CASES IN ADULT CONGENITAL HEART DISEASE Michael A. Gatzoulis, MD, PhD, FACC, FESC Professor of Cardiology and Congenital Heart Disease Consultant Cardiologist Head, Adult Congenital Heart Centre and Centre for Pulmonary Hypertension Royal Brompton Hospital National Heart and Lung Institute, Imperial College London, United Kingdom Gary D. Webb, MD, FRCPC, FACC Professor of Medicine Director of the Philadelphia Adult Congenital Heart Center University of Pennsylvania School of Medicine The Hospital of the University of Pennsylvania Philadelphia, Pennsylvania Craig S. Broberg, MD Assistant Professor School of Medicine Director, Adult Congenital Heart Disease Program Oregon Health & Science University Portland, Oregon Hideki Uemura, MD Consultant Cardiac Surgeon Royal Brompton Hospital London, United Kingdom 1600 John F. Kennedy Boulevard Suite 1800 Philadelphia, PA 19103-2899 CASES IN ADULT CONGENITAL HEART DISEASE ISBN: 978-0-443-06712-9 Copyright © 2010 by Churchill Livingstone, an imprint of Elsevier Inc. All rights reserved. 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. Permissions may be sought directly from Elsevier’s Rights Department: phone: (+1) 215 239 3804 (U.S.) or (+441865 84380 (UK); fax: (+441865 853333; e-mail: [email protected]. You may also complete your request on-line via the Elsevier (U.S.) website at http://www.elsevier.com/permissions. Notice Knowledge and best practice in this field are constantly changing. As new research and experience broaden our knowledge, changes in practice, treatment and drug therapy may become necessary or appropriate. 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 the practitioner, relying on their own experience and knowledge of the patient, 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 Editors assume any liability for any injury and/or damage to persons or property arising out or related to any use of the material contained in this book. The Publisher Library of Congress Cataloging-in-Publication Data Cases in adult congenital heart disease : atlas with DVD / [edited by] Michael A. Gatzoulis … [et al.]. – 1st ed. p. ; cm. Includes bibliographical references. ISBN 978-0-443-06712-9 1. Congenital heart disease–Case studies–Atlases. I. Gatzoulis, Michael A. II. Title. [DNLM: 1. Heart Defects, Congenital–Atlases. 2. Heart Defects, Congenital–Case Reports. 3. Adult. WG 17 C338 2009] RC687.C375 2009 616.1’2043–dc22 2009011148 Executive Publisher: Natasha Andjelkovic Developmental Editor: Agnes H. Byrne Design Direction: Steven Stave Working together to grow libraries in developing countries Printed in China www.elsevier.com | www.bookaid.org | www.sabre.org Last digit is the print number: 9 8 7 6 5 4 3 2 1 To my late father Athanasios Konstantinos Gatzoulis (1921–2008). MAG To the patients I have had the privilege of serving in Toronto and Philadelphia, to the excellent colleagues who have helped me care for them, and to all the ACHD patients whose lives we value. GDW To patients with congenital heart disease throughout the world, who have done the real work represented herein. CSB To all the patients for whom I have had the privilege of providing care and to all my colleagues and mentors. HU Contributors Dominic J. Abrams, MD, MRCP Lee N. Benson, MD Consultant Cardiologist, St. Bartholomew’s and Great Professor of Pediatrics, University of Toronto School of Ormond Street Hospitals, London, United Kingdom Medicine; Director, Cardiac Diagnostic and Interventional Catheter Ablation for Atrial Arrhythmia Unit, The Hospital for Sick Children, Toronto, Ontario, Canada William Alazawi, MA (Cantab), MB BChir, PhD, Catheter Intervention for Baffle Leak or Venous Obstruction MRCP Academic Specialist Registrar, Barts and The London School Philipp Bonhoeffer, MD of Medicine & Dentistry, Queen Mary University of London, Professor of Cardiology, Institute of Child Health; Chief London, United Kingdom of Cardiology, Director of the Cardiac Catheterisation Syncope in a Patient with Noonan Syndrome Laboratory, Great Ormond Street Hospital for Children, London, United Kingdom Dimitrios Alexopoulos, MD Catheter Implantation of Stented Pulmonary Valve Professor and Director of Cardiology, Patras University