A handbook for clinical practice - part 1 docx

Zipes Distinguished Professor, Professor Emeritus of Medicine, Pharmacology and Toxicology, Director Emeritus, Division of Cardiology, Indiana University, School of Medicine, Krannert In

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A handbook for clinical practice

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THE ESC EDUCATION SERIES

Sudden cardiac

death

A handbook for clinical practice

A publication based on ESC Guidelines

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©2006 European Society of Cardiology

2035 Route des Colles, Les Templiers, 06903 Sophia-Antipolis, France

For further information on the European Society of Cardiology, visit our website:

www.escardio.org

Published by Blackwell Publishing

Blackwell Publishing, Inc., 350 Main Street, Malden, Massachusetts 02148-5020, USA Blackwell Publishing Ltd, 9600 Garsington Road, Oxford OX4 2DQ, UK

Blackwell Science Asia Pty Ltd, 550 Swanston Street, Carlton, Victoria 3053, Australia All rights reserved No part of this publication may be reproduced in any form or by any electronic or mechanical means, including information storage and retrieval systems, without permission in writing from the publisher, except by a reviewer who may quote brief passages in a review.

First published 2006

Library of Congress Cataloging-in-Publication Data

Sudden cardiac death: a handbook for clinical practice/edited by

Silvia G Priori, Douglas P Zipes.

p cm.

Includes bibliographical references.

ISBN-13: 978-1-4051-3292-3

ISBN-10: 1-4051-3292-2

1 Cardiacarrest–Handbooks, manuals, etc I Priori, Silvia G.

II Zipes, Douglas P.

[DNLM: 1 Death, Sudden, Cardiac WG205 S9435 2006]

RC685.C173S7713 2006

616.1’23025–dc22

2005014112 ISBN-13: 978-1-4051-3292-3

ISBN-10: 1-4051-3292-2

A catalogue record for this title is available from the British Library

Set in 9.5/12 Meridien by Newgen Imaging Systems(P) Ltd, Chennai, India

Printed and bound by TJ International, Padstow, UK

Commissioning Editor: Gina Almond

Development Editor: Vicki Donald

For further information on Blackwell Publishing, visit our website:

www.blackwellcardiology.com

The publisher’s policy is to use permanent paper from mills that operate a sustainable forestry policy, and which has been manufactured from pulp processed using acid-free and elementary chlorine-free practices Furthermore, the publisher ensures that the text paper and cover board used have met acceptable environmental accreditation standards.

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Contents

List of contributors, vii

Preface, ix

Section one: Epidemiology and mechanisms

Chapter 1 Epidemiology of cardiac arrest, 3

Robert J Myerburg and Hein J.J Wellens

Chapter 2 Geneticpredisposition and pathology of sudden cardiacdeath, 21

Xavier Jouven, Allen P Burke, and Renu Virmani

Chapter 3 Arrhythmogenicmechanisms, 33

Michiel J Janse and Douglas P Zipes

Chapter 4 Risk stratification for SCD, 47

Stefan H Hohnloser and Wojciech Zareba

Chapter 5 Autonomic nervous system: Emerging concepts and clinicalapplications, 62

Peter J Schwartz and Richard L Verrier

Chapter 6 Clinical characteristics of sudden cardiac death victims andprecipitating events, 74

Christine M Albert and Stuart M Cobbe

Section two: Disease states and special populationsChapter 7 Ischemic heart disease, 91

William Wijns and Elliott M Antman

Chapter 8 The cardiomyopathies, 109

William J McKenna, Srijita Sen-Chowdhry, and Barry J Maron

Chapter 9 Inherited arrhythmogenicdiseases, 132

Silvia G Priori and Charles Antzelevitch

Chapter 10Sudden cardiac death and valvular heart diseases, 147

David Messika-Zeitoun, Bernard J Gersh, Olivier Fondard, and Alec Vahanian

Chapter 11Heart failure, 162

William G Stevenson and Helmut Drexler

v

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Chapter 12Drug-induced sudden death, 177

