Clinical Cardiac Electrophysiology in the Young

Macdonald Dick, II Editor

Clinical Cardiac Electrophysiology in the Young

Second Edition

ISBN 978-1-4939-2738-8 ISBN 978-1-4939-2739-5 (eBook) DOI 10.1007/978-1-4939-2739-5

Library of Congress Control Number: 2015945964

Springer New York Heidelberg Dordrecht London © Springer Science+Business Media New York 2006, 2015 This 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. The 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. The 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.

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Editor Macdonald Dick, II, MD Pediatric Cardiology The University of Michigan Congenital Heart Center Department of Pediatrics and Communicable DiseasesUniversity of MichiganAnn Arbor, MI, USA

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The text of Clinical Cardiac Electrophysiology in the Young provides a sys-tematic approach to the anatomy, pathophysiology, basic electrophysiology, diagnosis, and therapy of atrial and ventricular arrhythmias as well as con-duction abnormalities in the young. It elucidates the broad spectrum of rhythm disturbances that may occur from the fetus to young adult, as an isolated abnormality, in the presence of underlying congenital heart disease, both prior to and subsequent to surgical repair. The clinical manifestations, diagnosis, and appropriate pharmacologic and interventional therapy by a trained healthcare team are fully discussed. Science is consistently used to explain the electrophysiologic diagnoses, pharmacologic, interventional, and surgical treatment. Some prior knowledge and understanding of electro-physiology and rhythm disturbances is helpful and the information provided here may be utilized as a guidebook, resource, and reference for residents, cardiology fellows, trained cardiologists, and electrophysiologists as well as other allied health professionals. The rapid advances in the fi eld in such areas as interventional and surgical cryoablation techniques, complexity of rhythm disturbances, new monitoring devices, and pharmaceuticals make it an invaluable text.

Dr. Macdonald Dick as an author and editor of the book is an internation-ally recognized scholar and clinical pediatric electrophysiologist. A superb teacher and role model for trainees and faculty, his affability and diligent effort have brought about the compilation and publication of the book. The majority of the knowledgeable and experienced contributors have received their training in pediatric cardiology at the University of Michigan. The authors are indebted to their medical and surgical colleagues, fellows, family members, and respective institutions for the support and encouragement in the endeavor.

University of Michigan Medical School Amnon Rosenthal , MD Ann Arbor , MI , USA

Foreword to the First Edition

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It takes a certain hubris to come forth with a book entitled Clinical Cardiac Electrophysiology in the Young . There are a number of excellent texts, mono-graphs, and reviews on cardiac arrhythmias in both adults and children—Josephson’s and also Zipes and Jalife’s comprehensive texts come to mind, as well as a number of others, including Deal, Wolff, and Gelband’s, the several volumes from Gillette, and the recent text from Walsh, Saul, and Triedman, the latter three texts focusing on children.

Nonetheless, the past three decades have witnessed enormous advances in the understanding and management of human cardiac arrhythmias. This development represents the fruits of both basic and clinical investigations in cardiac impulse formation and propagation at the organ, tissue, and more recently, cellular and molecular levels. This information explosion may result in information overload and frustrate the student, the young physician in training, as well as the seasoned practitioner. This book focuses on the practi-cal (and theoretical when applicable) aspects of clinical electrophysiology of cardiac arrhythmias in the young. Our intention is that the young house offi -cer or mature physician who is faced with a child with a cardiac arrhythmia will fi nd this book useful in increasing their understanding, sparking their interest, and perhaps leading them to a therapeutic solution.

This book emerges from the clinical practice and research of the pediatric cardiac electrophysiology group in the Division of Pediatric Cardiology at the C.S. Mott Children’s Hospital, the University of Michigan in Ann Arbor, and the former pediatric electrophysiology fellows from Michigan, now established electrophysiologists in their own right. It represents a compilation of the clinical course, electrocardiograms, electrophysiologic studies, phar-macological management, and transcatheter ablation therapy in patients from infancy through young adulthood seen in Ann Arbor and at the current clini-cal sites of the former Michigan fellows. Thus, while the product may be idiosyncratic, it is not provincial. We are interested in “how it is done” but not to the exclusion of other approaches. This is only one (or several) way to address the clinical problem of arrhythmias in children, and surely not the only way, especially as one views the future of emerging energy sources for ablation, nonionizing radiation imaging techniques, and molecular diagnostic possibilities.