Hospital, Rion, Patras, Greece Beatriz Bouzas, MD Dyspnea in a Patient with a Loud Murmur Consultant, Cardiology Department, Complejo Hospitalario Universitario Juan Canalejo, Coruña, Spain Khalid Alnajashi, MD Pregnancy and Fetal Death in a Patient with Ebstein Anomaly; Division of Pediatric Cardiology, Prince Sultan Cardiac Congenital Pulmonary Stenosis Turned to Pulmonary Center, Riyadh, Saudi Arabia Regurgitation Late Outcome Following Systemic Tricuspid Valve Replacement William Bradlow, BM, BS Naser M. Ammash, MD Cardiovascular Magnetic Resonance Unit, Royal Brompton Associate Professor of Medicine, Mayo Medical School; Hospital, London, United Kingdom Consultant, Division of Cardiovascular Diseases, Department The Role of Pulmonary Vasodilators in Pulmonary Hypertension of Internal Medicine, Mayo Clinic, Rochester, Minnesota Subaortic Stenosis: Indications for Surgery Craig S. Broberg, MD Assistant Professor, School of Medicine; Director, Adult Kai Andersen, MD, PhD Congenital Heart Disease Program, Oregon Health & Science Consultant Cardiologist, Rikshospitalet University Hospital, University, Portland, Oregon Oslo, Norway Straddling of the Tricuspid Valve: Long-Term Outcome; Long-Term The Criss-Cross Heart Follow-Up of Atrioventricular Septal Defect; Congenital Pulmonary Stenosis Turned to Pulmonary Regurgitation; Pulmonary Atresia Panagiotis D. Arvanitis, MD with Intact Ventricular Septum; Late Complications after the Honorary Clinical/Research Fellow, Adult Congenital Heart Arterial Switch Operation; Palliative Mustard for Transposition Programme, Royal Brompton Hospital, London, United and Ventricular Septal Defect; The Criss-Cross Heart; Long-Term Kingdom Survival in Eisenmenger Syndrome; Iron Deficiency in Cyanotic Acute Presentation with Cerebral Hemorrhage Heart Disease; Pulmonary Artery Thrombosis and Recurrent Hemoptyses in Eisenmenger Syndrome; Contraception Counseling Sonya V. Babu-Narayan, MBBS, MRCP Honorary Clinical Research Fellow, National Heart and Lung Qi-Ling Cao, MD Institute, Imperial College London; Specialist Registrar in Associate Professor, Department of Cardiology, Rush Adult Cardiology and Adult Congenital Heart Disease, Royal Medical College, Rush Center for Congenital and Structural Brompton Hospital, London, United Kingdom Heart Disease, Rush University Medical Center, Chicago, Timing of Pulmonary Valve Replacement; Management Illinois of Tachyarrhythmia in Tetralogy: Ablation versus Catheter Closure of Ventricular Septal Defects Surgery; Consideration for Automatic Implantable Cardioverter- Defibrillator in Tetralogy Pedro A. Catarino, MD Department of Cardiothoracic Surgery, Royal Brompton Elisabeth Bédard, MD Hospital, London, United Kingdom Adult Congenital Heart Centre, Royal Brompton Hospital; Successful Tricuspid Valve Repair of Ebstein Anomaly Imperial College of Science and Medicine, London, United Kingdom Aikaterini Chamaidi, MD Right Heart Enlargement of Uncertain Cause (Sinus Venosus Consultant Cardiologist, University Hospital Larissa, Larissa Atrial Septal Defect); Left Atrioventricular Valve Regurgitation: Medical School, Larissa, Greece Criteria for Intervention; Late Complications after the Arterial Long-Term Outcome after Rastelli Repair Switch Operation; Intracardiac Thrombus in the Fontan Circulation; Contraception Counseling Cases in Adult Congenital Heart Disease vii Joseph Y.S. Chan, MRCP Richard M. Donner, MD s Associate Consultant, Division of Cardiology, Department of Clinical Professor of Pediatrics, University of Pennsylvania or ut Medicine and Therapeutics, Prince of Wales Hospital, Hong School of Medicine; Senior Cardiologist, The Children’s b ontri KTroanngscatheter Options for Atrial Arrhythmia after the Mustard HReocsoparictatal toiofn P: hCirlaitdereilap fhoria I,n Ptehrivlaendteiolpnh; iCay, aPneonsinss iynl vEabnstieain C Procedure Anomaly and Catheter Closure of Atrial Septal Defect Jonathan B. Choy, MD, FRCPC, FACC Nigel E. Drury, BM (Hons), MRCS University of Alberta; Director, Adult Echocardiography Fellow in Congenital Cardiology, Wessex Cardiothoracic Laboratory, Associate Clinical Professor of Medicine, Centre, Southampton General Hospital, Southampton, University of Alberta