Dan M Roden and Milou-Daniel Drici

Chapter 13Sudden death in athletes, 189

Domenico Corrado, Cristina Basso, Mark S Link, Gaetano Thiene, and

N.A Mark Estes III

Section three: Treatment

Chapter 14Pharmacology of sudden cardiac death, 205

Timothy W Smith, Michael E Cain, Günter Breithardt, and

Paulus Kirchhof

Chapter 15Implantable devices, 220

A John Camm and Arthur J Moss

Chapter 16Sudden cardiac death: ablation, 237

Prashanthan Sanders, John M Miller, Mélèze Hocini,

Pierre Jạs, and Michel Hạssaguerre

Chapter 17External automated defibrillators, 249

M.A Peberdy, K.A Ellenbogen, and D.A Chamberlain

Chapter 18Cost-effectiveness of implantable cardioverter-defibrillators, 263

Giuseppe Boriani and Greg Larsen

Index, 281

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List of contributors

Editors

Silvia G Priori, MD, PhD, Department of Cardiology, University of Pavia, Salvatore Maugeri

Foundation, Pavia, Italy

Douglas P Zipes Distinguished Professor, Professor Emeritus of Medicine, Pharmacology

and Toxicology, Director Emeritus, Division of Cardiology, Indiana University, School of Medicine, Krannert Institute of Cardiology, Indianapolis, IN, USA

Contributors

Christine M Albert, MD, Brigham and Women’s Hospital, Boston, MA, USA

Elliott M Antman, MD, Brigham and Women’s Hospital, Boston, MA, USA

Charles Antzelevitch, PhD, Masonic Medical Research Laboratory, Utica, NY, USA Cristina Basso, MD, PhD, Department of Cardiology and Pathology, University of

Padua, Italy

Giuseppe Boriani, MD, PhD, Institute of Cardiology, University of Bologna, Bologna,

Italy

Günter Breithardt, MD, FESC, FACC, Department of Cardiology and Angiology,

Hospital of the University of Münster, Germany

Allen P Burke, MD, University of Maryland, Baltimore, MD, USA

Michael E Cain, MD, Cardiovascular Division, Washington University School of

Medicine, St Louis, MO, USA

A John Camm, MD, Cardiological Sciences, St George’s Hospital Medical School,

London, UK

D.A Chamberlain, Wales Heart Research Institute and Prehospital Emergency

Research Unit, College of Medicine, Cardiff University, Cardiff, UK

Stuart M Cobbe, MD, FRCP, Glasgow Royal Infirmary, Glasgow, UK

Domenico Corrado, MD, PhD, Department of Cardiology and Pathology, University of

Padua, Italy

Milou-Daniel Drici, MD, PhD, Department of Pharmacology, Nice-Sophia Antipolis

University Medical Center, Hơpital Pasteur, Nice, Cedex, France

Helmut Drexler, MD, Cardiovascular Division, Medical University of Hannover,

Hannover, Germany

K.A Ellenbogen, MD, Department of Cardiology, Virginia Commonwealth University

Health System, Richmond, VA, USA

N.A MarkEstes III, MD, Tufts University, New England Medical Center, Boston,

MA, USA

Olivier Fondard, MD, Cardiology Department, Bichat Hospital, Paris, France

Bernard J Gersh, MD, ChB, DPhil, FACC, Division of Cardiovascular Diseases and

Internal Medicine, Mayo Clinic and Mayo College of Medicine, Rochester, MN, USA

Michel Hạssaguerre, MD, Hơpital Cardiologique du Haut-Lévêque, Bordeaux, France Mélèze Hocini, MD, Hơpital Cardiologique du Haut-Lévêque, Bordeaux, France

Stefan H Hohnloser, MD, Division of Cardiology, J W Goethe University, Frankfurt,

