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  1. 1. Echocardiography in Pediatric and Adult Congenital Heart Disease EDITORS Benjamin W. Eidem, MD, FACC, FASE Associate Professor of Pediatrics Director, Pediatric and Fetal Echocardiography Laboratory Divisions of Pediatric Cardiology and Cardiovascular Diseases College of Medicine, Mayo Clinic Rochester, Minnesota Frank Cetta, MD, FACC, FASE Professor of Pediatrics and Medicine Divisions of Pediatric Cardiology and Cardiovascular Diseases College of Medicine, Mayo Clinic Rochester, Minnesota Patrick W. O’Leary, MD, FACC, FASE Associate Professor of Pediatrics Divisions of Pediatric Cardiology and Cardiovascular Diseases College of Medicine, Mayo Clinic Rochester, MinnesotaLWW-EIDEM-09-0305-FM.indd i 9/2/09 3:50:46 PM
  2. 2. Acquisitions Editor: Frances R. DeStefano Product Manager: Leanne McMillan Production Manager: Bridgett Dougherty Senior Manufacturing Manager: Benjamin Rivera Marketing Manager: Kimberly Schonberger Design Coordinator: Stephen Druding Production Service: KnowledgeWorks Global Limited © 2010 by LIPPINCOTT WILLIAMS & WILKINS, a WOLTERS KLUWER business 530 Walnut Street Philadelphia, PA 19106 USA Mayo Foundation for Medical Education and Research holds copyright on photographs, drawings, images, video clips, and other illustrations submitted by Mayo editors and contributors. All rights reserved. This book is protected by copyright. No part of this book may be re- produced in any form by any means, including photocopying, or utilized by any information storage and retrieval system without written permission from the copyright owner, except for brief quotations embodied in critical articles and reviews. Materials appearing in this book prepared by individuals as part of their official duties as U.S. government employees are not covered by the above-mentioned copyright. Printed in China Library of Congress Cataloging-in-Publication Data Echocardiography in pediatric and adult congenital heart disease / editors, Benjamin W. Eidem, Frank Cetta, Patrick W. O’Leary. p. ; cm. Includes bibliographical references. ISBN-13: 978-0-7817-8136-7 ISBN-10: 0-7817-8136-1 1. Congenital heart disease—Ultrasonic imaging. 2. Congenital heart disease in children– Ultrasonic imaging. 3. Echocardiography. I. Eidem, Benjamin W. II. Cetta, Frank. III. O’Leary, Patrick W. [DNLM: 1. Heart Defects, Congenital–ultrasonography. 2. Echocardiography—methods. WG 220 E185 2010] RC687.E26 2010 616.1207543—dc22 2009029035 Care has been taken to confirm the accuracy of the information presented and to describe generally accepted practices. However, the authors, editors, and publisher are not responsible for errors or omissions or for any consequences from application of the information in this book and make no warranty, expressed or implied, with respect to the currency, completeness, or accuracy of the contents of the publication. Application of the information in a particular situation remains the professional responsibility of the practitioner. The authors, editors, and publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accordance with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in gov- ernment regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any change in in- dications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new or infrequently employed drug. Some drugs and medical devices presented in the publication have Food and Drug Administration (FDA) clearance for limited use in restricted research settings. It is the respon- sibility of the health care provider to ascertain the FDA status of each drug or device planned for use in their clinical practice. To purchase additional copies of this book, call our customer service department at (800) 638- 3030 or fax orders to (301) 223-2320. International customers should call (301) 223-2300. Visit Lippincott Williams & Wilkins on the Internet: at Lippincott Williams & Wilkins customer service representatives are available from 8:30 am to 6 pm, EST. 10 9 8 7 6 5 4 3 2 1LWW-EIDEM-09-0305-FM.indd ii 9/2/09 3:50:51 PM