Hospital, Edmonton, Alberta, Canada United Kingdom Anomalous Left Coronary Artery from the Pulmonary Artery Right Ventricular Outflow Obstruction in a Pregnant Woman Louise Coats, PhD, MRCP Mark J. Earley, MD, MRCP Clinical Research Fellow, Institute of Child Health; Clinical Consultant Cardiologist, St. Bartholomew’s Hospital, Research Fellow, Great Ormond Street Hospital for Children, London, United Kingdom London, United Kingdom Catheter Ablation for Atrial Arrhythmia Catheter Implantation of Stented Pulmonary Valve Michael A. Gatzoulis, MD, PhD, FACC, FESC Andrew Crean, BM, MRCP, MSc, FRCR, MPhil Professor of Cardiology and Congenital Heart Disease, Assistant Professor, University of Toronto; Staff Cardiologist Consultant Cardiologist; Head, Adult Congenital Heart and Radiologist, Toronto Congenital Cardiac Centre for Centre and Centre for Pulmonary Hypertension, Royal Adults, Toronto General Hospital, Toronto, Ontario, Canada Brompton Hospital, National Heart and Lung Institute, Cor Triatriatum; Recoarctation: Criteria for Intervention Imperial College London, United Kingdom Aortic Stenosis and Endocarditis during Pregnancy; Extraanatomic Piers E. F. Daubeney, MA, DM, MRCP, MRCPCH Bypass Graft Repair of Coarctation; Transcatheter Options for Honorary Senior Lecturer, National Heart and Lung Atrial Arrhythmia after the Mustard Procedure Institute, Imperial College London; Consultant Paediatric and Fetal Cardiologist, Royal Brompton Hospital, London, Deborah R. Gersony, MD United Kingdom Assistant Professor of Clinical Medicine, Department of Pulmonary Atresia with Intact Ventricular Septum Medicine and Pediatrics, Columbia University Medical Center, New York, New York Periklis A. Davlouros, MD Pregnancy and the Systemic Right Ventricle Senior Lecturer in Cardiology, Patras University Hospital, Rion, Patras, Greece Marc Gewillig, MD, PhD Dyspnea in a Patient with a Loud Murmur Professor of Pediatric Cardiology, University Leuven, Leuven, Belgium Barbara J. Deal, MD Protein-Losing Enteropathy Professor of Pediatrics, Northwestern University Feinberg School of Medicine; Marvin E. Wodika Research Professor Derek G. Gibson, MB, FRCP of Cardiology and Division Head, Cardiology, Children’s Royal Brompton Hospital, London, United Kingdom Memorial Hospital, Chicago, Illinois Pericardial Constriction after Relief of Subaortic Stenosis Arrhythmia Intervention at the Time of Total Cavopulmonary Conversion Omer Goktekin, MD Adult Congenital Heart Centre, Royal Brompton Hospital, Joseph A. Dearani, MD London, United Kingdom Consultant, Division of Cardiovascular Surgery; Director, Extraanatomic Bypass Graft Repair of Coarctation Thoracic Surgery Resident; Consultant, Division of Surgery; Professor of Surgery, Mayo Clinic, Rochester, Minnesota Massimo Griselli, FRCS Subaortic Stenosis: Indications for Surgery Adult Congenital Heart Centre, Department of Cardiac Surgery, Royal Brompton Hospital, London, United Kingdom Gerhard-Paul Diller, MD Long-Term Outcome after Rastelli Repair Imperial College, London, United Kingdom; University Hospital, Muenster, Germany Maarten Groenink, MD Atrial Septal Defect Associated with Pulmonary Hypertension— Associate Professor, Department of Cardiology, Academic Cause and Effect; Erythrocytosis with Normal Oxygen Saturation Medical Center, Amsterdam, The Netherlands Recurrent Aortic Dissection in Marfan Syndrome Konstantinos Dimopoulos, MD, MSc, PhD Consultant and Senior Fellow, Adult Congenital Heart Sheila G. Haworth, MD, FRCP, FRCPATH, Centre for Pulmonary Hypertension, Royal Brompton FRCPCH, FMedSci Hospital and National Heart and Lung Institute, London, Professor of Developmental Cardiology, Institute of Child United Kingdom Health, University College London; Honorary Consultant Ebstein Anomaly and Sudden Cardiac Death in Paediatric Cardiology, Great Ormond Street Hospital for Children, London, United Kingdom Pulmonary Hypertension after Repair of Congenital Diaphragmatic Hernia viii  Cases in Adult Congenital Heart Disease Howard C. Herrmann, MD Omar Khalid, MD Professor of Medicine; Director, Interventional Cardiology The University of Chicago Pritzker School of Medicine, C o and Cardiac Catheterization Laboratories, University of