The book is divided into two parts. The fi rst part, Background (Chapters 1 – 3 ), discusses the cardiac conduction system—development, anatomy, and physi-ology. Particular attention is directed to the clinical electrophysiology of the

Preface to the First Edition

viii

cardiac conduction system and the techniques of electrophysiologic study that are specifi c to children and that have been developed and practiced at the University of Michigan and at other centers. The second part, Cardiac Electrophysiology in Infants and Children (Chapters 4 – 23 ), focuses on the clinical science of cardiac arrhythmias in infants and children.

Chapters 4 – 12 discuss the mechanism, the ECG characteristics, the elec-trophysiologic fi ndings, the treatment, and the prognosis of tachyarrhyth-mias. Chapters 13 – 16 focus on bradyarrhythmias. Chapters 17 – 20 address certain specialized subjects including syncope, cardiac pacemakers, implant-able cardiac defi brillators, genetic disorders of the cardiac impulse, fetal arrhythmias, and sudden cardiac death as it occurs in the young. Chapters 21 – 22 center on the pharmacology of antiarrhythmic agents, indications for use, doses, side effects, and toxicity, as well as on transcatheter arrhythmia ablation. Finally, what the practitioner can expect to see from the impact of cardiac arrhythmias on the life of the patient and family is discussed from the nursing point of view in Chapter 23 .

The intent of the book is practical and thus the suggested readings are selected and not encyclopedic. They are meant as a starting place for the interested reader. Examples and tables are included in the anticipation that the reader will rapidly be able to match the clinical problem to the examples and the accompanying text.

A text or technical book is rarely the product of a single individual. With that in mind, any value or sense that can be made of this work is solely due to the terrifi c efforts of the authors; any error or fault can be correctly attributed to me. I am deeply grateful to all of the authors for their contributions, as well as their patience in bringing the project together. I want to recognize the gen-erosity of my colleagues at Michigan in providing coverage when I would hide out (including a sabbatical) to work on the text. Thanks also to the medi-cal electrophysiology group at Michigan for encouragement and support for the pediatric program. I also want to thank my local editor, Kathryn Clark, for all her efforts in keeping me on task, endlessly and repeatedly formatting the multiple revisions of the text, and fi nding and eliminating too many examples of “nonsense” to count. Finally, I want to thank Melissa Ramondetta at Springer for her great patience, great good humor, and sound advice through-out the course of the project. Carolin, my wife, graciously permitted me to weed the book of its unwanted wordage (probably missed a bit) rather than our yard of unwanted plant life on numerous weekends.

Ann Arbor, MI Macdonald Dick, II, MD August 2005

Preface to the First Edition

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A second edition of any text book calls for some justifi cation. Of course, the short answer is that new technology and information have emerged, but no doubt a longer answer is required. First, a wider application of cryotherapy for arrhythmia in young patients has reduced the low but real risk of heart block (Chapter 23 and others). Second, navigation systems detecting elec-trode catheters within the heart and displaying their relative positions and shapes in real time in “virtual three dimensions” on a monitor screen have led to a marked reduction in radiation exposure to the young patient (Chapters 3 , 23 , and others). Third, classical embryogenesis has relinquished center stage to molecular biology exploring the role of a vast and sequential (and often dizzying) array of multiple genetic signals and protein products for success-ful cardiac development, including that of the structure and function of the cardiac conduction system (Chapters 1 and 2 ). Fourth, our understanding of inherited genetic mechanisms of arrhythmias has greatly enlarged. It is now clear that 10–15 % of SIDS victims have associated mutations in their cardiac ion channels, likely contributing to their demise. This rapid growth in our understanding of genetic mechanisms of disease not only has led to molecu-lar diagnoses for inherited arrhythmias but also has unmasked the complexity of genetic mutation and phenotypic expression (Chapter 19 ). Fifth, best prac-tice policies and consensus statements have given guidance to both referring physicians and electrophysiology cardiologists who take care of children with arrhythmias. Sixth, more information and discussion, if not yet fi rm policy, has emerged regarding sudden cardiac death in the young, particularly athletes. Conferences and symposia have been held, papers have been pub-lished, and the discussion has been thoughtful and vigorous. The role of car-diac devices and ECG screening in young patients is challenged by the infrequency of an abnormality posing risk, the low frequency of an event even in an genotype positive individual, the cost of detecting a low risk a priori and, fi nally, competing values (cost-effective analysis; individual ver-sus the public interest). This discussion is importantly expanded in the chap-ter on sudden cardiac death in the young (Chapter 21 ). Finally, a new cohort of young electrophysiologists brings their energy, insight, questions, and research to the fi eld. All of the authors are practicing clinical cardiac electro-physiologists. All, as in the fi rst edition, are linked to the Michigan Congenital Heart Center at the University of Michigan, directly as faculty or former fel-lows; many have produced their own generation of independent cardiologists who have signed on as authors.