Germany

Michiel J Janse, MD, The Experimental and Molecular Cardiology Group, Academic

Medical Center, University of Amsterdam, Amsterdam, The Netherlands

vii

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viii List of contributors

Pierre Jạs, MD, Hơpital Cardiologique du Haut-Lévêque, Bordeaux, France

Xavier Jouven, MD, PhD, Hopital Européen Georges Pompidou, University René

Descartes, Paris, France

Paulus Kirchhof, MD, Department of Cardiology and Angiology, Hospital of the

University of Münster, Germany

Greg Larsen, MD, Cardiology Section, Oregon VA Medical Center, Portland, USA MarkS Link, MD, Tufts University, New England Medical Center, Boston, MA, USA Barry J Maron, MD, Minneapolis Heart Institute Foundation, Minneapolis, MN, USA William J McKenna, BA, MD, DSc, The Heart Hospital, University College, London

NHS Foundation Trust, London, UK

David Messika-Zeitoun, MD, Cardiology Department, Bichat Hospital, Paris, France John M Miller, MD, Indiana University School of Medicine, Indianapolis, IN, USA Arthur J Moss, MD, University of Rochester Medical Center, Rochester, NY, USA Robert J Myerburg, MD, University of Miami, Miami, FL, USA

M.A Peberdy, MD, Department of Cardiology, Virginia Commonwealth University

Health System, Richmond, VA, USA

Dan M Roden, MD, Department of Medicine and Pharmacology, Vanderbilt

University School of Medicine, Nashville, TN, USA

Prashanthan Sanders, MBBS, PhD, Hơpital Cardiologique du Haut-Lévêque,

Bordeaux, France

Peter J Schwartz, MD, University of Pavia and Policlinico S Matteo IRCCS, Pavia,

Italy

Srijita Sen-Chowdhry, MA, MBBS, MRCP, The Heart Hospital, University College,

London NHS Foundation Trust, London, UK

Timothy W Smith, MD, DPhil, Cardiovascular Division, Washington University

School of Medicine, St Louis, MO, USA

William G Stevenson, MD, Harvard Medical School, Brigham and Women’s Hospital,

Boston, MA, USA

Gaetano Thiene, MD, Department of Cardiology and Pathology, University of Padua,

Italy

Alec Vahanian, MD, Cardiology Department, Bichat Hospital, Paris, France

Richard L Verrier, PhD, Harvard Medical School, Beth Israel Deaconess Medical

Center, Boston, MA, USA

Renu Virmani, MD, CVPath, International Registry of Pathology, Gaithersburg, MD,

USA

Hein J.J Wellens, MD, Academic Hospital, Maastricht, The Netherlands

William Wijns, MD, Cardiovascular Centre, Aalst, Belgium

Wojciech Zareba, MD, PhD, Department of Medicine, University of Rochester,

Rochester, NY, USA

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Preface

Sudden cardiac death continues to present an important challenge in Europe,the United States, and other developed countries Major difficulties exist inidentifying individuals at risk prior to an episode of a ventricular tachyar-rhythmia or a sudden cardiac arrest, and in responding in a timely fashion tothe person suffering from the catastrophic event out of hospital The EuropeanSociety of Cardiology has established guidelines on how to address some ofthese issues Another set of guidelines on evaluation and treatment of patientswith ventricular arrhythmias and sudden cardiac arrest, created by joint writ-ing committees from the American College of Cardiology, the American HeartAssociation, the European Society of Cardiology, and the Heart Rhythm Soci-ety, will further promote the approaches to individuals with diverse cardiacproblems who are at risk of ventricular tachyarrhythmias and sudden cardiacdeath

This book is part of the ESC Education Series and provides backgroundinformation about the guidelines The focus is to present an update on what

we know about sudden cardiacarrest, from basicexperimental studies to ical trials The book also serves as a compliment to the core syllabus on thistopic

clin-Because sudden cardiac arrest is no respecter of geographic boundaries, wethought a unique contribution would be to have chapters co-authored byexperts on both sides of the Atlanticto derive a truly international view onthe topic Therefore, each chapter has one or more European and Americanauthors presenting a united view of the topic