Chicago, Illinois n Pennsylvania Medical Center, Philadelphia, Pennsylvania Catheter Closure of Ventricular Septal Defects trib u Cyanosis in Ebstein Anomaly and Catheter Closure of Atrial Septal to Defect Sachin Khambadkone, MBBS, DCH, MD, DNB, rs MRCP(UK), CCT Ziyad M. Hijazi, MD, MPH, FSCAI, FACC Honorary Senior Lecturer, Institute of Child Health; Professor of Pediatrics and Internal Medicine; Director, Rush Consultant Paediatric Cardiologist, Cardiothoracic Unit, Center for Congenital and Structural Heart Disease, Rush Great Ormond Street Hospital for Children, London, United University Medical College, Chicago, Illinois Kingdom Catheter Closure of Ventricular Septal Defects Catheter Implantation of Stented Pulmonary Valve Eric M. Horlick, MD, FRCPC Arif Anis Khan, FCPS Assistant Professor of Medicine, Toronto General Hospital, Research Fellow, Cardiology Department, Royal Brompton & Toronto, Ontario, Canada Harefield NHS Trust, London, United Kingdom Catheter Closure of a Patent Ductus Arteriosus Patent Foramen Ovale with Transient Ischemic Attack; Arrhythmia and Syncope in a Patient with a Childhood Murmur; Pulmonary Emmeline F. Hou, MD Atresia with Intact Ventricular Septum; Late Complications after Cardiologist, The Permanente Medical Group, Inc., Roseville, the Arterial Switch Operation California Scimitar Syndrome; Endocarditis in a Young Man; Long-Term Christoph Kiesewetter, MD Survival in Eisenmenger Syndrome Kings College London; Consultant Cardiologist, Guy’s and St Thomas’s NHS Foundation Trust, London, United Kingdom Ross J. Hunter, MRCP Right Ventricular Outflow Obstruction in a Pregnant Woman Research Fellow, Cardiology Research Department, St Bartholomew’s Hospital, London, United Kingdom Philip J. Kilner, MD, PhD Arrhythmia and Syncope in a Patient with a Childhood Murmur Reader in CMR, Imperial College London; Consultant in CMR, Royal Brompton Hospital, London, United Kingdom Toru Ishizaka, MD, PhD Consideration for Automatic Implantable Cardioverter-Defibrillator Chief Cardiovascular Surgeon, Higashi Takarazuka Satoh in Tetralogy Hospital, Takarazuka City, Hyogo, Japan Atrial Septal Defect with Cyanosis: The Hypoplastic Right Igor Knez, MD Ventricle; Ventricular Septal Defect and the Aortic Valve; Total Professor of Cardiovascular Surgery, Medical University of Cavopulmonary Conversion: When and How Graz; Clinical Department of Cardiac Surgery, University Clinic of Surgery, Graz, Austria Bengt Johansson, MD, PhD Congenitally Corrected Transposition of the Great Arteries with Cardiovascular Magnetic Resonance Unit, Royal Brompton Pulmonary Stenosis and Ventricular Septal Detect: When to Hospital; Imperial College of Science and Medicine, London, Intervene United Kingdom Intracardiac Thrombus in the Fontan Circulation Masahiro Koh, MD Clinical Fellow, Paediatric Cardiac Surgery, Royal Brompton Sofian Johar, MB BChir, PhD, MRCP Hospital, London, United Kingdom Specialist Registrar in Cardiology, Royal Brompton Hospital, Partial Anomalous Pulmonary Venous Return; Ebstein Anomaly London, United Kingdom and Wolff-Parkinson-White Syndrome; Complications of Aortic Stenosis and Endocarditis during Pregnancy Ventricular Septation in a Patient with a Single Ventricle Lesley Jones George Krasopoulos, MD, PhD, FRCS-CTH Clinical Nurse Specialist, Adult Congenital Heart Centre, Consultant Cardiac Surgeon, Liverpool Heart & Chest NHS Royal Brompton Hospice, London, United Kingdom Trust, Liverpool, United Kingdom Contraception Counseling Successful Tricuspid Valve Repair of Ebstein Anomaly Henryk Kafka, MD, FRCPC, FACP, FACC Yat-Yim Lam, MRCP Departments of Cardiology and Radiology, Queen’s Associate Professor, Division of Cardiology, Department of University, Kingston, Ontario, Canada Medicine and Therapeutics, Prince of Wales Hospital, The Late Repair of Tetralogy of Fallot; Assessment of Systemic Right Chinese University of Hong Kong, Hong Kong Ventricle Function; The Criss-Cross Heart Transcatheter Options for Atrial Arrhythmia after the Mustard Procedure Thomas K. Kaltsas, MD Clinical and Research Fellow, Department of Adult Astrid E. Lammers, MD Congenital Heart Disease, Royal Brompton Hospital, University College London; Fellow, Pulmonary