Visual Guide to Neonatal Cardiology

Visual Guide to Neonatal Cardiology

Edited by

Ernerio T. Alboliras, Ziyad M. Hijazi, Leo Lopez, and Donald J. Hagler

This edition first published 2018© 2018 John Wiley & Sons Ltd

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Library of Congress Cataloging-in-Publication Data

Names: Alboliras, Ernerio T., editor. | Hijazi, Ziyad M., editor. | Lopez,Leo, editor. | Hagler, Donald J., editor.

Title: Visual guide to neonatal cardiology / edited by Ernerio T. Alboliras,Ziyad M. Hijazi, Leo Lopez, and Donald J. Hagler.

Description: Hoboken, NJ : Wiley, 2018. | Includes bibliographical referencesand index. |

Identifiers: LCCN 2017054083 (print) | LCCN 2017054741 (ebook) | ISBN9781118635346 (pdf) | ISBN 9781118635223 (epub) | ISBN 9781118635148(hardback)

Subjects: | MESH: Heart Diseases | Infant, Newborn, Diseases | Fetal Diseases| Infant, Newborn

Classification: LCC RJ421 (ebook) | LCC RJ421 (print) | NLM WS 290 | DDC618.92/12–dc23

LC record available at https://lccn.loc.gov/2017054083

Cover Design: WileyCover Image: © garymilner/Gettyimages

Set in 10/12pt WarnockPro by SPi Global, Chennai, India

10 9 8 7 6 5 4 3 2 1

We would like to dedicate this book to our spouses, families, colleagues, trainees both past and present, sonographers,and friends. We would also like to thank the international group of notable authors of this book for their exceptionalscholarly contributions to the understanding of the complexity of neonatal heart disease.

vii

Contents

Preface xxiList of Contributors xxiii

Part I Prenatal and Perinatal Issues 1

1 Cardiac Embryology and Embryopathy 3Robert H. Anderson, Nigel A. Brown, and Timothy J. MohunInitial Stages of Development 3Looping of the Heart Tube 3The Process of Ballooning 5Formation of the Atrial Chambers 5Atrial Septation 7Ventricular Development 12Development and Maldevelopment of the Outflow Tract 15Development of the Coronary Circulation 19Acknowledgments 21References 21

2 Maternal, Familial, and Non-Cardiac Fetal Conditions Affecting the Fetal and Neonatal Heart 23Miwa K. Geiger and Anita J. Moon-GradyMaternal Factors 23

Metabolic 23Autoimmune 23Infection 23Cardiac Teratogens 25

Twins 25Familial 25Non-cardiac Fetal Conditions 27

High Output Lesions 27Space-Occupying Thoracic Lesions 29Placental Abnormalities 29

References 29

3 The Natural and Unnatural History of Fetal Heart Disease 31Karim A. Diab and Samer MasriReferences 39

Part II General Neonatal Issues 41

4 Epidemiology of Heart Defects 43Gregory H. Tatum and Piers C.A. BarkerPrevalence of Individual Lesions 43Changes in Prevalence Over Time 43

viii Contents

Regional and Racial Variation 43Impact of Fetal Testing 45Non-Genetic Risk Factors 45References 46

5 The Transitional and Neonatal Heart and Cardiovascular System 48Timothy M. CordesBirth and Neonatal Period 48Acknowledgment 52References 52

6 History and Physical Examination 53Ernerio T. AlbolirasHistory 53Physical Examination 54Further Reading 55

7 The Cyanotic Newborn 56Ernerio T. AlbolirasReference 57Further Reading 58

8 The Tachypneic Newborn 59Ernerio T. AlbolirasReference 60Further Reading 60

9 The Hypoperfused Newborn 61Deepti BhatEtiology 61History 61Physical Examination 61Diagnostic Tests 62Management 62Further Reading 63

10 The Dysmorphic Newborn 64Stephanie Burns Wechsler and Marie McDonaldFurther Reading 69

Part III Diagnostic Procedures 71

11 Chest Roentgenogram 73Randy Richardson, Darshit Thakrar, and Deepak KauraPulmonary Vascularity 73Cardiomegaly 74Situs 74Great Vessels 75Extracardiac Evaluation 76Lines and Tubes in Patients With Congenital Heart Disease 78Complications 78

Contents ix

12 Electrocardiogram 80Bryan CannonECG Leads 80Basic ECG Setup 80Wave Morphologies 80ECG Intervals 80Heart Rate 81Cardiac Rhythm 81Axis 81Right-sided Chest Leads 81Abnormalities on the ECG 82

