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Dynamic Echocardiography ii Section XIV—Congenital Heart Disease Dynamic Echocardiography Roberto M Lang, MD, FASE, FACC, FAHA, FESC, FRCP Professor of Medicine President, American Society of Echocardiography Director, Noninvasive Cardiac Imaging Laboratories University of Chicago Medical Center Chicago, Illinois Steven A Goldstein, MD, FACC Director, Noninvasive Cardiology Lab Washington Hospital Center Washington, District of Columbia Itzhak Kronzon, MD, FASE, FACC, FAHA, FESC, FACP Professor of Medicine Director, Non Invasive Cardiology New York University Medical Center New York, New York Bijoy K Khandheria, MD, FASE, FACC, FESC, FACP Director, Echocardiography Services Aurora Health Care, Aurora Medical Group Aurora/St Luke Medical Center, Aurora/Sinai Medical Center Milwaukee, Wisconsin 3251 Riverport Lane St Louis, Missouri 63043 DYNAMIC ECHOCARDIOGRAPHY ISBN: 978-1-4377-2262-8 Copyright © 2011 by Saunders, an imprint of Elsevier Inc No part of this publication may be reproduced or transmitted in any form or by any means, electronic or mechanical, including photocopying, recording, or any information storage and retrieval system, without permission in writing from the publisher Details on how to seek permission, further information about the Publisher’s permissions policies and our arrangements with organizations such as the Copyright Clearance Center and the Copyright Licensing Agency, can be found at our website: www.elsevier.com/permissions This book and the individual contributions contained in it are protected under copyright by the Publisher (other than as may be noted herein) Notices Knowledge and best practice in this field are constantly changing As new research and experience broaden our understanding, changes in research methods, professional practices, or medical treatment may become necessary Practitioners and researchers must always rely on their own experience and knowledge in evaluating and using any information, methods, compounds, or experiments described herein In using such information or methods they should be mindful of their own safety and the safety of others, including parties for whom they have a professional responsibility With respect to any drug or pharmaceutical products identified, readers are advised to check the most current information provided (i) on procedures featured or (ii) by the manufacturer of each product to be administered, to verify the recommended dose or formula, the method and duration of administration, and contraindications It is the responsibility of practitioners, relying on their own experience and knowledge of their patients, to make diagnoses, to determine dosages and the best treatment for each individual patient, and to take all appropriate safety precautions To the fullest extent of the law, neither the Publisher nor the author assumes any liability for any injury and/or damage to persons or property as a matter of products liability, negligence or otherwise, or from any use or operation of any methods, products, instructions, or ideas contained in the material herein Library of Congress Cataloging-in-Publication Data Dynamic echocardiography / American Society of Echocardiography ; [edited by] Roberto M Lang.—1st ed    p ; cm   Includes bibliographical references and index   ISBN 978-1-4377-2262-8 (hardcover : alk paper)   1.  Echocardiography.  I.  Lang, Roberto M.  II.  American Society of Echocardiography   [DNLM:  1.  Cardiovascular Diseases—ultrasonography.  2.  Echocardiography—methods WG 141.5.E2 D997 2010]   RC683.5.U5D96 2010   616.1′207543—dc22 2010017586 Vice President and Publisher: Linda Belfus Executive Editor: Natasha Andjelkovic Editorial Assistant: Bradley McIlwain Publishing Services Manager: Patricia Tannian Project Manager: Carrie Stetz Design Direction: Steven Stave Working together to grow libraries in developing countries Printed in the United States of America Last digit is the print number:  9  8  7  6  5  4  3  www.elsevier.com | www.bookaid.org | www.sabre.org Preface For more than a quarter of a century, echocardiography has made unparalleled contributions to clinical cardiology as a major tool for real-time imaging of cardiac dynamics Echocardiography is currently widely used every day in hospitals and clinics around the world for assessing cardiac function while simultaneously providing invaluable noninvasive information for the diagnosis of multiple disease states The American Society of Echocardiography (ASE) is an organization of professionals committed to excellence in cardiovascular ultrasound and its application to patient care through education, advocacy, research, innovation, and service to our members and public ASE’s goal is to be its members’ primary resource for education, knowledge exchange, and professional development This comprehensive textbook on echocardiography constitutes a major step toward the achievement of this goal Dynamic Echocardiography is a comprehensive project several years in the making This text and the