London, United Kingdom Hypertension, Great Ormond Street Hospital for Children, Outflow Tract Obstruction after Atrioventricular Septal Defect London, United Kingdom Repair Pulmonary Hypertension after Repair of Congenital Diaphragmatic Hernia Ageliki A. Karatza, DS Senior Lecturer in Paediatric Cardiology, Patras University Hospital, Rion, Patras, Greece Dyspnea in a Patient with a Loud Murmur Cases in Adult Congenital Heart Disease ix Wei Li, MD, PhD Michael J. Mullen, MD, FRCP s Adult Congenital Heart Disease Centre, Royal Brompton Consultant Cardiologist, Cardiology Department, Royal or ut Hospital; National Heart Lung Institute, Imperial College Brompton & Harefield NHS Trust, London, United Kingdom b ontri SRcighhoto Hl oeafr St cEiennlacreg eamnedn tM oef dUincicneretain Cause (Sinus Venosus RSyanticoonpael ei nfo ar PSeaptiteanl tD weiftehct a C Clohsiuldrhe oiond t Mhe uErlmdeurrly; Arrhythmia and C Atrial Septal Defect); Rationale for Septal Defect Closure in the Elderly Ed Nicol, MD, MRCP Cardiology and General (Internal) Medicine Specialist Per Lunde, MD Registrar, John Radcliffe Hospital, Oxford, United Kingdom; Senior Consultant Cardiologist, University Hospital of Cardiac Imaging Fellow, National Heart and Lung Institute, Northern Norway, Tromsø, Norway Royal Brompton Hospital, London, United Kingdom Long-Term Follow-Up of Atrioventricular Septal Defect; Pacemaker Fever in a Patient with a Single Ventricle Infection in a Cyanotic Patient; Iron Deficiency in Cyanotic Heart Disease Elena Nikiphorou, MD Royal Brompton Hospital, London, United Kingdom Jonathan Lyne, MRCP Outflow Tract Obstruction after Atrioventricular Septal Defect Royal Brompton Hospital, Cardiovascular Magnetic Repair Resonance Unit, London, United Kingdom Ventricular Arrhythmia Following a Ross Procedure Koichiro Niwa, MD, PhD Director, Department of Adult Congenital Heart Disease, Vaikom S. Mahadevan, MD, MRCP (UK) Chiba Cardiovascular Center, Chiba, Japan Consultant Cardiologist, Manchester Heart Centre, Aortopathy in Tetralogy of Fallot Manchester Royal Infirmary, Manchester, United Kingdom Catheter Closure of a Patent Ductus Arteriosus; Recoarctation: Erwin Oechslin, MD Criteria for Intervention; Catheter Intervention for Baffle Leak or Associate Professor, University of Toronto; Director, Toronto Venous Obstruction Congenital Cardiac Centre for Adults, Toronto, Ontario, Canada Constantine Mavroudis, MD Management of Acute Hemoptysis Professor of Surgery, Case Western Reserve University School of Medicine; Ross Professor of Surgery, Chairman of George Pantely, MD Pediatric and Congenital Heart Surgery, Cleveland Clinic Professor of Medicine (Cardiology), Adult Congenital Heart Foundation, Cleveland, Ohio Disease Program, Oregon Health & Science University, Arrhythmia Intervention at the Time of Total Cavopulmonary Portland, Oregon Conversion Scimitar Syndrome; Endocarditis in a Young Man; Long-Term Survival in Eisenmenger Syndrome Peter R. McLaughlin, MD, FRCP(C) Clinical Adjunct Professor of Medicine, University of Sabrina D. Phillips, MD Toronto; Chief of Staff, Peterborough Regional Health Assistant Professor of Medicine, Mayo College of Medicine; Centre, Peterborough, Ontario, Canada Consultant, Cardiovascular Disease and Internal Medicine, Catheter Closure of a Patent Ductus Arteriosus; Catheter Mayo Clinic, Rochester, Minnesota Intervention for Baffle Leak or Venous Obstruction Interrupted Aortic Arch in a Patient with DiGeorge Syndrome; Coronary Artery Fistulae and Their Significance Victor Menashe, MD Department of Pediatrics, Oregon Health and Science Antonia Pijuan-Domenech, MD University, Portland, Oregon Cardiologist, Hospital Vall d’Hebron, Barcelona, Spain Scimitar Syndrome; Endocarditis in a Young Man; Long-Term Palliative Mustard for Transposition and Ventricular Septal Defect; Survival in Eisenmenger Syndrome The Criss-Cross Heart David B. Meyer, MD Daniele Prati, MD Cardiothoracic Surgery, Schneider Children’s Hospital, New Research Fellow, Adult Congenital Heart Disease Hyde Park, New York Programme, Royal Brompton Hospital, London, United Considerations for the Ross Procedure Kingdom Palliative Mustard for Transposition and Ventricular Septal Defect Shelley