  3. 3. Preface Evolution of Echocardiography in Congenital Heart Disease advent of three-dimensional echocardiography have all added to the clinical echocardiographer’s ability to properly assess anatomy The editors are indebted to the historic and ground-breaking work and physiology and impact clinical care for patients with congenital that Drs. James B. Seward and A. Jamil Tajik performed in the heart disease. Currently, the vast majority of children who are born 1970s and 1980s as pioneers of the use of echocardiography in con- with heart disease and require surgery within the first 6 months of genital heart disease. The editors were educated by these two great life generally go to surgery with only echocardiographic assessment echocardiographers and much, if not all, of what follows in this text of their cardiac anatomy and physiology. It is rare in the current is a result of the genius and perspiration of Drs. Seward and Tajik. clinical era for cardiac catheterization to be performed for diag- In 1987, Drs. Seward and Tajik in conjunction with Dr. William nostic purposes in young infants. In fact, congenital cardiac cath- D. Edwards (Mayo Clinic Department of Pathology) and Dr. Donald J. eterization has become more interventional and has evolved as an Hagler (Mayo Clinic Division of Pediatric Cardiology) published one alternative to surgical procedures rather than a field of pure diagno- of the earliest textbooks dedicated to the field of congenital echo- sis. Echocardiographic and, to a large extent, magnetic resonance cardiography, entitled Two-Dimensional Echocardiographic Atlas of imaging advances in the past three decades have streamlined the Congenital Heart Disease. In the introduction of that textbook, the evaluation of these patients and made noninvasive assessment the following quote appeared: cornerstone of diagnosis. Similar to the Atlas that Drs. Seward, Tajik, Edwards, and Hagler The tomographic approach to congenital anomalies is the constructed in the 1980s, this textbook will provide a comprehen- imaging modalities of the 1980s and is applicable to echocar- sive review of congenital anomalies that shall serve as a reference diography, computerized tomography and magnetic resonance for echocardiographers at all levels of interest and expertise. imaging. It is the building block from which the expected three The editors and all those who practice congenital echocardio- dimensional imaging techniques of the 1990s will be deve- graphy are indebted to the immense contributions by Drs. Seward loped. The widespread application of these imaging moda- and Tajik. lities has rekindled interest in cardiac anatomy and pathology, Special mention should also be made to the current contribu- particularly in the evaluation of patients with congenital heart tions of Drs. Edwards and Hagler. Dr. Edwards is a renowned car- disease. Each clinical cardiologist now has the tools at hand to diac pathologist who for many years has served as an invaluable make preliminary, if not detailed, morphologic diagnoses. No resource to the Mayo Clinic Echocardiography Laboratory. Dozens longer is it necessary to refer a patient to the cardiac catheteri- of photographs of pathologic specimens from his collection have zation laboratory or surgical amphitheater with less than a been used in this textbook. Dr. Hagler has been instrumental in near complete evaluation of the underlying congenital ano- the development of noninvasive imaging in fetuses, children, and maly. This capability has led to and will continue to contribute adults with congenital heart disease and has been a pioneer in to the rationale and