companion online library of cases together comprise a state-of-the-art publication on all aspects of clinical echocardiography written by more than 100 medical experts affiliated with ASE The book consists of 111 chapters divided into 14 sections: Native Valvular Heart Disease: Aortic Stenosis/Aortic Regurgitation; Native Valvular Heart Disease: Mitral Stenosis/Mitral Regurgitation; Prosthetic Heart Valve Disease; Interventional/ Intraoperative Echocardiography; Transesophageal Echocardiography; Coronary Artery Disease; Mechanical Complications of Myocardial Infarction; Pericardial Disease and Intracardiac Masses; Myocardial Diseases; Heart Failure Filling Pressures/Diastology; Cardiac Resynchronization Therapy; New Technology; Cases From Around the World; and Congenital Heart Disease Most sections include a commentary chapter written by a leading authority summarizing the current knowledge on each topic as well as a chapter written by a sonographer describing the technical aspects required for optimal data acquisition and display Each of the 111 chapters has a companion online library of didactic slides that include multiple cases Once readers have completed review of the written chapter, we encourage them to review the accompanying slides and case presentations This exercise will allow the reader to visualize dynamic echocardiographic clips of multiple cardiac pathologies We believe that this combined approach is the most effective way of learning clinical echocardiography Our hope is that physicians and cardiac sonographers will use this text and the companion online materials as a reference and self-assessment tool The editors and the authors wish to thank the sonographers with whom we have had the privilege of working throughout the years Without their daily pursuit of quality, hard work, and desire to continuously learn, this project would never have been completed We also especially want to thank each of the section editors: Randolph Martin, Patricia Pellikka, Fausto Pinto, Mani Vannan, Neil Weissman, Malissa Wood, and William Zoghbi for their time and expertise in bringing this product to fruition We owe a great debt to our ASE staff, who has collaborated with us closely in every aspect of this project, including Chelsea Flowers, who helped obtain the required permissions; Hilary Lamb, who assisted us with all aspects of the artwork; and Anita Huffman and Debra Fincham, who assembled the list of contributors In particular, we would like to acknowledge the tireless and invaluable help of Andrea Van Hoever and Robin Wiegerink, who helped us complete this project in a timely and effective manner We would like to also thank Dr Harry Rakowski, who has provided us with practical, positive encouragement and advice We also wish to thank our families for their continuous support while we worked on this project—our wives Lili, Simoy, Ziva, and Priti; our children Daniella, Gabriel, Lauren, Derek, Iris, Rafi, Shira, Vishal, and Trishala; and our grandchildren Ella, Adam, Lucy, and Eli Roberto M Lang, MD, FASE Steven Goldstein, MD Itzhak Kronzon, MD, FASE Bijoy Khandheria, MD, FASE v Foreword It is difficult for contemporary cardiology fellows to imagine a day when echocardiography was not the focal point for patient diagnosis and management, but cardiovascular ultrasound is still a relatively young discipline It has been less than 60 years since Inge Edler and Helmuth Hertz first directed a shipyard reflectoscope toward their own hearts and noted moving echoes on an oscilloscope screen, a development that the normally clairvoyant Paul Dudley White termed “ingenious” but of little clinical value Even by the 1980s, when two-dimensional echocardiography and continuous wave Doppler were well established, one of the factors influencing me to choose an echo fellowship over electrophysiology was that echocardiography was so little regarded clinically that echo fellows were never called in at night or on the weekends! I recall the day in 1988 when this all changed for me I was attending the weekly catheterization laboratory conference at Massachusetts General Hospital, traditionally a setting for pointing out the perceived failings of the echo lab On that fateful day, however, Peter Block, director of the cath lab, announced that in his mind echo was the gold standard for quantification of aortic stenosis, leading to Ned Weyman nearly falling out of his chair! Flash forward to 2010 At the Cleveland Clinic, we now approximately 100,000 cardiovascular ultrasound studies, more than five times the combined total of nuclear, magnetic resonance, and computed tomographic studies The echo lab is the hub of decision making in valvular heart disease, adult and pediatric congenital abnormalities, congestive heart failure, arrhythmia management, aortic and vascular disease, and cardiac ischemia And, in a cruel irony, it is now the echo lab that is far more likely to be called in after hours than electrophysiology! As the utility of echocardiography has expanded, the technical and clinical knowledge base required