D. Miyamoto, MD Assistant Professor of Pediatrics, University of Colorado Peter J. Pugh, MD, MRCP, FESC Denver Health Sciences Center; Director, Cardiomyopathy Consultant Cardiologist, Addenbrooke’s Hospital, and Heart Failure Program, The Children’s Hospital, Cambridge University Hospitals NHS Foundation Trust, University of Colorado, Denver, Colorado Cambridge, United Kingdom Timing and Merits of Transplantation in a Fontan Patient Liver Dysfunction after the Mustard Procedure Barbara J.M. Mulder, MD Ivan M. Rebeyka, MD, FRCS Professor of Cardiology, Academic Medical Center, Clinical Professor, Department of Surgery and Pediatrics, Amsterdam, The Netherlands University of Alberta; Head, Pediatric Cardiovascular Recurrent Aortic Dissection in Marfan Syndrome Surgery, Stollery Children’s Hospital, Edmonton, Alberta, Canada Anomalous Left Coronary Artery from the Pulmonary Artery x  Cases in Adult Congenital Heart Disease Andrew N. Redington Mark S. Spence, MB BCH, MD, BAO (Hons), Division Head, Department of Cardiology, Hospital for Sick MRCP C o CTohrioldnrteon, ;O Snetnairoior ,A Csasnocaidaate Scientist, University of Toronto, HCoonnsourlatrayn St eCcaiordr iLoelocgtuisrte, rR, oQyuael eVni’sst oUrniai vHeorssiptyit aBle, lBfaeslfta; st ntrib u Late Outcome Following Systemic Tricuspid Valve Replacement Trust, Belfast, Northern Ireland, United Kingdom to rs Multiple Aortic-Pulmonary Collaterals: Too Many or Too Few Jonathan Rome, MD, FACC Associate Professor of Pediatrics, University of Pennsylvania Martin St. John Sutton, MB, FRCP School of Medicine; Director, Cardiac Catheterization John W. Bryfogle Professor of Medicine, University of Laboratory, The Children’s Hospital of Philadelphia, Pennsylvania School of Medicine; Director, Cardiovascular Philadelphia, Pennsylvania Imaging; Director, Cardiovascular Fellowship Program, The Recoarctation: Criteria for Intervention Hospital of the University of Pennsylvania, Philadelphia, Pennsylvania Marlon S. Rosenbaum, MD Cyanosis in Ebstein Anomaly and Catheter Closure of Atrial Septal Associate Attending, Columbia University College of Defect Physicians & Surgeons; Director, Schneeweiss Columbia Adult Congenital Heart Center, Columbia University Philip J. Steer, MBBS, MD, FRCOG Medical Center, New York, New York Emeritus Professor of Obstetrics, Imperial College London; Pregnancy and the Systemic Right Ventricle Consultant Obstetrician, Chelsea and Westminster Hospital, London, United Kingdom Jonathon B. Ryan, MBBS, PhD, FRACS Pregnant Patient with Unoperated Truncus Arteriosus Department of Cardiac Surgery, Royal Brompton Hospital, London, United Kingdom Nilesh Sutaria, MD, MRCP Left Atrioventricular Valve Regurgitation: Criteria Consultant Cardiologist, Imperial College Healthcare NHS for Intervention Trust, London, United Kingdom Pericardial Constriction after Relief of Subaortic Stenosis Richard J. Schilling, MD, MRCP Reader, Queen Mary University of London; Consultant Lorna Swan, MB ChB, MD, MRCP Cardiologist, St. Bartholomew’s Hospital, London, United Senior Lecturer, National Heart & Lung Institute, Imperial Kingdom College; Consultant Cardiologist, Royal Brompton & Catheter Ablation for Atrial Arrhythmia Harefield NHS Trust, London, United Kingdom Ebstein Anomaly and Prepregnancy Counseling Babulal Sethia, FRCS Consultant Cardiac Surgeon, Royal Brompton Hospital, Jonathan Swinburn, MBBS, MD London, United Kingdom Consultant Cardiologist, Royal Berkshire Hospital, Reading, Management of Tachyarrhythmia in Tetralogy: Ablation versus United Kingdom Surgery Left Atrial Isomerism Mary N. Sheppard, MD Ju-Le Tan, MBBS, MRCP Royal Brompton Hospital, London, United Kingdom Senior Consultant Cardiologist, Adult Congenital Heart Pericardial Constriction after Relief of Subaortic Stenosis Disease, National Heart Centre, Singapore Right Heart Enlargement of Uncertain Cause (Sinus Venosus Elliot A. Shinebourne, MD, FRCP, FRCPH Atrial Septal Defect); Rationale for Septal Defect Closure in the Consultant Paediatric Cardiologist, Royal Brompton Elderly Hospital, London, United Kingdom Timing of Pulmonary Valve Replacement Judith Therrien, MD Assistant Professor of Medicine, McGill University; Adult Darryl F. Shore, FRCS