Moss and Adams' V 0 L Heart Disease U M E In Infants, Children, and Adolescents I Including the Fetus and Young Adult e i g h t h e d i t i o n Hugh D. Allen David J. Driscoll Robert E. Shaddy Timothy F. Feltes Moss and Adams Heart Disease in Infants, Children, and Adolescents Including the Fetus and Young Adult E I G H T H E D I T I O N V O L U M E EDITORS Hugh D. Allen, MD, FACC, Robert E. Shaddy, MD FAAP, FAHA Professor of Pediatrics Department of Pediatrics Professor of Pediatrics (Cardiology) University of Pennsylvania Perelman Baylor College of Medicine School of Medicine Staff Cardiologist Chief, Division of Pediatric Cardiology Texas Children's Hospital Vice Chair, Department of Pediatrics Houston, Texas The Children's Hospital of Pennsylvania Philadelphia, Pennsylvania David J. Driscoll, MD Professor of Pediatrics Timothy F. Feltes, MD Division of Pediatric Cardiology Andy Paxon Chair in Cardiology Department of Pediatric and Adolescent Co-Director of the Heart Center Medicine Chief, Pediatric Cardiology Mayo Clinic College of Medicine Professor of Pediatrics Mayo Clinic Foundation The Ohio State University Rochester, Minnesota Nationwide Children's Hospital Columbus, Ohio Wolters Kluwer Lippincott Williams & Wilkins Health Philadelphia • Baltimore • New York • London Buenos Aires • Hong Kong • Sydney • Tokyo Acquisitions Editor: Julie Goolsby Product Manager: Leanne Vandetty Production Manager: Alicia Jackson Senior Manufacturing Manager: Benjamin Rivera Marketing Manager: Kimberly Schonberger Design Coordinator: Teresa Mallon Production Service: SPiGlobal © 2013 by LIPPINCOTT WILLIAMS &WILKINS, aWOLTERS KLUWER business Two Commerce Square 2001 Market Street Philadelphia, PA 19103 USA LWW.com 7th Edition © 2008 by Lippincott Williams & Wilkins 6th Edition © 2001 by Lippincott Williams & Wilkins All rights reserved. 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C\I 0; 10 9 8 7 6 5 4 3 2 1 o CIl o o Dedication To my grandchildren, Christopher, Stella, and Adam, and to my families at home as well as at work. -HDA To my grandchildren, Brendan and Emmaleine. -DJD To family and friends for their patience and support. -RES Jethro got it right; life's a long song starting with you, Mr. Johnson. But was the deal worth it? It may be a buckdancer's choice to travel with traffic or to take the lonely Ventura Highway, but we always seem to end up in Winslow Arizona. To Taj, Ste- vie Rae, John Lee, Lightnin', Eric, and the King himself, thanks for the tunes boys. Pete, glad the Detours name didn't stick but then the lead balloon" did! And to Club cc 27's Jim, Jimi, and Janis, your poetry lives on. I'm bad ... I'm Nationwide! -TFF Contributors ix 11 Cardiac Computed Tomography Preface xvii in Children with Congenital Heart Acknowledgments xutu Disease 247 Tribute to Moss, Adams, & Emmanouilides xix Frederick R.Long and Joachim G.Eichhorn The Development of Pediatric Cardiology: Historical Milestones http://solution.lww.com 12 Cardiac Catheterization and Angiography 258 Nathaniel W Taggart and Allison K.Cabalka VOLUME I 13 Therapeutic Cardiac Catheterization .. 288 I: SECTION Ralf J. Holzer and John PCheatham Structure and Function ofthe Cardiovascular System 1 III: ---- SECTION Electrophysiology 348 1 Cardiac Anatomy and Examination of Cardiac Specimens 1 14 Development and Functional William D.Edwards and Joseph J. Maleszewski Maturation of the Cardiac 2 Classification and Terminology Conduction System 348 of Cardiovascular Anomalies 32 Aleksander Sizarov, Antoon F.M.Moorman, and Arthur S.Pickoff William D.Edwards and Joseph J. Maleszewski 15 The Normal Electrocardiogram ..... 