to apply this technique to its fullest potential has grown exponentially Learning the many nuances of echocardiography must be a lifelong commitment With this goal in mind, the American Society of Echocardiography has published Dynamic Echocar­ diography, a comprehensive text and atlas of echocardiography Conceived and executed by editor in chief Roberto Lang, 2009/2010 President of ASE, and senior editors Steven Goldstein, Itzhak Kronzon, and Bijoy Khandheria, this book provides a comprehensive and practical approach to the basic principles and clinical application of echocardiography This really is two educational products in one First is an expansive book with more than 100 chapters that detail the myriad ways that echocardiography can be used to solve clinical problems Complementing this book is the accompanying online library that provides a wealth of classic examples of the various pathologies likely to be encountered clinically Combined, the book and online library provide the perfect study guide for fellows initially learning echocardiography, those studying for the echocardiography boards, and practicing cardiologists looking for a refresher and update to improve their clinical echo skills In this era of multimodality imaging, many have predicted the decline of echocardiography Those of us who have spent our careers in the field, however, have long marveled at the capacity of echo for reinvention, most obviously in its technical capabilities but even more impressively in its expanded clinical applications By publishing Dynamic Echocardio­ graphy, the American Society of Echocardiography continues its commitment to educational excellence I commend this resource to you with great enthusiasm James D Thomas, MD, FACC, FAHA, FESC Cleveland, Ohio vii Contributors Theodore P Abraham, MD, FASE, FACC Sripal Bangalore, MD, MHA Hari P Chaliki, MD, FASE, FACC Director, Hypertrophic Cardiomyopathy Clinic Division of Cardiology Johns Hopkins University Baltimore, Maryland Division of Cardiology Brigham and Women’s Hospital Harvard Medical School Boston, Massachusetts Assistant Professor of Medicine Division of Cardiovascular Diseases Mayo Clinic Scottsdale, Arizona Harry Acquatella, MD, FASE, FACC, FAHA Manish Bansal, MD, DNB Kwan-Leung Chan, MD, FACC, FRCPC Professor of Medicine Universidad Central de Venezuela, Caracas Department of Echocardiography Centro Medico de Caracas Caracas, Venezuela Consultant Cardiologist Indraprastha Apollo Hospital New Delhi, India Professor of Medicine Division of Cardiology University of Ottawa Heart Institute Ottawa, Ontario, Canada David Adams, RCS, RDCS, FASE Cardiac Sonographer Duke Echocardiography Laboratory Duke University Hospital Durham, North Carolina Deborah A Agler, RCT, RDCS, FASE Coordinator of Education and Training Cardiovascular Imaging Cleveland Clinic Cleveland, Ohio Josef Aichinger, MD Senior Cardiologist Cardiology, Angiology, Intensive Care Elisabethinen Hospital Linz Linz, Austria Bilal Shaukat Ali, MD Fellow in Advanced Cardiac Imaging Division of Cardiology Brigham and Women’s Hospital Boston, Massachusetts Samuel J Asirvatham, MD, FACC, FHRS Consultant, Division of Cardiovascular Diseases and Internal Medicine Division of Pediatric Cardiology Professor of Medicine Mayo Clinic College of Medicine Vice Chair, Cardiovascular Division—Innovations Program Director, EP Fellowship Program Mayo Clinic Rochester, Minnesota David S Bach, MD, FASE Professor Department of Internal Medicine, Division of Cardiovascular Medicine University of Michigan Ann Arbor, Michigan viii Helmut Baumgartner, MD, FACC, FESC Professor of Cardiology Adult Congenital and Valvular Heart Disease Center University Hospital Muenster Muenster, Germany Sonal Chandra, MD Advanced Imaging Fellow Section of Cardiology University of Chicago Chicago, Illinois Jeroen J Bax, MD, PhD Krishnaswamy Chandrasekaran, Professor of Cardiology Department of Cardiology Leiden University Medical Center Leiden, The Netherlands MD, FASE S Michelle Bierig, MPH, RDCS, RDMS, FASE Manager, Core Echocardiography Laboratory St John’s Mercy Heart and Vascular Hospital St Louis, Missouri Gabe B Bleeker, MD, PhD Department of Cardiology Leiden University Medical Center Leiden, The Netherlands Professor of Medicine Mayo Clinic College of Medicine Consultant, Division of Cardiovascular Diseases Mayo Clinic Scottsdale, Arizona Nithima Chaowalit, MD Assistant Professor Division of Cardiology, Department of Medicine Siriraj Hospital Mahidol University Bangkok, Thailand Farooq A Chaudhry, MD, FASE, FACC, William B Borden, MD FAHA, FACP Assistant Professor of Medicine Cardiovascular Disease Weill Cornell Medical College New York, New York Associate Professor of Medicine Columbia University College of Physicians and Surgeons Associate Chief of Cardiology Director of Echocardiography St Luke’s Roosevelt Hospital Center New York, New York Darryl J Burstow, MBBS, FRACP Senior Staff Cardiologist Associate Professor of Medicine Department of Cardiology The Prince Charles Hospital Brisbane, Queensland, Australia