Congenital Heart Disease Fellowship Director, MAUDE Unit Divisional Director, Heart Division, Consultant Cardiac and Beth Raby Adult Congenital Heart Disease Clinic, Jewish Surgeon, Royal Brompton Hospital and Harefield NHS Trust, General Hospital, Montreal, Quebec, Canada London, United Kingdom Management of Systemic Ventricular Failure Extraanatomic Bypass Graft Repair of Coarctation; Consideration for Automatic Implantable Cardioverter-Defibrillator in Tetralogy Eapen Thomas, MD Grown-Up Congenital Heart Disease Unit, Queen Elizabeth Candice K. Silversides, MD, MS, FRCPC Hospital, Edgbaston, Birmingham, United Kingdom Assistant Professor, University of Toronto; Staff Cardiologist, Anticoagulation in a Pregnant Patient with a Mechanical Valve; University Health Network, Toronto General Hospital, Early Appraisal of Adults with Norwood Correction Toronto Congenital Cardiac Centre for Adults, Toronto, Ontario, Canada Sara Thorne, MBBS, MD Cor Triatriatum; Considerations for Tricuspid Valve Replacement Honorary Senior Lecturer, Consultant Cardiologist, in Patients with a Systemic Right Ventricle University of Birmingham, Birmingham, United Kingdom Anticoagulation in a Pregnant Patient with a Mechanical Valve; Thomas L. Spray, MD Early Appraisal of Adults with Norwood Correction Professor of Surgery, University of Pennsylvania School of Medicine; Chief, Division of Cardiothoracic Surgery, Alice Jan Till, MD Langdon Warner Endowed Chair, The Children’s Hospital of Consultant Electrophysiologist, Royal Brompton Hospital, Philadelphia, Philadelphia, Pennsylvania London, United Kingdom Considerations for the Ross Procedure Management of Tachyarrhythmia in Tetralogy: Ablation versus Surgery; Left Atrial Isomerism Cases in Adult Congenital Heart Disease xi Filippos Triposkiadis, MD, FESC, FACC Gary D. Webb, MD, FRCPC, FACC s Professor of Cardiology, University of Thessaly Medical Professor of Medicine, Director of the Philadelphia Adult or ut School; Director, Department of Cardiology, Larissa Congenital Heart Center, University of Pennsylvania School b ontri ULonnigv-eTresrimty O Huotcsopmitea la, fLtearr Risassat,e lGli rReeepcaeir oPfh Milaeddeilcpinheia, ,T Pheen Hnsoyslpviatnali aof the University of Pennsylvania, C Considerations for Tricuspid Valve Replacement in Patients with a Etsuko Tsuda, MD Systemic Right Ventricle; Recurrent Aortic Dissection in Marfan Staff, Department of Pediatrics, National Cardiovascular Syndrome Center, Suita, Osaka, Japan Long-Term Management of Kawasaki Disease Tom Wong, MRCP Consultant Electrophysiologist, Royal Brompton and Tomohiro Tsunekawa Harefield Hospitals, London, United Kingdom National Cardiovascular Center, Suita City, Osaka, Japan Ventricular Arrhythmia Following a Ross Procedure Ventricular Septal Defect and the Aortic Valve Toshikatsu Yagihara, MD Anselm Uebing, MD Deputy Director and Chairman, Cardiovascular Surgery, Fellow in Adult Congenital Heart Disease, Royal Brompton National Cardiovascular Surgery Center, Osaka, Japan Hospital and Harefield NHS Trust, Adult Congenital Heart Partial Anomalous Pulmonary Venous Return; Ebstein Anomaly Disease Unit, London, United Kingdom and Wolff-Parkinson-White Syndrome; Complications of Pregnancy-Related Complications in Coarctation; Fontan and Ventricular Septation in a Patient with a Single Ventricle; Long- Pregnancy; Pregnant Patient with Unoperated Truncus Arteriosus; Term Management of Kawasaki Disease Left Coronary Artery Arising from the Right Coronary Sinus Steve M. Yentis, MBBS, FRCA, MD, MA Hideki Uemura, MD Honorary Senior Lecturer, Imperial College; Consultant Consultant Cardiac Surgeon, Royal Brompton Hospital, Anaesthetist, Chelsea and Westminster Hospital NHS London, United Kingdom Foundation Trust, London, United Kingdom Atrial Septal Defect with Cyanosis: The Hypoplastic Right Pregnant Patient with Unoperated Truncus Arteriosus Ventricle; Partial Anomalous Pulmonary Venous Return; Ventricular Septal Defect and the Aortic Valve; Left Anji T. Yetman, MD Atrioventricular Valve Regurgitation: Criteria for Intervention; Professor of Pediatrics, Adjunct Professor of Internal Ebstein Anomaly and Wolff-Parkinson-White Syndrome; Successful Medicine, University