Atrial Enlargement 82Ventricular Hypertrophy 82Low Voltage QRS 82QRS Duration 82Wolff–Parkinson–White 83Abnormal Q Waves 83T-wave Changes 84ST Segments 84

Further Reading 84

13 Echocardiogram 85Michael D. QuartermainHistory 85Physics 85Performance of a Pediatric Echocardiogram 85

Subcostal (Subxiphoid) View 86Apical View 87Parasternal View 87Suprasternal View 90

References 90

14 Cardiac Catheterization and Angiocardiography 91Howaida El-Said and Sergio BartakianPatent Ductus Arteriosus (Figures 14.1 and 14.2) 91Pulmonary Valve Stenosis (Figure 14.3) 91Critical Aortic Valve Stenosis (Figure 14.4) 91Coarctation of the Aorta (Figure 14.5) 91Major Aorto-Pulmonary Collateral Arteries (Figure 14.6) 91Transposition of the Great Arteries (Figure 14.7) 95Hypoplastic Left Ventricle (Figure 14.8) 95Total Anomalous Pulmonary Venous Connection (Figure 14.9) 96Rotational Angiography (Figure 14.10) 96References 97

15 Computed Tomography 98Randy RichardsonScanning Technique for Cardiac CTA in Neonates 98Advantages of Cardiac CTA Over Other Imaging

Modalities 100Postprocessing of Cardiac CTA 102Further Reading 103

x Contents

16 Magnetic Resonance Imaging 104Shaine A. Morris and Timothy C. SlesnickIndications for Neonatal CMR 106

Intrathoracic Vascular Evaluation 106Native Intracardiac Anatomy and Surgical Planning 107Postoperative Assessment 108Neonatal Tumor Evaluation 108

References 108

17 Electrophysiologic Testing, Transesophageal Pacing and Pacemakers 109Bryan CannonPacemakers 109Implantable Cardioverter-Defibrillators 109Electrophysiology Studies 109Transesophageal and Temporary Pacing 110Reference 111

Part IV Specific Morphologic Conditions 113

18 Total Anomalous Pulmonary Venous Connection 115David W. Brown and Tal GevaDefinition 115Etiology 115Embryologic Basis 115Anatomy 115Pathophysiology 118Total Anomalous Pulmonary Venous Drainage 119Treatment 119References 120

19 Other Anomalies of Pulmonary and Systemic Venous Connections 121Mark V. Zilberman and Clifford L. CuaAchnowledgment 125References 125

20 Anomalies of Atrial Septation 127Darren Hutchinson and Lisa HornbergerBackground and Anatomy 127Neonatal Presentation 127Management and Treatment Options 129References 132

21 Atrial Chamber Obstruction 133Lisa HornbergerCor Triatriatum Sinister 133

Background and Anatomy 133Neonatal Presentation 133Investigations to Make the Diagnosis 133Management 135

Cor Triatriatum Dexter 135References 136

22 Common Atrioventricular Canal Defects 137Meryl S. Cohen, MDPathophysiology 137Clinical Manifestation 137

Contents xi

Investigations 138Management 139References 143

23 Ventricular Septal Defect 145Lowell FrankPathophysiology 145Clinical Presentation and Diagnosis 145Nomenclature and Anatomy 145Management 149References 151

24 Tricuspid Atresia 152Nathaniel W. TaggartAnatomy 152Physiology 152

Type I: Normally Related Great Arteries 152Type I-A: Pulmonary Atresia 152Type I-B: Pulmonary Stenosis and Restrictive VSD 153Type I-C: Large VSD and No PS 153

Type II: D-Transposition of the Great Arteries 153Type II-A: D-TGA with Pulmonary Atresia 153Type II-B: D-TGA with PS 153Type II-C: D-TGA with no PS 153

Physical Examination 153Electrocardiogram 156Chest X-Ray 156Echocardiography 156Cardiac Catheterization 157Preoperative Management 157Surgical Management 157Postoperative Management 157References 157

25 Ebstein Malformation and Tricuspid Valve Dysplasias 158Sameh M. Said, Donald J. Hagler, and Joseph A. DearaniEbstein Malformation 158