372 3 Development of Myocardial Structure George F.Van Hare and Anne M. Dubin and Function 52 16 Cardiac Channelopathies, Syncope, PamelaALucchesi, KirkR.Hutchinson, Luciana Martins, andLorenE.Wold and Sudden Death 389 4 Circulation Physiology 69 Michael J.Ackerman David F.Teitel, Steven C.Cassidy, and Jeffrey R.Fineman 17 Electrophysiology Studies and Electrophysiologic Therapeutic SECTION II: Catheterization 401 Diagnostic and Therapeutic Methods 82 J. Philip Saul and John D.Kugler 18 Disorders of Cardiac Rhythm 5 History and Physical Examination .... 82 and Conduction 441 Steven C Cassidy, Hugh D.Allen, and John R.Phillips Bryan C.Cannon and Christopher S.Snyder 6 Exercise Screening and Sports Participation 93 IV: SECTION Julie A Brothers, Paul Stephens, Jr..and Stephen M. Paridon Pediatric Cardiac Intensive Care 473 7 Exercise Testing 118 Paolo 1 Pianosi and David J. Driscoll 19 Physiology of the Preterm and Term 8 Echocardiography: Basic Principles Infant 473 and Imaging 134 Timothy M. Hoffman and Stephen E.Welty Thomas R.Kimball and ErikC.Michelfelder 20 Pediatric Cardiovascular Intensive 9 Echocardiographic Assessment Care 483 of Cardiac Dimensions, Cardiac Dean B.Andropoulos and Anthony C.Chang Function, and Valve Function 172 21 Cardiac Mechanical Support LucL.Mertens and Mark K.Friedberg Therapies 530 10 Magnetic Resonance Imaging 207 Elizabeth D.Blume, Ravi R.Thiagarajan, and Peter C.Laussen TalGevaand Andrew J. Powell v vi Contents 22 Cardiopulmonary and Right-Left VOLUME II Heart Interactions 546 PART C: Venous Abnormalities Andrew N.Redington 35 Anomalies of the Pulmonary Veins... 809 23 Cardiac Trauma 552 David W. Brown andTalGeva GaryA.Smith andTimothy F.Feltes 36 Abnormal Systemic Venous Connections 840 V: SECTION TalGeva From the Gene to the Neonate 560 37 Vascular Tumors and Anomalies .... 864 24 Molecular Determinants of Cardiac Ahmad I.Alomari Development and Disease 560 38 Tricuspid Atresia, Stenosis, Regurgitation, and Uhl's Anomaly ... 877 Deepak Srivastava and H.Scott Baldwin 25 Epidemiology and Prevention Michael L.Epstein of Congenital Heart Defects 577 39 Ebstein's Anomaly, Congenital Tricuspid Valve Regurgitation, Lorenzo D.Botto and Dysplasia 889 26 Genetic Aspects of Congenital Heart Defects 617 Sameh M. Said and Joseph A. Dearani 40 Pulmonary Stenosis 913 Elizabeth Goldmuntz, Melissa L.Crenshaw, and Angela E.Lin 27 Fetal Echocardiography and Fetal Lourdes R.Prieto and LarryA.Latson Cardiology 644 41 Pulmonary Atresia and Intact Ventricular Septum 939 Charles S Kleinman, Julie S.Glickstein, Ganga Krishnamurthy, andJodie K.Votava-Smith David G.Nykanen 42 Pulmonary Atresia and Ventricular Septal Defect 959 VI: SECTION Patrick W. O'Leary, William D.Edwards, PaulR.Julsrud, Congenital Cardiovascular Malformations 672 and Harold MacDonald Burkhart PART A: Septal Defects 43 Tetralogy of Fallot with Pulmonary 28 Atrial Septal Defects 672 Stenosis and Tetralogy of Fallot with Absent Pulmonary Valve 969 Ritu Sachdeva 29 Atrioventricular Septal Defects 691 S.LucyRoche, Steven C.Greenway, and Andrew N.Redington FrankCetta, Luann L.Minich, Joseph J. Maleszewski, PART 0: Left Ventricular Inflow and Outflow Joseph A.Dearani, and Harold MacDonald Burkhart Abnormalities 30 Ventricular Septal Defects 713 44 Truncus Arteriosus 990 Agustin E Rubio and Mark B.Lewin Allison K.Cabalka, William D.Edwards, andJoseph A.Dearani PART B: Arterial Abnormalities 45 Anatomical and Functional Mitral 31 Patent Ductus Arteriosus and Valve Abnormalities in the Pediatric Aortopulmonary Window 722 Population 1003 Phillip Moore and Michael M. Brook Andrew S.Mackie andJeffrey F.Smallhorn 32 Congenital Anomalies of the Coronary 46 Aortic Stenosis 1023 Vessels and the Aortic Root 746 Douglas J. Schneider andJohn W. Moore D.Scott Limand G.PaulMatherne 47 Coarctation of the Aorta 1044 33 Aortic Arch and Vascular Robert H.Beekman, III Anomalies 758 48 Hypoplastic Left Heart Syndrome .. 