Preface to the Second Edition

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The intent of the book is to present the “thinking and style” of the authors when they manage young patients with arrhythmias. Thus, the “Suggested Reading” rather than cited references in the text. There is two exceptions—Chapters 1 and 19 . Because of the complex nature of the material, the specifi c citations within the text are retained.

I am indebted to all of the authors for their contributions, and especially, to my wife, who graciously reviewed many of the manuscripts. I also want to thank Mr. Michal D. Sova, Springer Developmental Editor, for his generous patience in extending our deadline as needed. In addition, thanks go to Ms. Lana Emmons for securing permissions for copyrighted material, review-ing and organizing “Suggested Readings” lists, and preparing much of the manuscript. Any errors, as before, are mine.

Ann Arbor, MI Macdonald Dick II, MD February 2015

Preface to the Second Edition

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Part I Background

1 Development and Structure of the Cardiac Conduction System ....................................................................... 3 Parvin Dorostkar and Mark W. Russell

2 Physiology of the Cardiac Conduction System .......................... 31 Adam C. Kean and Peter S. Fischbach

3 Clinical Electrophysiology of the Cardiac Conduction System ....................................................................... 49 Martin J. LaPage , Ian H. Law , and Macdonald Dick, II

Part II Clinical Electrophysiology in Infants and Children

4 Atrioventricular Reentry Tachycardia........................................ 75 Vincent C. Thomas , Nicholas Von Bergen , and Ian H. Law

5 Atrioventricular Nodal Reentry Tachycardia ............................ 95 David J. Bradley , Nicholas Von Bergen , and Martin J. LaPage

6 Persistent Junctional Reciprocating Tachycardia ...................... 107 Parvin Dorostkar

7 Sinoatrial Reentrant Tachycardia: Inappropriate Sinus Tachycardia ......................................................................... 117 Macdonald Dick, II

8 Atrial Flutter Intra-Atrial Reentrant Tachycardia ................... 123 Nicholas Von Bergen , Ian H. Law , and Macdonald Dick, II

9 Atrial Fibrillation .......................................................................... 141 Peter S. Fischbach

10 Atrial Ectopic Tachycardias ......................................................... 149 Burt I. Bromberg and Andrew Papez

11 Junctional Tachycardia: Congenital, Acquired, Postoperative ................................................................................. 157 Adam C. Kean , Matthew Hazle , Martin J. LaPage , and Burt I. Bromberg

Contents

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12 Multifocal Atrial Tachycardia...................................................... 171 Johannes C. von Alvensleben and David J. Bradley

13 Ventricular Tachycardia ............................................................... 175 Nicholas Von Bergen and Craig J. Byrum

14 Sick Sinus Syndrome .................................................................... 197 Ira Shetty and William A. Scott

15 First- and Second-Degree Atrioventricular Block ..................... 209 Anna Kamp and William A. Scott

16 Complete Atrioventricular Block Third-Degree Heart Block .................................................................................... 221 Anna Kamp and William A. Scott

17 Syncope .......................................................................................... 231 Margaret J. Strieper , Robert Campbell , and William A. Scott

18 Cardiac Pacemakers and Implantable Cardioverter-Defibrillators .......................................................... 245 Gerald A. Serwer and Ian H. Law

19 Genetic Disorders of the Cardiac Impulse .................................. 267 Adam S. Helms , Patricia L. Arscott , Stephanie Wechsler , and Mark W. Russell

20 Fetal Arrhythmias ......................................................................... 297 Elizabeth Vickers Saarel and Carlen G. Fifer

21 Sudden Cardiac Death in the Young ........................................... 315 Christopher B. Stefanelli , Aarti Dalal , and Robert Campbell

22 Pharmacology of Anti-arrhythmic Agents ................................. 333 Peter S. Fischbach and Srikant Das

23 Transcatheter Ablation of Cardiac Arrhythmias in the Young ................................................................................... 357 Martin J. LaPage , Ian H. Law , and Macdonald Dick, II

24 Allied Professional Roles in the Management of Arrhythmias in the Young ....................................................... 381 Brynn E. Dechert and Sarah S. LeRoy

Index ....................................................................................................... 389

Contents

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Patricia L. Arscott , MS Pediatric Cardiology , The University of Michigan Congenital Heart Center, Department of Pediatrics and Communicable Diseases, University of Michigan , Ann Arbor , MI , USA

David J. Bradley , MD Pediatric Cardiology , The University of Michigan Congenital Heart Center, Department of Pediatrics and Communicable Diseases, University of Michigan , Ann Arbor , MI , USA