Clinical Presentation 158Newborn 158Children and Adults 158

Preoperative Evaluation 158Indications for Surgery 158

Neonates 158Children and Adults 159

Surgical Techniques 159Neonate 159Children and Adults 159

Postoperative Management 163Mayo Clinic Experience 164

Tricuspid Valve Dysplasia 164Uhl Anomaly 165

References 165

26 Pulmonary Valve and Pulmonary Arterial Stenosis 167Evan M. Zahn and Darren P. BermanPulmonary Valve Stenosis 167

xii Contents

Pulmonary Arterial Stenosis 171References 172

27 Pulmonary Atresia with Intact Ventricular Septum 173Kiran K. Mallula and Zahid AminEmbryology 173Pathology 173Physical Examination 174Chest X-ray 174Electrocardiography 174Echocardiography 174Cardiac Catheterization 175Medical Therapy 176Surgery 177Transcatheter Therapy 178Outcomes 178Conclusions 178References 178

28 Tetralogy of Fallot with Pulmonary Stenosis or Atresia 179Muhammad Yasir Qureshi and Frank CettaTetralogy of Fallot with Pulmonary Stenosis 179

Morphologic and Anatomic Features 179Clinical Manifestations 179Laboratory and Imaging Investigations 181Management 181Outcome 182

Pulmonary Valve Atresia with Ventricular Septal Defect (Tetralogy of Fallot with Pulmonary Atresia) 183Morphologic and Anatomic Features 183Clinical Manifestations 186Laboratory and Imaging Investigations 186Management 188Outcome 189

Further Reading 189

29 Absent Pulmonary Valve 190Brieann Muller and Sawsan AwadReferences 193

30 Transposition of the Great Arteries 194Adam L. DorfmanReferences 198

31 Congenitally Corrected Transposition of the Great Arteries 199Camden L. Hebson and William L. BorderMorphology and Associated Lesions 199Clinical Presentation 201Outcomes and Interventions 202References 204

32 Common Arterial Trunk (Truncus Arteriosus) 205Michael C. Mongé, Osama Eltayeb, Andrada Popescu, and Carl L. BackerReferences 209

33 Mitral Valve Apparatus Abnormalities 210Shubhika SrivastavaNormal Mitral Valve Complex 210

Contents xiii

Embryology 210Abnormalities of Mitral Valve Apparatus 210

Abnormality of Leaflet 210Mitral Valve Prolapse 210Cleft Mitral Valve 211Double Orifice Mitral Valve 211Supramitral Ring 211Ebstein Malformation of Mitral Valve 212Accessory Mitral Valve 212Mitral Valve Duplication 212

Anomalies of the Chordae and Papillary Muscles (Tensor Apparatus) 212Chordal Anomalies 212Anomalies of the Papillary Muscles (Most Common of All Described Abnormalities) 213

Conclusions 214References 215

34 Hypoplastic Left Heart Syndrome 217Aaron Bell and Hannah Bellsham-RevellAnatomy 217Physiology 218Diagnosis 220Management 222Prognosis 223References 225

35 Aortic Stenosis 226Salwa Gendi and Ra-id AbdullaAortic Valve Stenosis 226

Definition 226Incidence 226Pathology 226Pathophysiology 226Natural History 226Clinical Manifestations 227Electrocardiography 227Chest X-ray 227Echocardiography 227Cardiac Catheterization 229Treatment 229Follow-up 229

Subaortic Stenosis 231Supravalvar Aortic Stenosis 232

References 232Further Reading 232

36 Coronary Artery Anomalies 233Grace Choi and Peter KoenigReferences 237

37 Aorto-Left Ventricular Tunnel 238Patrick W. O’LearyAorto-Left Ventricular Tunnel: Anatomic Characteristics 238Associated Anomalies 239Clinical Presentation 239Diagnostic Methods 241

xiv Contents

Management 243Conclusions 244References 244

38 Coronary Cameral Fistulas 245Gareth J. Morgan and Shakeel A. QureshiDefinition 245Etiology 245Pathophysiology and Natural History 245Diagnosis 245Management Strategies 247Interventional Catheterization 247Surgical Therapy 247Conclusions 248References 248

39 Aortopulmonary Window 249Carl L. Backer, Michael C. Mongé, Andrada R. Popescu, and Osama EltayebReference 252Further Reading 252

40 Anomalous Origin of a Branch Pulmonary Artery From the Ascending Aorta (Hemitruncus) 253Michael C. Mongé, Osama Eltayeb, Andrada R. Popescu, and Carl L. BackerFurther Reading 254