1061 PaulM. Weinberg, Shobha Natarajan, and Lindsay S.Rogers James S Tweddell, George M. Hoffman, Nancy S.Ghanayem, 34 Abnormalities of the Aortic Root .... 799 Michele A.Frommelt, Kathleen A.Mussatto, and Stuart Berger Matina Prapa, John Pepper,and Michael A.Gatzoulis Index 1-1 Contents vii PART E: Abnormalities ofthe Origin ofthe Great 64 Myocardial Ischemia 1377 Arteries Ali N.Zaidi and Curt J. Daniels 49 Transposition ofthe Great Arteries... 1097 65 Pediatric Heart Transplantation .... 1384 GilWernovsky Robert E.Shaddy and Francesco Parisi 50 Congenitally Corrected Transposition of the Great Arteries VIII: SECTION (Atrioventricular and Pulmonary Vascular Disease 1401 Ventriculoarterial Discordance) .... 1147 Joseph Atallah, Jennifer M. Rutledge, andJohn D.Dyck 66 Pathophysiology of Pulmonary Hypertension 1401 F: PART Complex Cardiac Abnormalities Marlene Rabinovitch 51 Double Outlet Right Ventricle ..... 1161 67 Clinical Management of Pediatric Gail E.Wright, Katsuhide Maeda, Norman H.Silverman, Pulmonary Arterial Hypertension ... 1433 FrankL.Hanley, and Stephen J. Roth D.Dunbar Ivy 52 Univentricular Atrioventricular Connection 1175 IX: Michael G.Earing, Donald J. Hagler, and William D.Edwards SECTION 53 Cardiac Malpositions and Abnormalities The Young Adult with Congenital of Atrial and Visceral Situs 1195 Heart Disease 1463 Patrick W. O'Leary and Donald J. Hagler 68 The Adolescent and Adult with Congenital Heart Disease..... 1463 VII: SECTION Ali N.Zaidi and CurtJ. Daniels Diseases ofthe Endocardium, 69 Pregnancy in Young Women Myocardium, and Pericardium 1217 with Congenital Heart Disease..... 1494 54 Hypertrophic Cardiomyopathy .... 1217 Candice K.Silversides, Jack M. Colman, and Samuel C.Siu BarryJ. Maron 55 Dilated Cardiomyopathy 1235 X: SECTION Timothy M. Olson and David P.Chan Other Special Problems and Issues 1509 ._----- 56 Myocarditis 1247 Jeffrey A.Towbin. Angela l.orts. andJohn LynnJefferies 70 Chest Pain in Children 57 Restrictive Cardiomyopathy 1267 and Adolescents 1509 Susan W. Denfield Jonathan N.Johnson and David J. Driscoll 58 TheHeart inMuscular Dystrophies... 1277 71 Coronary Risk Factors in Children.. 1514 Hugh D.Allen, Jerry R.Mendell, Kevin M. Flanigan, Stephen R.Daniels andTimothy M. Hoffman 72 Cardiac Tumors 1549 59 Kawasaki Disease (Mucocutaneous Gerald R.Marx andAdrian M. Moran Lymph Node Syndrome) 1287 73 Chronic Heart Failure in Children .. 1565 Sarah D.DeFerranti andJane W. Newburger Robert E.Shaddy and Lloyd Y.Tani 60 Rheumatic Fever and Rheumatic 74 NeurodevelopmentalOutcomes Heart Disease 1303 after Heart Surgery in Children .... 1579 Lloyd Y.Tani Caren S.Goldberg and Jane W. Newburger 61 Inflammatory Noninfectious 75 Hematologic Aspects of Pediatric Cardiovascular Diseases 1331 and Adolescent Heart Disease: Charles H.Spencer, Anjali Patwardhan, and Sharon L.Roble Bleeding,Clotting, and Blood 62 Pericardial Diseases 1350 Component Abnormalities 1589 Jonathan N.Johnson and FrankCetta Therese M. Giglia and CharWitmer 63 InfectiveEndocarditis and Prevention 1363 Michael Gewitz and Kathryn A.Taubert viii Contents 76 Evaluation of Quality of Life in Children 78 Design, Execution, and Appraisal and Adolescents with Congenital and of Clinical Trials 1636 Acquired Heart Disease 1608 Brian W McCrindle Bradley S.Marino 79 Pharmacology 1661 77 Safety and Quality in the Heart Michael Artman and Gregory L.Kearns Center 1622 Index I-I Anthony Y Lee,Thomas Taghon, Richard Eugene McClead, Jr., Terrance Davis,and Richard J. Brilli Michael J.Ackerman. MD. PhD,FACC