expeditious management of patients with the use of echocardiography as a complementary imaging modality congenital heart disease. in the congenital cardiac catheterization laboratory. His contribu- tion to this textbook is demonstrated by the number of authors These statements from the 1980s have become fact in this cen- who he has mentored during his career. Both Dr. Hagler’s and Dr. tury. Advances in resolution of two-dimensional imaging with tech- Edward’s work at Mayo have significantly impacted the assess- niques such as harmonic imaging, advances in Doppler technology ment and management of thousands of patients with congenital including spectral, color, and tissue Doppler techniques and the heart disease. iiiLWW-EIDEM-09-0305-FM.indd iii 9/2/09 3:50:51 PM
  4. 4. iv Contents Contributors Naser M. Ammash, MD Michael G. Earing, MD Peter C. Frommelt, MD Associate Professor of Medicine Associate Professor of Internal Medicine and Professor Department of Cardiovascular Diseases Pediatrics Department of Pediatric Cariology College of Medicine, Mayo Clinic Divisions of Adult Cardiovascular Medicine Medical College of Wisconsin Rochester, Minnesota and Pediatric Cardiology Director Medical College of Wisconsin Pediatric Echocardiography Nancy Ayres, MD Director of the Adult Congenital Heart Children’s Hospital of Wisconsin Assistant Professor Disease Program Milwaukee, Wisconsin Department of Pediatrics (Cardiology) Children’s Hospital of Wisconsin Baylor College of Medicine Froedtert Memorial Lutheran Hospital Javier Ganame, MD, PhD Director of Non-Invasive Imaging and Milwaukee, Wisconsin Assistant Professor Perinatal Cardiology Cardiology Department Texas Children’s Hospital William D. Edwards, MD Catholic University of Leuven Houston, Texas Professor of Pathology Staff Cardiologist Laboratory Medicine and Pathology Cardiology/Radiology Departments Peter J. Bartz, MD College of Medicine, Mayo Clinic University Hospital Leuven Assistant Professor of Pediatrics and Consultant, Anatomic Pathology Leuven, Belgium Medicine Laboratory Medicine and Pathology Departments of Pediatric Cardiology and Mayo Clinic Sarah Gelehrter, MD Cardiovascular Medicine Rochester, Minnesota Assistant Professor, Clinical Medical College of Wisconsin Department of Pediatrics Children’s Hospital of Wisconsin Benjamin W. Eidem, MD, FACC, FASE University of Michigan Milwaukee, Wisconsin Associate Professor of Pediatrics Pediatric Cardiologist Director, Pediatric and Fetal Department of Pediatrics Stuart Berger, MD Echocardiography Laboratory C.S. Mott Children’s Hospital Professor Divisions of Pediatric Cardiology and Ann Arbor, Michigan Department of Pediatrics Cardiovascular Diseases Medical College of Wisconsin College of Medicine, Mayo Clinic Edmund Gillis, BS, RDCS Medical Director Rochester, Minnesota Herma Heart Center Martha Grogan, MD Children’s Hospital of Wisconsin Gregory Ensing, MD Assistant Professor of Medicine Milwaukee, Wisconsin Professor of Pediatrics Division of Cardiovascular Diseases Department of Pediatrics Mayo Foundation William L. Border, MD University of Michigan Rochester, Minnesota Pediatric Cardiology Pediatric Cardiologist Cincinnati Children’s Hospital Medical Department of Pediatrics Donald J. Hagler, MD Center C.S. Mott Children’s Hospital Consultant, Professor of Pediatrics Cincinnati, Ohio Ann Arbor, Michigan Departments of Pediatric Cardiology Mayo Clinic Allison K. Cabalka, MD Malek M. El Yaman, MD Rochester, Minnesota Associate Professor of Pediatrics Pediatric Cardiology Fellow Mayo Clinic College of Medicine Department of Pediatrics Frederick D. Jones, RDCS Consultant Mayo Clinic Pediatric Cardiac Sonographer Division of Pediatric Cardiology Rochester, Minnesota Echocardiography Laboratory Mayo Clinic Kentucky Children’s Heart Center Rochester, Minnesota Bernadette Fenstermaker, RDCS Kentucky