Scipione Carerj, MD Professor of Cardiology Department of Medicine and Pharmacology University of Messina Messina, Italy Namsik Chung, MD, PhD, FASE, FAHA Dean Yonsei University College of Medicine Professor of Cardiology Yonsei University College of Medicine Seoul, Korea Patrick D Coon, RDCS, FASE Program Director, Echocardiography Division of Cardiology, Department of Pediatrics The Cardiac Center at The Children’s Hospital of Philadelphia Philadelphia, Pennsylvania Contributors ix Ronan J Curtin, MD, MSc Mario J Garcia, MD, FACC, FACP Judy W Hung, MD, FASE Consultant Cardiologist Department of Cardiology Cork University Hospital Cork, Ireland Chief, Division of Cardiology Montefiore Medical Center Albert Einstein College of Medicine Bronx, New York Jeanne M DeCara, MD, FASE Eulogio García-Fernández, MD Associate Director, Echocardiography Assistant Professor of Medicine Harvard Medical School Cardiology Division, Department of Medicine Massachusetts General Hospital Boston, Massachusetts Associate Professor of Medicine University of Chicago Medical Center Chicago, Illinois Cardiology Department Hospital General Universitario “Gregorio Marón” Madrid, Spain Geneviève Derumeaux, MD, PhD, FESC Professor of Physiology Explorations Fonctionnelles Cardiovasculaires Lyon University Lyon, France Veronica Lea J Dimaano, MD Senior Research Fellow Division of Cardiology Johns Hopkins University, School of Medicine Baltimore, Maryland Jean G Dumesnil, MD, FACC, FRCPC Professor of Medicine Laval University Cardiologist, Quebec Heart and Lung Institute, Laval University Quebec City, Quebec, Canada Miguel Angel García-Fernandez, James G Jollis, MD, FACC Cardiology Department Hospital General Universitario “Gregorio Marón” Madrid, Spain Professor of Medicine and Radiology Duke University Durham, North Carolina José Antonio García-Robles, MD Cardiology Department Hospital General Universitario “Gregorio Marón” Madrid, Spain Steven A Goldstein, MD, FACC Director, Noninvasive Cardiology Lab Washington Hospital Center Washington, District of Columbia José Luis Zamorano Gomez, Senior Cardiologist Cardiology, Angiology, Intensive Care Elisabethinen Hospital Linz Linz, Austria MD, PhD, FESC West-German Heart Center University Duisburg-Essen Essen, Germany Raimund Erbel, MD, FACC, FAHA, FESC Professor of Medicine/Cardiology European Cardiologist Department of Cardiology West-German Heart Center University Duisburg-Essen Essen, Germany Rebecca B Fountain, RN, BSN Section of Internal Medicine and Cardiovascular Diseases Mayo Clinic Rochester, Minnesota Andreas Franke, MD, FESC Department of Cardiovascular Medicine Okayama University Okayama, Japan MD, PhD Christian Ebner, MD Holger Eggebrecht, MD Hiroshi Ito, MD, PhD Professor of Medicine Universidad Complutense de Madrid Director, Cardiovascular Institute University Clinic San Carlos Madrid, Spain José Juan Gómez de Diego, MD Cardiology Staff Laboratorio de Imagen Cardíaca Hospital Universitario La Paz Madrid, Spain Jose Luis Gutierrez-Bernal, MD Hospital Español Mexico City, Mexico Jong-Won Ha, MD, PhD, FESC Cardiology Division Professor of Medicine Yonsei University College of Medicine Seoul, South Korea David R Holmes, Jr., MD, FACC Medical Clinic I RWTH University Hospital Aachen, Germany Consultant, Cardiovascular Diseases Professor of Medicine Mayo Clinic Rochester, Minnesota William K Freeman, MD, FACC Kenneth Horton, RCS, RCIS, FASE Associate Professor of Medicine Division of Cardiovascular Diseases Mayo Clinic Rochester, Minnesota Echo/Vascular Research Coordinator Intermountain Healthcare Salt Lake City, Utah Christine Attenhofer Jost, MD, FESC Professor of Cardiology Cardiovascular Center Zurich Zurich, Switzerland Gudrun Kabicher, MD Senior Cardiologist Cardiology, Angiology, Intensive Care Elisabethinen Hospital Linz Linz, Austria Sanjiv Kaul, MD, FASE, FACC Professor and Division Head, Cardiovascular Medicine Oregon Health & Sciences University Portland, Oregon Bijoy K Khandheria, MD, FASE, FACC, FACP, FESC Director, Echocardiography Services Aurora Health Care, Aurora Medical Group Aurora/St Luke Medical Center, Aurora/Sinai Medical Center Milwaukee, Wisconsin James N Kirkpatrick, MD, FASE, FACC Assistant Professor of Medicine Division of Cardiovascular Medicine University of Pennsylvania Philadelphia, Pennsylvania Allan L Klein, MD, FASE, FACC, FAHA, FRCP(C) Director of Cardiovascular Imaging Research Director of the Center for the Diagnosis and Treatment of Pericardial Diseases Cardiovascular Medicine Cleveland Clinic Cleveland, Ohio Smadar Kort, MD, FASE, FACC Associate Professor of Medicine Director, Cardiovascular Imaging Division of Cardiology Stony Brook University Medical Center Stony Brook, New York x Contributors Itzhak Kronzon, MD, FASE, FACC, FAHA, Boris S Lowe, BHB, MB ChB, FRACP Hector I Michelena, MD, FACC FESC, FACP Consultant Cardiologist Green Lane Cardiovascular Service Auckland City Hospital Auckland, New Zealand Assistant Professor of Medicine Mayo Clinic College of Medicine Consultant, Division of Cardiovascular Diseases