Chapter topics include epidemiology, genetics, arrhythmogenic isms, risk stratification, autonomic nervous system, phenotypes, and thereare also chapters on disease states and special populations, including coronaryartery disease, cardiomyopathies, inherited diseases, valvular heart disease,heart failure, drugs, and athletes Finally, there are chapters on drug, deviceand ablation treatments, and cost-effectiveness

mechan-We plan future updates as new evidence from clinical and basic scienceprovide substantial innovations to the field An update of the book will parallelthe publication of new sets of Guidelines

We would like to dedicate this book to the memory of two outstanding ologists, Ronald Campbell and Anthony Ricketts, who have made importantcontributions to our understanding of sudden cardiac arrest and tragicallysuccumbed to it

cardi-ix

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x List of contributors

Finally, we would like to thank our spouses, Giulio Zuanetti and Joan Zipes,for their tolerance of the time we have spent in this and other endeavors, andthe support of our children, Andrea and Gabriele Zuanetti, and Debra, Jeffrey,and David Zipes

Silvia G PrioriDouglas P Zipes

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Section one:

Epidemiology and mechanisms

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CHAPTER 1

Epidemiology of cardiac

arrest

Robert J Myerburg and Hein J.J Wellens

Epidemiological studies related to sudden cardiac death (SCD) remainchallenging for both theoretical and practical reasons There are persistingfundamental questions about definition, inconsistencies in access to data, vari-ations in pathophysiological mechanisms and their clinical recognition, anddistinctions between population risk and individual risk In addition, the emer-ging field of genetic epidemiology adds a new dimension for study, and there isneed for focus on interventional epidemiology, the latter being a term coined

to define the population dynamics of therapeutic outcomes This chapter willprovide an overview of each of these components of the epidemiology of SCD

Basic definitions of SCD

A generally accepted definition of SCD is natural death due to cardiac causes,heralded by abrupt loss of consciousness within an hour of the onset of acutesymptoms Preexisting heart disease may or may not have been previouslyrecognized, but the time and mode of death are unexpected [1] The term

“unexpected” is the hallmark of the definition because it permits inclusion of

a broad range of preceding clinical states, having different levels risk

Four time elements must be considered in the construction of a definition ofSCD to satisfy clinical, scientific, legal, and social considerations: prodromes,onset, cardiac arrest, and progression to biological death The proximate cause

of SCD is an abrupt cessation of blood flow that is incompatible with taining life if allowed to persist The 1-h definition is arbitrary and refers tothe duration of the “terminal event,” which defines the interval between theonset of symptoms signaling the pathophysiological disturbance leading tocardiac arrest and the onset of the cardiac arrest itself A 24-h definition may

main-be used as a SCD definition for unwitnessed deaths of victims known to main-be

alive and functioning normally prior to being found, and this is appropriatewithin obvious limits However, the temporal definition used affects the relat-ive incidence of cardiac causes of sudden death and the frequency of specificcardiac disorders [1]

3

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Biological death was viewed as an immediate consequence of cardiac arrest

in the past, usually occurring within minutes However, since the ment of community-based interventions and life support systems, patientsmay now remain biologically alive for a long period of time after the onset of

develop-a pdevelop-athophysiologicdevelop-al process thdevelop-at hdevelop-as cdevelop-aused irreversible ddevelop-amdevelop-age In this cumstance, the causative pathophysiological and clinical event is the cardiacarrest itself, rather than the factors responsible for the delayed biological death.However, for legal, forensic, and certain social considerations, biological death

cir-is the absolute definition, in contrast to cardiac arrest, which retains survivalpotential

Clinical definitions of cardiac arrest and SCD are categorized as “primary”

or “secondary.” These classifications are used in many clinical trials and someepidemiological surveys “Secondary” refers to a cardiac arrest or SCD in anindividual who has survived a prior cardiac arrest or its equivalent Commonuse of the term “primary” is more complex, generally referring to an event

in an individual who has not had a prior cardiac arrest, regardless of the clinicalseverity of the underlying disease The term also refers to arrthythmic collapse

as an initial or isolated feature of the disease (primary cardiac arrest – PCA), inthe absence of a recognized acute state (such as acute myocardial infarction)that is an identified trigger for the event By strict epidemiological definitions,however, none of these usages of “primary” is correct, since the term refers

to the prevention of the underlying disease state, rather than of a clinicalmanifestation Conversely, all cardiac arrests associated with underlying dis-eases are “secondary” events Despite these differences epidemiologically, thecommon usage remains ingrained in clinical medicine