H.ScottBaldwin, MD Michael M. Brook,MD Windland Smith Rice Cardiovascular Professor ofPediatrics and Celland Professor of Clinical Pediatrics Genomics Research Professor Developmental Biology Director, Pediatric and Congenital Professor ofMedicine, Pediatrics, and Chief, Division ofPediatric Echocardiography Pharmacology Cardiology University of California-San Francisco Director, Long QT Syndrome/Genetic Co-Director ofthe Pediatric Heart BenioffChildren's Hospital Heart Rhythm Clinic Institute SanFrancisco, California Director, Windland Smith RiceSudden Vanderbilt Children's Hospital Death Genomics Laboratory Nashville, Tennessee Julie A. Brothers,MD Medicine, Pediatrics, and Molecular Assistant Professor Pharmacology and Experimental RobertH.Beekman, III, MD Department ofPediatrics Therapeutics Professor Perelman SchoolofMedicine at the Mayo Clinic Department ofPediatric University ofPennsylvania Rochester, Minnesota Cardiology Medical Director, Lipid Heart Clinic UniversityofCincinnatiCollegeof Division of Cardiology HughD.Allen, MD, FACC,FAAP.FAHA Medicine The Children's Hospital ofPhiladelphia Professor ofPediatrics (Cardiology) Professor Philadelphia, Pennsylvania Baylor CollegeofMedicine Heart Institute StaffCardiologist Cincinnati Children's Hospital Medical DavidW. Brown, MD Texas Children's Hospital Center Assistant Professor Houston, Texas Cincinnati, Ohio Department ofPediatrics Harvard Medical School Ahmad I.Alomari. MD, MSc Stuart Berger,MD Associate Assistant Professor Professor ofPediatrics Department of Cardiology Division ofVascular and Interventional The Medical CollegeofWisconsin Children's Hospital Boston Radiology Medical Director ofthe Herma Heart Boston, Massachusetts Children's Hospital Boston Center Harvard Medical School Children's Hospital ofWisconsin Harold MacDonald Burkhart, MD Boston, Massachusetts Milwaukee, Wisconsin Associate Professor of Surgery/ Cardiovascular Surgeon Dean B.Andropoulos. MD Elizabeth D.Blume, MD Department of Cardiovascular Surgery Professor ofAnesthesiology Associate Professor Mayo Clinic Director, Pediatric Cardiovascular Department ofPediatrics Rochester, Minnesota Anesthesia Harvard Medical School Texas Children's Hospital Medical Director, Heart Failure and Allison K.Cabalka, MD, FACC,FASE, Houston, Texas Transplant Program FSCAI Department of Cardiology Associate Professor ofPediatrics Michael Artman, MD Children's Hospital, Boston Division ofPediatric Cardiology Chair Boston, Massachusetts Mayo Clinic Department ofPediatrics Rochester, Minnesota School ofMedicine lorenzo D.Botto,MD University ofMissouri-Kansas City Professor ofPediatrics Bryan C.Cannon,MD Joyce c. Hall Distinguished Professor Department ofPediatrics Associate Professor and Chair University ofUtah Department ofPediatrics Department ofPediatrics Attending Physician Director Children's Mercy Hospitals and Clinics Division ofMedical Genetics Pediatric Arrhythmia and Kansas City,Missouri Primary Children's Medical Pacing Service Center Mayo Clinic JosephAtallah, MD CM, MSc(Epi), SaltLake City,Utah Rochester, Minnesota FRCPC Assistant Professor RichardJ. Brilli, MD Steven C.Cassidy.MD Departments ofPediatrics and Public Professor Associate Professor Health Sciences Department ofPediatrics Department ofPediatrics University ofAlberta The Ohio StateUniversity Collegeof Ohio StateUniversityCollegeof Attending Staff Medicine Medicine Departments ofPediatrics and Medicine ChiefMedical Officer Director, Inpatient Services Stollery Children's Hospital and Univer- Hospital Administration Heart Center sityofAlberta Hospital Nationwide Children's Hospital Nationwide Children's Hospital Edmonton, Alberta, Canada Columbus, Ohio Columbus, Ohio ix