Mayo Clinic Rochester, Minnesota Professor of Medicine Director, Non Invasive Cardiology New York University Medical Center New York, New York Karla M Kurrelmeyer, MD, FASE Assistant Professor of Medicine Weill Cornell Medical College Department of Cardiology Methodist DeBakey Heart & Vascular Center Houston, Texas Roberto M Lang, MD, FASE, FACC, FAHA, Joan L Lusk, RN, RDCS, ACS, FASE Registered Adult and Pediatric Cardiac Sonographer Adult Congenital Heart Disease Clinic Advanced Clinical/Research Sonographer Mayo Clinic Cardiac Ultrasound Imaging and Hemodynamic Laboratory Mayo Clinic Scottsdale, Arizona FESC, FRCP Professor of Medicine President, American Society of Echocardiography Director, Noninvasive Cardiac Imaging Laboratories University of Chicago Medical Center Chicago, Illinois Pui Lee, MBChB Advanced Fellow in Echocardiography Echocardiography Laboratory Mayo Clinic Rochester, Minnesota Vera Lennie, MD, FESC Cardiologist Department of Cardiac Imaging Hospital Carlos III Madrid, Spain Steven J Lester, MD, FASE, FACC, FRCPC Consultant, Department of Medicine, Division of Cardiology Associate Professor of Medicine, College of Medicine Director of Echocardiography Mayo Clinic Scottsdale, Arizona Dominic Y Leung, MBBS, PhD, FACC, FRCP(Edin), FRACP, FHKCP, FCSANZ Professor of Cardiology, Department of Cardiology Liverpool Hospital, University of New South Wales Sydney, New South Wales, Australia Jonathan R Lindner, MD, FASE Professor and Associate Chief for Education Cardiovascular Division Oregon Health & Science University Portland, Oregon Joseph A Lodato, MD Section of Cardiology Department of Medicine University of Chicago Medical Center Chicago, Illinois Joseph F Malouf, MD Professor of Medicine Mayo Clinic College of Medicine Department of Internal Medicine Mayo Clinic Rochester, Minnesota Randolph P Martin, MD, FASE, FACC, FESC Medical Director, Cardiovascular Imaging Piedmont Hospital Chief, Structural & Valvular Heart Disease Piedmont Heart Institute Professor of Medicine, Emeritus Emory University School of Medicine Atlanta, Georgia Thomas H Marwick, MBBS, PhD Victor Mor-Avi, PhD, FASE Professor Section of Cardiology, Department of Medicine Director of Cardiac Imaging Research University of Chicago Chicago, Illinois Sherif F Nagueh, MD, FASE Professor of Medicine Weill Cornell Medical College Associate Director of Echocardiography Laboratory Methodist DeBakey Heart and Vascular Center Houston, Texas Hans Joachim Nesser, MD, FASE, FACC, FESC Professor of Medicine Head, Department of Cardiology, Angiology, Intensive Care Hospital Vice Director Cardiology, Angiology, Intensive Care Elisabethinen Hospital Linz Linz, Austria Johannes Niel, MD Professor of Medicine Institution University of Queensland Brisbane, Queensland, Australia Senior Cardiologist Cardiology, Angiology, Intensive Care Elisabethinen Hospital Linz Linz, Austria Gerald Maurer, MD, FACC, FESC Steve R Ommen, MD Professor of Medicine Director, Division of Cardiology Chair, Department of Medicine II Medical University of Vienna Vienna, Austria Consultant Vice-Chair for Education Director, Hypertrophic Cardiomyopathy Clinic Division of Cardiovascular Diseases Professor of Medicine Mayo Clinic Rochester, Minnesota Patrick M McCarthy, MD, FACC Chief of Cardiac Surgery Division, Director of the Bluhm Cardiovascular Institute, and Heller-Sacks Professor of Surgery Division of Cardiac Surgery Northwestern University/Northwestern Memorial Hospital Chicago, Illinois Alan S Pearlman, MD, FASE, FACC, FAHA Professor of Medicine Division of Cardiology University of Washington School of Medicine Seattle, Washington Patricia A Pellikka, MD, FASE, FACC, Ivàn Melgarejo, MD FAHA, FACP Cardiologist, Echocardiographer Department of Noninvasive Cardiology Fundaciòn A Shaio Professor of Cardiology Universidad del Rosario Bogotà, Colombia Professor of Medicine Mayo Clinic College of Medicine Co-Director, Echocardiography Laboratory Division of Cardiovascular Diseases and Internal Medicine Mayo Clinic Rochester, Minnesota Contributors xi Esther Pérez-David, MD, PhD Ricardo E Ronderos, MD, PhD Masaaki Takeuchi, MD, PhD, FASE Cardiology Department Hospital General Universitario “Gregorio Marón” Madrid, Spain Associate Professor of Cardiology Director, Instituto de Cardiologia La Plata Chief, Cardiovascular Imaging Department Instituto Cardiovascular de Buenos Aires Universidad Nacional de La Plata La Plata, Buenos Aires, Argentina Associate Professor Second Department of Internal Medicine University of Occupational and Environmental Health School of Medicine Kitakyushu, Japan Muhamed Saric, MD, PhD, FASE, FACC Hélène Thibault, MD, PhD Associate Professor of Medicine Noninvasive Cardiology New York University New York, New York Docteur of Cardiology Echocardiography Laboratory Hôpital Louis Pradel Lyon, France Partho P Sengupta, MD, DM Wolfgang Tkalec, MD Assistant Professor of Medicine Mayo Clinic College of Medicine Cardiovascular Division Mayo Clinic Scottsdale, Arizona Senior Cardiologist Cardiology, Angiology, Intensive Care Elisabethinen