Burt I. Bromberg , MD Department of Pediatrics , Mercy Clinic , St. Louis , MO , USA

Craig J. Byrum , MD Pediatrics—Cardiology , Syracuse , NY , USA

Robert Campbell , MD Sibley Heart Center , Children’s Healthcare of Atlanta, Department of Pediatrics, Emory University School of Medicine , Atlanta , GA , USA

Aarti Dalal , DO Sibley Heart Center , Children’s Healthcare of Atlanta, Department of Pediatrics, Emory University School of Medicine , Atlanta , GA , USA

Srikant Das , MD Department of Pediatric Cardiology , Arkansas Children’s Hospital, University of Arkansas for Medical Sciences , Little Rock , AR , USA

Brynn E. Dechert , MSN, CPNP Pediatric Cardiology , The University of Michigan Congenital Heart Center, Department of Pediatrics and Communicable Diseases, University of Michigan , Ann Arbor , MI , USA

Macdonald Dick II , MD Pediatric Cardiology , The University of Michigan Congenital Heart Center, Department of Pediatrics and Communicable Diseases, University of Michigan , Ann Arbor , MI , USA

Parvin Dorostkar , MD, MS, MBA Pediatric Cardiology, Department of Pediatrics , University of Minnesota , St. Paul , MN , USA

Carlen G. Fifer , MD Pediatric Cardiology , The University of Michigan Congenital Heart Center, Department of Pediatrics and Communicable Diseases, University of Michigan , Ann Arbor , MI , USA

Contributors

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Peter S. Fischbach , MD, MS Sibley Heart Center , Children’s Healthcare of Atlanta, Department of Pediatrics, Emory University School of Medicine , Atlanta , GA , USA

Matthew Hazle , MD Pediatric Cardiology , Wake Forest Baptist Health, Wake Forest University , Winston Salem , NC , USA

Adam S. Helms , MD, MS Department of Internal Medicine , University of Michigan Health Systems , Ann Arbor , MI , USA

Anna Kamp , MD Pediatric Cardiology, Department of Pediatrics , Ohio State University , Columbus , OH , USA

Adam C. Kean , MD, MPH Section of Pediatric Cardiology , Indiana University School of Medicine , Indianapolis , IN , USA

Martin J. LaPage , MD, MS Pediatric Cardiology , The University of Michigan Congenital Heart Center, Department of Pediatrics and Communicable Diseases, University of Michigan , Ann Arbor , MI , USA

Ian H. Law , BSE, MD Division of Cardiology, Department of Pediatrics , University of Iowa Children’s Hospital, University of Iowa Carver College of Medicine , Iowa City , IA , USA

Sarah S. LeRoy , RN, MSN, DNP Department of Nursing , Wayne State University College of Nursing , Detroit , MI , USA

Andrew Papez , MD Pediatric Cardiology , Arizona Pediatric Cardiology/Phoenix Children’s Hospital , Phoenix , AZ , USA

Mark W. Russell , MD Pediatric Cardiology , The University of Michigan Congenital Heart Center, Department of Pediatrics and Communicable Diseases, University of Michigan , Ann Arbor , MI , USA

Elizabeth Vickers Saarel , MD Department of Pediatrics , University of Utah , Salt Lake City , UT , USA

William A. Scott , MD Department of Pediatrics , UT Southwestern Medical Center , Dallas , TX , USA

Gerald A. Serwer , MD Pediatric Cardiology , The University of Michigan Congenital Heart Center, Department of Pediatrics and Communicable Diseases, University of Michigan , Ann Arbor , MI , USA

Ira Shetty , MD Pediatric Cardiology , Advocate Children’s Hospital , Oak Lawn , IL , USA

Christopher B. Stefanelli , MD Department of Pediatric Cardiology , Hawaii Permanente Medical Group , Honolulu , HI , USA

Margaret J. Strieper , DO Sibley Heart Center , Children’s Healthcare of Atlanta, Department of Pediatrics, Emory University School of Medicine , Atlanta , GA , USA

Contributors

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Vincent C. Thomas , MD, MHA Department of Pediatrics , University of Nevada School of Medicine , Las Vegas , NV , USA

Johannes C. von Alvensleben , MD Pediatric Cardiology , The University of Michigan Congenital Heart Center, Department of Pediatrics and Communicable Diseases, University of Michigan , Ann Arbor , MI , USA

Nicholas Von Bergen , MD Department of Pediatrics , University of Wisconsin—Madison , Madison , WI , USA

Stephanie Wechsler , MD Department of Pediatric Cardiology , DUMC , Durham , NC , USA

Contributors