Children’s Hospital Technical Director Lexington, Kentucky Frank Cetta, MD, FACC, FASE Echocardiography Laboratory, Professor of Pediatrics and Medicine The Heart Center Thomas R. Kimball, MD Divisions of Pediatric Cardiology and Nationwide Children’s Hospital Professor of Pediatrics Cardiovascular Diseases Columbus, Ohio University of Cincinnati College of Medicine College of Medicine, Mayo Clinic Director, Echocardiography Rochester, Minnesota Mark A. Fogel, MD, FACC, FAAP Cincinnati Children’s Hospital Medical Center Associate Professor of Pediatrics, Cardiology Cincinnati, Ohio Heidi M. Connolly, MD and Radiology Cardiologist Director of Cardiac MR John P. Kovalchin, MD Division of Cardiovascular Diseases The University of Pennsylvania School of Associate Professor Mayo Clinic Medicine Department of Pediatrics St. Mary’s Hospital and Rochester Methodist The Children’s Hospital of Philadelphia The Ohio State University Hospital Philadelphia, Pennsylvania Director of Echocardiography Rochester, Minnesota The Heart Center at Nationwide Children’s Michele A. Frommelt, MD Hospital David J. Driscoll, MD Assistant Professor Columbus, Ohio Professor of Pediatrics Department of Pediatric Cardiology Departments of Cardiovascular Diseases Medical College of Wisconsin and Pediatrics Director College of Medicine, Mayo Clinic Fetal Echocardiography Mayo Clinic Transplant Center Children’s Hospital of Wisconsin Rochester, Minnesota Milwaukee, Wisconsin ivLWW-EIDEM-09-0305-FM.indd iv 9/2/09 3:50:53 PM
  5. 5. Contributors v Mark Lewin, MD Shobha Natarajan, MD James B. Seward, MD Professor of Pediatrics and Chief of Attending Cardiologist Consultant, Division of Cardiovascular Cardiology Assistant Professor of Pediatrics Diseases University of Washington School of Medicine The Children’s Hospital of Philadelphia Mayo Clinic Co-Director, Heart Center Philadelphia, Pennsylvania John M. Nasseff, Sr., Professor in Cardiology Department of Pediatrics in Honor of Dr. Burton Onofrio Seattle Children’s Hospital Patrick W. O’Leary, MD, FACC, FASE Professor of Medicine and of Pediatrics Seattle, Washington Associate Professor of Pediatrics Mayo Clinic College of Medicine Divisions of Pediatric Cardiology and Rochester, Minnesota Robert Lichtenberg, MD, FACC Cardiovascular Diseases Director, Adult Congenital Heart Clinic College of Medicine, Mayo Clinic Girish S. Shirali, MBBS Department of Medicine Rochester, Minnesota Professor Heart Care Centers of Illinois Departments of Pediatrics and Obstetrics & Mokena, Illinois Jae K. Oh, MD Gynecology Professor of Medicine Medical University of South Carolina Leo Lopez, MD Co-Director, Echocardiography Laboratory Director, Pediatric Echocardiography Associate Professor Department of Cardiovascular Diseases Medical University of South Carolina Department of Clinical Pediatrics Mayo Clinic Children’s Hospital Albert Einstein College of Medicine Rochester, Minnesota Charleston, South Carolina Director, Pediatric Cardiac Noninvasive Imaging Angira Patel, MD, MPH Jeffrey F. Smallhorn, MBBS, FRACP, Pediatric Cardiology Division Pediatric Cardiology Fellow FRCP(C) Children’s Hospital at Montefiore Department of Pediatrics Professor Bronx, New York Northwestern University, Feinberg School of Department of Pediatrics Medicine University of Alberta William T. Mahle, MD Department of Pediatric Cardiology Head, Section of Echocardiography Staff Pediatric Cardiologist Children’s Memorial Hospital Pediatric Cardiology Children’s Healthcare of Atlanta, Emory Chicago, Illinois Stollery Children’s Hospital University School of Medicine Edmonton, Alberta, Canada Atlanta, Georgia Sabrina D. Phillips, MD Assistant Professor of Medicine Ronald Springer, BS, RDCS Colin J. McMahon, MD Department of Cardiovascular Disease Consultant Paediatric Cardiologist Mayo Clinic Anita L. Szwast, MD Department of Paediatric