Hospital Linz Linz, Austria Philippe Pibarot, DVM, PhD, FACC, FAHA Professor of Medicine Department of Medicine Laval University Québec City, Quebec, Canada Michael H Picard, MD, FASE, FACC, FAHA Director, Echocardiography Massachusetts General Hospital Associate Professor Harvard Medical School Boston, Massachusetts Fausto J Pinto, MD, PhD, FASE, FACC, FESC, FSCAI Professor of Cardiology/Medicine Department of Cardiology Lisbon University Medical School Lisbon, Portugal Heidi Pollard, RDCS Cardiac Sonographer Department of Cardiology University of Chicago Medical Centers Chicago, Illinois Tamar S Polonsky, MD Post Doctoral Fellow Cardiovascular Epidemiology and Prevention Northwestern University Chicago, Illinois Thomas R Porter, MD, FASE Professor of Cardiology Courtesy Professor of Radiology and Pediatric Cardiology Department of Internal Medicine–Division of Cardiology University of Nebraska Medical Center Omaha, Nebraska Brian D Powell, MD Assistant Professor of Medicine Cardiovascular Division Mayo Clinic Rochester, Minnesota Jose E Riarte, MD Staff, Cardiovascular Ultrasound Service Cardiac Imaging Department Instituto Cardiovascular de Buenos Aires Ciudad de Buenos Aires, Argentina Vera H Rigolin, MD, FASE, FACC, FAHA Associate Professor of Medicine Northwestern University Feinberg School of Medicine Medical Director, Echocardiography Laboratory Northwestern Memorial Hospital Chicago, Illinois Dipak P Shah, MD Cardiology Fellow Section of Cardiology University of Chicago Chicago, Illinois Paul A Tunick, MD Professor, Department of Medicine Noninvasive Cardiology Laboratory New York University Medical Center New York, New York Matt M Umland, RDCS, FASE, RT(R), Stanton K Shernan, MD, FASE, FAHA (CT), (QM) Associate Professor of Anesthesia Director of Cardiac Anesthesia Department of Anesthesiology, Perioperative and Pain Medicine Brigham and Women’s Hospital Harvard Medical School Boston, Massachusetts Echocardiography Quality Coordinator Advanced Hemodynamic and Cardiovascular Laboratory Aurora Medical Group Advanced Cardiovascular Services Milwaukee, Wisconsin Kirk T Spencer, MD, FASE Associate Professor of Medicine University of Chicago Chicago, Illinois Monvadi B Srichai, MD Assistant Professor Department of Radiology and Medicine, Cardiology Division New York University School of Medicine New York, New York Kathleen Stergiopoulos, MD, PhD, Mani A Vannan, MBBS, FACC Professor of Clinical Internal Medicine Joseph M Ryan Chair in Cardiovascular Medicine Director, Cardiovascular Imaging The Ohio State University Columbus, Ohio Philippe Vignon, MD, PhD Professor of Critical Care Medicine Medical-Surgical ICU and Clinical Investigation Center Teaching Hospital of Limoges Limoges, France FASE, FACC Assistant Professor of Medicine Director, Inpatient Cardiology Consultation Stony Brook University School of Medicine SUNY Health Sciences Center Stony Brook, New York G Monet Strachan, RDCS, FASE Supervisor, Echocardiography Lab University of California San Diego Medical Center San Diego, California Lissa Sugeng, MD, MPH Assistant Professor of Clinical Medicine Non-Invasive Cardiovascular Imaging Lab University of Chicago Medical Center Chicago, Illinois Hector R Villarraga, MD, FASE, FACC Assistant Professor of Medicine Mayo Clinic College of Medicine Division of Cardiovascular Diseases and Internal Medicine Mayo Clinic Rochester, Minnesota R Parker Ward, MD, FASE, FACC Associate Professor of Medicine Non-Invasive Imaging Laboratories Section of Cardiology University of Chicago Medical Center Chicago, Illinois xii Contributors Nozomi Watanabe, MD, PhD, FACC Malissa J Wood, MD, FASE, FACC Qiong Zhao, MD, PhD, FASE Department of Cardiology Kawasaki Medical School Kurashiki, Japan Co-director MGH Heart Center Corrigan Women’s Heart Health Program Assistant Professor of Medicine Harvard Medical School Departments of Medicine/Cardiology Massachusetts General Hospital Boston, Massachusetts Assistant Professor of Medicine Cardiology Division, Department of Medicine Northwestern University, Feinberg School of Medicine Chicago, Illinois Kevin Wei, MD Associate Professor of Medicine Cardiovascular Division Oregon Health & Science University Portland, Oregon Neil J Weissman, MD, FASE Professor of Medicine, Georgetown University President, MedStar Health Research Institute at Washington Hospital Center Washington, District of Columbia Siegmund Winter, MD Senior Cardiologist Cardiology, Angiology, Intensive Care Elisabethinen Hospital Linz Linz, Austria Feng Xie, MD Associate Professor of Medicine Division of Cardiology University of Nebraska Medical Center Omaha, Nebraska Hyun Suk Yang, MD, PhD Division of Cardiovascular Diseases Mayo Clinic Scottsdale, Arizona Danita M Yoerger Sanborn, MD, FASE, MMSc Assistant Physician, Instructor in Medicine Cardiology Division Massachusetts General Hospital Harvard Medical School Boston, Massachusetts Concetta Zito, MD Cardiology Assistant Unit of Intensive and Invasive Heart Care Department of Medicine and Pharmacology University of Messina Messina, Italy