyopathy/Dysplasia: From the Macro- to the Nanoscale . . . . . . . . . . . . . . . . . 19
Esperanza Agullo-Pascual, Marina Cerrone, Mario Delmar
Chapter 3 Genetic Background of Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia . . . 31
Judith Groeneweg, Richard Hauer
Chapter 4 Genetic Counseling for Patients and Relatives . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 43
Argelia Medeiros-Domingo
Chapter 5 Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia:
Clinical Presentation, Differential Diagnosis, and Diagnostic Challenges . . . . . . . . . . 49
Rajesh Janardhanan, Frank I. Marcus
Chapter 6A The Role of Echocardiography in Arrhythmogenic Right Ventricular
Cardiomyopathy/Dysplasia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 59
Alessandra Vecchiati, Felix C. Tanner
Chapter 6B The Role of Cardiac Magnetic Resonance Imaging in Arrhythmogenic
Right Ventricular Cardiomyopathy/Dysplasia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 69
Robert Manka, Markus Niemann
Chapter 7 Drug Therapy in Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia:
When and How to Use Which Drugs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 79
Thomas Wichter
Chapter 8 The Role of Implantable Defibrillators and Catheter Ablation in the Management of
Patients with Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia . . . . . . . . 93
Florence Porterfield, Hugh Calkins
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viii Current Concepts in Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia
Chapter 9 Risk Stratification and Prognosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 105
Alessandro Zorzi, Domenico Corrado
Chapter 10 The Zurich ARVC Program . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 117
Ardan M. Saguner
Case 1 Difficult-to-Treat Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia . . . . . . . 127
Richard Kobza, Paul Erne
Case 2 Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia in Sports:
Rare Incident or the “Tip of the Iceberg”? . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 133
Christian M. Schmied
Impressions from the First Zurich ARVC/D Symposium . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 141
Index . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 145
Contributors
Editors
Prof. Dr. Corinna Brunckhorst, MD
Co-Director Arrhythmia Service, University Heart Center, Zurich, Switzerland
Prof. Dr. Firat Duru, MD
Director Arrhythmia Service, University Heart Center, Zurich, Switzerland
Associate Editor
Ardan M. Saguner, MD
Fellow Arrhythmia Service, University Heart Center, Zurich, Switzerland
Contributors
Esperanza Agullo-Pascual, PhD Mario Delmar, MD, PhD
Leon H. Charney Division of Cardiology, New Professor of Medicine/Cardiology, Leon H.
York University School of Medicine, New York, Charney Division of Cardiology, New York
New York, USA University School of Medicine, New York,
New York, USA
Hugh Calkins, MD
Professor of Medicine, Nicholas J. Fortuin Paul Erne, MD
Professor of Cardiology, Johns Hopkins Medical CardioVasc Schweiz, Klinik St. Anna, Lucerne,
Institutions, Johns Hopkins, Baltimore, Switzerland; Department of Cardiology,
Maryland, USA University Heart Center, Zurich, Switzerland
Marina Cerrone, MD Guy H. Fontaine, MD, PhD, HDR
Cardiovascular Genetics Program, Leon H. Unité de Rythmologie, University Pierre et
Charney Division of Cardiology, New York Marie Curie, La Salpêtrière Hospital, Paris,
University School of Medicine, New York, France
New York, USA
Robert Frank, MD
Domenico Corrado, MD, PhD Unité de Rythmologie, University Pierre et
Division of Cardiology, Inherited Marie Curie, La Salpêtrière Hospital, Paris,
Arrhythmogenic Cardiomyopathy Unit, France
Department of Cardiac, Thoracic and Vascular
Judith Groeneweg, MD
Sciences, University of Padua, Italy
Department of Cardiology, University Medical
Center Utrecht and Interuniversity Cardiology
Institute of the Netherlands, Utrecht, The
Netherlands
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