General epidemiology of SCD

Overview

The worldwide incidence of SCD is difficult to estimate because it varies largely

as a function of prevalence of coronary heart disease in different countries[2,3] Estimates for the United States, largely based upon retrospective deathcertificate analyses [4–6] and an emergency rescue database in one study [7]range from less than 200 000 to more than 450 000 SCDs annually, withthe most widely used estimates in the range of 300 000–350 000 SCDs [8].This accounts for an incidence of 0.1–0.2% per year among the population

>35 years of age Event rates in Europe are similar to those in the United States

[9] These ranges of estimates are based, in part, on the definition of suddendeath and inclusion criteria used in individual studies, and the correct num-ber can only be defined from a carefully designed prospective epidemiologicalstudy A recent study in a single city in the United States, using a prospectivedesign for data collection, suggested a significantly lower national incidencewhen extrapolated to the entire country [10] Because of geographic pop-ulation variations [11], however, such extrapolations must be viewed withcaution

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Epidemiology of cardiac arrest 5

Approximately 50% of all coronary heart disease deaths are sudden andunexpected, often occurring shortly after the onset of symptoms Becausecoronary heart disease is the dominant cause of both sudden and nonsuddencardiac deaths in the United States and Europe, the fraction of total cardiacdeaths that are sudden is similar to the fraction of coronary heart disease deathsthat are sudden It is also of interest that the age-adjusted decline in coronaryheart disease mortality in the United States during the past half-century hasnot changed the fraction of coronary deaths that are sudden and unexpected[12,13] Furthermore, the decreasing age-adjusted mortality does not imply

a decrease in absolute numbers of cardiac or sudden deaths because of thegrowth and aging of the population and the increasing prevalence of chronicheart disease [14,15]

Population subgroups and SCD

When the more than 300 000 adult SCDs that occur annually in the UnitedStates are viewed as a global incidence in an unselected adult population, theoverall incidence is 1 to 2/1000 (0.1–0.2%) per year This large populationbase includes those victims whose SCDs occur as a first cardiac event, as well

as those whose SCDs can be predicted with greater accuracy because they areincluded in higher-risk subgroups Any intervention designed for the generalpopulation must be applied to the 999/1000 who will not have an event,

in order to reach and possibly influence the 1/1000 who will have The costand risk-to-benefit uncertainties limit the nature of broad-based interventionsand demand a higher resolution of risk identification Figure 1.1(a) highlightsthis problem by expressing the incidence (percent/year) of SCD among vari-ous subgroups and comparing the incidence figures to the total number ofevents that occur annually in each subgroup By moving from the total adultpopulation to a subgroup at higher risk because of the presence of selectedcoronary risk factors, there may be a 10-fold or greater increase in the incid-ence of events annually, with the magnitude of increase dependent on thenumber of risk factors operating in the subgroup [15] The size of the denomin-ator pool, however, remains very large, and implementation of interventionsremains problematic, even at this heightened level of risk Higher resolution

is desirable and can be achieved by identification of more specific subgroups.However, the corresponding absolute number of deaths become progressivelysmaller as the subgroups become more focused, limiting the potential benefit

of interventions to a much smaller fraction of the total number of patients

at risk Various estimates suggest that at least two-third of all SCDs due tocoronary heart disease occur as a first clinical event or among subgroups ofpatients thought to be at relatively low risk for SCD [12] (Figure 1.1(b)).Time-dependence of risk

Temporal influences on the risk of SCD have been analyzed in the context ofboth biological and clinical chronology In the former, epidemiological ana-lyses of SCD risk among populations have identified three patterns: diurnal,