Cardiology, Rochester, Minnesota Assistant Professor of Pediatric Cardiology Our Lady’s Hospital for Sick Children Department of Pediatrics, Division of Dublin, Ireland Ricardo H. Pignatelli, MD Cardiology Assistant Professor of Pediatrics University of Pennsylvania School of Shaji Menon, MD Department of Pediatrics Medicine Assistant Professor of Pediatrics Baylor College of Medicine Attending Cardiologist Adjunct Professor of Radiology Texas Children’s Hospital Department of Pediatrics, Division of Department of Pediatric Cardiology Houston, Texas Cardiology University of Utah Children’s Hospital of Philadelphia Salt Lake City, Utah Jack Rychik, MD Philadelphia, Pennsylvania Professor of Pediatrics Luc Mertens, MD, PhD The Children’s Hospital of Philadelphia Lloyd Y. Tani, MD Associate Professor University of Pennsylvania School of Professor of Pediatrics Department of Paediatrics Medicine Department of Pediatrics University of Toronto Director, Fetal Heart Program University of Utah School of Medicine Section Head, Echocardiography The Cardiac Center at The Children’s Division Chief, Pediatric Cardiology Department of Cardiology Hospital of Philadelphia Primary Children’s Medical Center The Hospital for Sick Children Philadelphia, Pennsylvania Salt Lake City, Utah Toronto, Ontario, Canada Ritu Sachdeva, MBBS, FACC Himesh V. Vyas, MD Erik C. Michelfelder, MD Associate Professor Assistant Professor Associate Professor Department of Pediatrics, Division of Department of Pediatric Cardiology Department of Pediatrics Pediatric Cardiology University of Arkansas for Medical Sciences University of Cincinnati College of Medicine University of Arkansas for Medical Assistant Professor Director, Fetal Heart Program Sciences Department of Pediatric Cardiology The Heart Institute Medical Director, Heart Station Arkansas Children’s Hospital Cincinnati Children’s Hospital Medical Center Arkansas Children’s Hospital Little Rock, Arkanasas Cincinnati, Ohio Little Rock, Arkansas Luciana T. Young, MD Fletcher A. Miller, MD Ivan Salgo, MD Associate Professor Department of Cardiovascular Diseases Chief, Cardiovascular Investigations Department of Pediatrics Mayo Clinic Fellow Northwestern University, Feinberg School of Rochester, Minnesota Ultrasound Research and Development Medicine Philips Healthcare Department of Pediatric Cardiology L. LuAnn Minich, MD Andover, Massachusetts Children’s Memorial Hospital Professor of Pediatrics Chicago, Illinois Pediatric Administration Amy H. Schultz, MD University of Utah Assistant Professor Robert Young, BA, RDCS Director of Clinical Research/Cardiologist Department of Cardiology Academic Instructor Department of Pediatric Cardiology University of Washington Echo Program Primary Children’s Medical Center Attending Physician Mayo School of Health Sciences Salt Lake City, Utah Department of Cardiology Cardiac Sonographer Seattle Children’s Hospital Mayo Echo Lab Seattle, Washington Mayo Clinic Rochester, MinnesotaLWW-EIDEM-09-0305-FM.indd v 9/2/09 3:50:54 PM
  6. 6. Acknowledgments To my wife, Debbie, my daughter, Katie, and my family for their love, support, and sacrifices throughout my medical career.—BWE I am extremely thankful to my children, Cate, Michael, and Matt, for the sacrifices they have made for my career.—FC To my mentors, patients, and, most of all, my family. Thank you for the examples you have provided, the patience you have shown, and the love you have given.—PWO The editors would like to sincerely thank Mark Zangs, Jeff Stelley, and Vern Weber for their significant efforts in the preparation of many of the figures and videos throughout this textbook. viLWW-EIDEM-09-0305-FM.indd vi 9/2/09 3:50:54 PM