William A Zoghbi, MD, FASE, FACC, FAHA William L Winters Endowed Chair in CV Imaging Professor of Medicine Weill-Cornell Medical College Director, Cardiovascular Imaging Institute The Methodist DeBakey Heart & Vascular Center Houston, Texas 450 Section XIV—Congenital Heart Disease progressive dyspnea on exertion, chronic hypoxemia with attendant cyanosis, clubbing and secondary erythrocytosis, hyperviscosity syndrome, and bleeding diathesis may develop, and patients are at risk for arrhythmias, sudden death, paradoxical embolism, and brain abscess Because the magnitude of right-to-left shunting is determined by the ratio of PVR to SVR, any decrease in SVR (such as that caused by systemic vasodilators) will increase the degree of reverse shunting and consequently lead to more cyanosis and breathlessness As Eisenmenger syndrome advances, the initial murmurs of the underlying shunt lesion (VSD, PDA, or ASD) progressively diminish and may disappear altogether With the development of pulmonary hypertension and RV hypertrophy, the pulmonic component of the second heart sound becomes loud and right parasternal heave appears Lung fields are clear on auscultation Electrocardiogram In persons with advanced Eisenmenger’s syndrome, atrial arrhythmias and signs of right atrial enlargement and RV hypertrophy are common findings Chest Radiography Radiographic hallmarks of Eisenmenger’s syndrome include marked enlargement of the right heart and proximal pulmonary arteries, rapid tapering of the peripheral pulmonary arterial tree (“pruning”), and oligemic lung fields.3 SVR, surgery is not an option In addition to baseline measurements, PVR also should be calculated after administration of pulmonary vasodilators (such as inhaled nitric oxide or intravenous epoprostenol) to assess for potential reversibility of pulmonary arterial hypertension Treatment Prognosis of patients who are not candidates for shunt closure is poor Their 5-year mortality rate is 12.5%, and their life span is approximately 20 years shorter than that of the general population For such patients, heart-lung transplant may be considered Heart-lung transplant appears to offer a survival benefit for patients with Eisenmenger’s syndrome resulting from VSD.11 Pulmonary vasodilators such as bosentan have been shown to improve the hemodynamics and exercise capacity of patients with Eisenmenger syndrome without worsening peripheral levels of oxygen saturation.12 However, they have not been shown to alter survival rates All patients with Eisenmenger’s syndrome should receive antibiotic prophylaxis and should avoid intravascular volume depletion and systemic vasodilators Phlebotomy with isovolumic fluid replacement should be performed only when erythrocytosis is severe (hemoglobin concentration >17 g/dL) and hyperviscosity neurologic symptoms are present Frequent phlebotomies may lead to iron deficiency and worsening neurologic symptoms as a result of rigidity of iron-deficient erythrocytes References Echocardiography The following three elements should be characterized on any echocardiographic examination in patients with Eisenmenger’s syndrome: The location and size of the shunt lesion (e.g., VSD, PDA, and ASD), as described in appropriate sections of this text The magnitude and directionality of the shunt flow It can be difficult to visualize by Doppler imaging because RV and LV pressure may already have equalized, resulting in minimal and/or bidirectional low-velocity flow However, the very difficulty in visualizing such a shunt elucidates Eisenmenger physiology and is an important diagnostic sign of Eisenmenger syndrome An estimate of pulmonary arterial pressures and their impact on the size and function of the right heart Other Imaging Techniques Cardiac catheterization is essential for determining PVR because in patients in whom PVR is more than two thirds of Goor DA, Lillehie W, Rees R: Isolated ventricular septal defect: development basis for various types and presentation of classification Chest 58:468-482, 1970 Brickner ME, Hillis D, Lange RA: Congenital heart disease: second of two parts N Engl J Med 342:334-342, 2000 Brickner ME, Hillis D, Lange RA: Congenital heart disease: first of two parts N Engl J Med 342:256-263, 2000 Amash NM, Warnes CA: Ventricular septal defects in adults Ann Intern Med 135:812-824, 2001 Craig B: Atrioventricular septal defect: from fetus to adult Heart 92:18791885, 2006 Arora R, Trehan V, Kumar A, et al: Transcatheter closure of congenital ventricular septal defects: experience with various devices J Intervent Cardiol 16:83-91, 2003 Wilson W, Taubert KA, Gewitz M, et al: Prevention of infective endocarditis: guidelines from the American Heart Association Circulation 116:1736-1754, 2007 Wood P: The Eisenmenger syndrome or pulmonary hypertension with reversed central shunt Br Med J 2:701-709, 755-762, 1958 Eisenmenger V: Congential ventricular septal defect of the heart [in German] Zeitschrift für Klinische Medizin 32(suppl):1-28, 1897 10 Diller GP, Dimopoulos K, Broberg CS, et al: Presentation, survival