of Utah; Director, Adult Congenital Tricuspid Valve Repair of Ebstein Anomaly; Total Cavopulmonary Cardiology Program, University Hospital; Director, Adult Conversion: When and How; Complications of Ventricular Congenital Cardiology Program, Primary Children’s Medical Septation in a Patient with a Single Ventricle; Long-Term Center, Salt Lake City, Utah Management of Kawasaki Disease Timing and Merits of Transplantation in a Fontan Patient Gruschen R. Veldtman, MB ChB, MRCP, Dip Panayiotis Zarvos, MD, FCP-SA Obstet Director, Pediatric Department, Arch. Makarios Hospital, Consultant Cardiologist, Congenital Cardiac Unit, University Medical Health Services, Nicosia, Cyprus of Southampton, Southampton University Hospital, Outflow Tract Obstruction after Atrioventricular Septal Defect Southampton, United Kingdom Repair Right Ventricular Outflow Obstruction in a Pregnant Woman Isabelle F. Vonder Muhll, MD Assistant Professor, University of Alberta; Staff Cardiologist, Department of Medicine, University of Alberta Hospital, Edmonton, Alberta, Canada Anomalous Left Coronary Artery from the Pulmonary Artery xii  Cases in Adult Congenital Heart Disease Foreword We live in an era of rapid advances in medicine, including questions about what they might expect from life with their cardiology and cardiac surgery. Because of that, life expectancy defect, my first answer is always, “I don’t know.” Each person’s of patients born with congenital heart disease has increased journey is dependent on the unique specifics of his or her steadily in the past four decades. The result of this is a growing anatomy, history of care, coexisting medical conditions, and the population of adults living with congenital heart disease. Every wild card of luck. The person best equipped to help answer day, teens and young adults with congenital heart disease are these questions is the patient’s own adult congenital heart spe- transitioning from the care of dedicated pediatric cardiologists cialist. into the uncertain world of adulthood. All over the world, spe- Drs. Gatzoulis, Webb, Broberg and Uemura have been at the cialized medical centers and groups are being formed to provide forefront of advances in care of adult congenital heart disease care appropriate for their complex and ongoing medical for many years and have edited or contributed to numerous needs. textbooks for medical professionals. This new book, however, The most common reason patients join the Adult Congenital is the first to focus on specific cases. It includes 85 cases from Heart Association is to find others living with the same condi- first presentation to the latest follow-up, and discusses the tion in the hope of finding others with these rare diagnoses who specific challenges in each patient. By choosing to focus on have shared their particular experiences and struggles. For most individual cases, the editors fully explore all aspects of the of us, survival was dependent on whatever treatments were congenital heart picture and illustrate the array of trajectories available at the time and place of our birth. As medicine that comprise life with congenital heart disease. By creating advanced, treatments were constantly evolving. Over time, all Cases in Adult Congenital Heart Disease, the editors have created the stents, patches, valves, and pacemakers have changed the a unique and essential tool to help ensure that these patients structure of our hearts, further affected by the process of aging find the care they seek. They guide the medical professional to and the specifics of our life and health. a much richer understanding of the complexity one must master What does it mean to be an adult living with congenital heart to help ensure that each of us, patient by patient, gets the life disease? As president of the Adult Congenital Heart Associa- and health care we deserve. tion, I am asked this question every day. A patient facing surgery asks, “Am I likely to feel better?” A newlywed asks, Amy Verstappen “Do you think I should have children?” A parent of an infant President and CEO asks, “Can my child live a normal life?” When patients ask me Adult Congenital Heart Association Cases in Adult Congenital Heart Disease xiii