  7. 7. Contents Preface iii Contributors iv Acknowledgments vi ■ CHAPTER 1 Principles of Cardiovascular Ultrasound 1 ROBERT YOUNG AND PATRICK W. O’LEARY ■ CHAPTER 2 Practical Issues Related to the Examination, Anatomic Image Orientation, and Segmental Cardiovascular Analysis 10 EDMUND GILLIS, RONALD SPRINGER, AND PATRICK W. O’LEARY ■ CHAPTER 3 Quantitative Methods in Echocardiography—Basic Techniques 29 BENJAMIN W. EIDEM AND PATRICK W. O’LEARY ■ CHAPTER 4 Quantitative Methods in Echocardiography—Advanced Techniques in the Assessment of Ventricular Function 48 JAVIER GANAME AND LUC MERTENS ■ CHAPTER 5 Anomalies of the Pulmonary and Systemic Venous Connections 70 FRANK CETTA AND NASER AMMASH ■ CHAPTER 6 Abnormalities of Atria and Atrial Septation 87 ALLISON K. CABALKA ■ CHAPTER 7 Atrioventricular Septal Defects 105 MALEK EL YAMAN, WILLIAM D. EDWARDS, AND FRANK CETTA ■ CHAPTER 8 Ebstein’s Malformation and Tricuspid Valve Diseases 116 PATRICK W. O’LEARY ■ CHAPTER 9 Echocardiographic Assessment of Mitral Valve Abnormalities 131 JEFFREY F. SMALLHORN ■ CHAPTER 10 Congenitally Corrected Transposition of the Great Arteries 145 MICHAEL EARING, NANCY AYRES, AND FRANK CETTA ■ CHAPTER 11 Ventricular Septal Defects 158 SARAH GELEHRTER AND GREGORY ENSING ■ CHAPTER 12 Univentricular Atrioventricular Connections 176 SHAJI MENON AND ALLISON K. CABALKA viiLWW-EIDEM-09-0305-FM.indd vii 9/2/09 3:50:55 PM
  8. 8. viii Contents ■ CHAPTER 13 Abnormalities of Right Ventricular Outflow 196 ERIK C. MICHELFELDER AND WILLIAM L. BORDER ■ CHAPTER 14 Abnormalities of Left Ventricular Outflow 215 LEO LOPEZ ■ CHAPTER 15 Tetralogy of Fallot 237 HIMESH VYAS AND BENJAMIN W. EIDEM ■ CHAPTER 16 d-Transposition of the Great Arteries 249 AMY H. SCHULTZ AND MARK B. LEWIN ■ CHAPTER 17 Double-Outlet Right and Left Ventricles 269 DONALD J. HAGLER ■ CHAPTER 18 Truncus Arteriosus 277 FREDERICK D. JONES, BERNADETTE FENSTERMAKER, AND JOHN P. KOVALCHIN ■ CHAPTER 19 Patent Ductus Arteriosus and Aortopulmonary Window 283 RITU SACHDEVA ■ CHAPTER 20 Abnormalities of the Aortic Arch 289 ANGIRA PATEL AND LUCIANA T. YOUNG ■ CHAPTER 21 Marfan Syndrome: Aortic Aneurysm and Dissection 298 HEIDI M. CONNOLLY ■ CHAPTER 22 Hypertrophic Cardiomyopathy 311 PATRICK W. O’LEARY ■ CHAPTER 23 Additional Cardiomyopathies 320 COLIN J. MCMAHON AND RICARDO H. PIGNATELLI ■ CHAPTER 24 Pericardial Disorders 330 MARTHA GROGAN AND JAE K. OH ■ CHAPTER 25 Vascular Abnormalities 342 MICHELE A. FROMMELT AND PETER C. FROMMELT ■ CHAPTER 26 Cardiac Tumors 355 DAVID J. DRISCOLL, FRANK CETTA, AND BENJAMIN W. EIDEM ■ CHAPTER 27 Evaluation of the Transplanted Heart 362 WILLIAM T. MAHLE ■ CHAPTER 28 Pulmonary Hypertension 368 PETER BARTZ AND STUART BERGERLWW-EIDEM-09-0305-FM.indd viii 9/2/09 3:50:56 PM
  9. 9. Contents ix ■ CHAPTER 29 Echocardiography in the Diagnosis and Management of Endocarditis 375 L. LUANN MINICH AND LLOYD Y. TANI ■ CHAPTER 30 Evaluation of Prosthetic Valves 383 SABRINA PHILLIPS AND FLETCHER MILLER ■ CHAPTER 31 Three-dimensional Echocardiography in Congenital Heart Diseases 388 GIRISH S. SHIRALI AND IVAN SALGO ■ CHAPTER 32 Stress Echocardiography 396 THOMAS R. KIMBALL ■ CHAPTER 33 Intracardiac and Intraoperative Transesophageal Echocardiography 406 DONALD J. HAGLER ■ CHAPTER 34 Cardiac Magnetic Resonance in Congenital Heart Disease 425 MARK A. FOGEL ■ CHAPTER 35 Aging and Congenital Heart Disease 438 JAMES B. SEWARD ■ CHAPTER 36 Evaluation of the Adult With Transposition After Atrial Switch Operation 442 FRANK CETTA, DAVID J. DRISCOLL, AND BENJAMIN W. EIDEM ■ CHAPTER 37 Tetralogy of Fallot With Pulmonary Regurgitation 450 NASER M. AMMASH ■ CHAPTER 38 Echocardiographic Evaluation of the Functionally Univentricular Heart After Fontan “Operation” 457 SABRINA D. PHILLIPS AND PATRICK W. O’LEARY ■ CHAPTER 39 Eisenmenger Syndrome 472 ROBERT C. LICHTENBERG AND FRANK CETTA ■ CHAPTER 40 Fetal Echocardiography 477 ANITA SZWAST, SHOBHA NATARAJAN, AND JACK RYCHIK Index 487LWW-EIDEM-09-0305-FM.indd ix 9/2/09 3:50:56 PM
  10. 10. LWW-EIDEM-09-0305-FM.indd x 9/2/09 3:50:57 PM