prospects, and predictors of death in Eisenmenger syndrome: a combined retrospective and case-control study Eur Heart J 27:1737-1742, 2006 11 Waddell TK, Bennett L, Kennedy R, et al: Heart-lung or lung transplantation for Eisenmenger syndrome J Heart Lung Transplant 21:731-737, 2002 12 Galiè N, Beghetti M, Gatzoulis MA, et al: Bosentan therapy in patients with Eisenmenger syndrome: a multicenter, double-blind, randomized, placebo-controlled study Circulation 114:48-54, 2006 Chapter 108 XIV Patent Ductus Arteriosus Muhamed Saric, MD, PhD, FASE, and Itzhak Kronzon, MD, FASE Definition Patent ductus arteriosus (PDA) results from the failure of physiologic closure of ductus arteriosus past the first year of life Ductus arteriosus is an essential element of the normal fetal circulation; it directs the blood away from the highresistance pulmonic circulation of the collapsed lungs to the low-resistance systemic circulation (physiologic right-to-left shunt) Embryologically, ductus arteriosus is a continuation of the proximal left pulmonary artery; ductus arteriosus develops from the distal and the left pulmonary artery from the proximal end of the left sixth branchial arch In utero, prostaglandin E2 (PGE2)—believed to be produced both locally and by the placenta—keeps the ductus arteriosus open.1 Immediately after delivery, the shunt direction reverses (transient physiologic left-to-right shunt) The high oxygen content of ductal blood activates an oxygen-sensitive potassium channel that leads to contraction of the ductal muscular layers and cessation of flow.2 By the end of the neonatal period, scarring completely obliterates ductus arteriosus in the vast majority of infants However, continued patency of ductus arteriosus may be life-saving in so-called PDA-dependent forms of congenital heart disease such as pulmonic atresia and congenitally uncorrected transposition of great arteries Epidemiology Isolated PDA is classified as a moderate form of congenital heart disease (see Table 105.1) Maternal exposure to rubella virus, prematurity, and living at high altitudes are known risk factors for PDA The incidence of PDA is estimated at approximately per 10,000 life births in term infants born near sea level and is similar to that of atrial septal defect (see Table 105.2) The rate more than doubles in patients living at high altitudes.3 It reaches a staggering 60% of all births in infants whose gestation lasted 8 mm/m2) on two-dimensional imaging and an unusual apical origin of the tricuspid regurgitant jet often are the first clues to the diagnosis of Ebstein’s anomaly (Fig 111.2) Paradoxic movement of the basal interventricular septum phasic with right atrial filling and emptying results from atrialization of the inlet portion of the RV Other Imaging Techniques On cardiac catheterization, Ebstein’s anomaly is suspected when a catheter placed in the right atrium records atrial pressures and ventricular electrograms Magnetic resonance imaging is useful for evaluation of RV size and function 464 Section XIV—Congenital Heart Disease Pregnancy References Pregnancy risk is dependent on the severity of Ebstein’s anomaly On one end of the spectrum are women with mild cases of this anomaly who face a low risk On the other end are women with severe cyanosis in whom pregnancy is contraindicated Ebstein W: Concerning a very rare case of insufficiency of the tricuspid valve caused by a congenital malformation [in German] Arch Anat Physiol 238-255, 1866 Hagler DJ: Echocardiographic assessment of Ebstein’s anomaly Prog Pediatr Cardiol 2:28-37, 1993 Attenhofer Jost CH, Connolly HM, et al: Ebstein’s anomaly Circulation 115:277-285, 2007 Marcu CB, Donohue TJ: A young man with palpitations and Ebstein’s anomaly of the tricuspid valve CMAJ 172(12):1553-1554, 2005 4a Hoffman JIE, Kaplan S: The incidence of congenital heart disease J Am Coll Cardiol 39:1890-1900, 2002 Cohen LS, Friedman JM, Jefferson JW, et al: A reevaluation of risk of in utero exposure to lithium JAMA 271:146-150, 1994 Fontana ME, Wooley CF, Goodwin RS, et al: Sail sound in Ebstein’s anomaly of the tricuspid valve Circulation 46:155-164, 1972 Brickner ME, Hillis D, Lange RA: Congenital heart disease: second of two parts N Engl J Med 342:334-342, 2000 Treatment Mild cases of Ebstein’s anomaly can be followed medically Severe cases should be referred to specialized tertiary centers for tricuspid valve reconstructive surgery or tricuspid valve replacement ... clinical echocardiography Our hope is that physicians and cardiac sonographers will use this text and the companion online materials as a reference and self-assessment tool The editors and the... ASE, and senior editors Steven Goldstein, Itzhak Kronzon, and Bijoy Khandheria, this book provides a comprehensive and practical approach to the basic principles and clinical application of echocardiography. .. project—our wives Lili, Simoy, Ziva, and Priti; our children Daniella, Gabriel, Lauren, Derek, Iris, Rafi, Shira, Vishal, and Trishala; and our grandchildren Ella, Adam, Lucy, and Eli Roberto M Lang, MD,