41 Interrupted Aortic Arch 255Michael C. Mongé, Hyde M. Russell, and Carl L. BackerReferences 259

42 Coarctation of the Aorta 260Hitesh Agrawal, John W. Bokowski, and Damien KennyPathophysiology 260Clinical Manifestation 260Investigations 260Management 261References 264

43 Vascular Rings and Pulmonary Slings 265Donald J. Hagler and Jessica BowmanAnomalies of the Aortic Arch 265

Embryology 265Vascular Rings 265

Double Aortic Arch 266Anomalous Subclavian Artery (Kommerell diverticulum) 267Diagnosis 270Pulmonary Artery Slings 271References 273

44 Double Outlet Right Ventricle 274Irene D. Lytrivi and H. Helen KoAcknowledgments 278References 279

45 Double Outlet Left Ventricle 280Sarah Chambers Gurson and Leo LopezEtiology 280Morphology 280

Contents xv

Pathophysiology 281Surgical Repair 282Long-term Outcomes 282References 282

46 Single Ventricle and Biventricular Hearts with Hypoplasia of One Ventricle 283Denise A. Hayes, Sujatha Budde, and Wyman W. LaiEmbryology and Genetics 283Prenatal Circulation 283

Dominant Right Ventricle 283Dominant Left Ventricle 283

Postnatal Circulation and Clinical Presentation 286Preoperative Evaluation 286Management 287

Prostaglandin E1 287Balloon Atrial Septostomy 287Other Medical Therapies 287

Surgical Strategies 287Biventricular Repair 287No Critical Outflow Obstruction 289Single Ventricle Palliation 289

Outcomes 289Conclusions 290References 290

47 Dextrocardia and the Heterotaxy Syndromes 292Sowmya Balasubramanian and Rajesh PunnHeterotaxy Syndrome 292Definitions 292Associated Non-Cardiac Anomalies 293Associated Cardiac Anomalies 294Management 296Outcomes 296Conclusions 296References 297

48 Ectopia Cordis and Thoracopagus Twins 298Sawsan AwadEctopia Cordis 298Thoracopagus Twins 298References 301

49 Patent Ductus Arteriosus 302Robert PuntelClinical Findings 306Imaging 306Management 306References 307

50 Neonatal Hypertrophic Cardiomyopathy and Syndromes with Infantile Cardiac Hypertrophy 308J. Martijn Bos and Michael J. AckermanHypertrophic Cardiomyopathy 308Common Causes of Cardiac Hypertrophy in Neonates 308Characteristics of Neonatal HCM and Syndromes with Concomitant Cardiac Hypertrophy 309Genetics of Neonatal HCM, Noonan Syndrome, and Pompe Disease 310

xvi Contents

Survival and Outcomes in Neonatal HCM and Syndromes with ConcomitantLVH 312

Conclusions 312References 312

51 Dilated Cardiomyopathy and Myocarditis 313Jonathan N. JohnsonEtiology 313Presentation 313Testing 314Neonatal Myocarditis 316

Etiology 316Incidence 316Presentation 316Testing 317

References 317

52 Cardiac Chamber Aneurysms and Diverticula 319Lisa HornbergerVentricular Diverticula and Aneurysms 319

Background and Anatomy 319Neonatal Presentation 319Investigations 319Management 321

Atrial Diverticula and Aneurysms 322References 322

53 Cardiac Tumors 323Nadine Choueiter and Rebecca S. BeroukhimCardiac Rhabdomyoma 323Intrapericardial Teratoma 323Fibromas 324Cardiac Hemangioma 326References 328

54 Arteriovenous Malformations 330Allison K. CabalkaIntracranial 330Extracranial Arteriovenous Malformations 333

Intrahepatic 333Other Systemic Arteriovenous Malformations 333Pulmonary Arteriovenous Malformation 334

Conclusions 335Acknowledgement 335References 335

55 Pericardial Defects 336Donald J. HaglerManifestations 336Diagnosis 337Treatment 337Ectopia Cordis 337Treatment 339References 340

Contents xvii

56 Miscellaneous Chest Abnormalities Affecting the Heart: Diaphragmatic Hernia and Eventration; CongenitalCystic Adenomatoid Malformation of the Lung 342Mark WylamDiaphragmatic Hernia 342

Embryologic Development of the Diaphragm 342Postnatal Anatomy and Physiology of the Diaphragm 343Types of Congenital Diaphragmatic Hernia 343Etiology of CDH 343Congenital Heart Disease in CDH 343Newborn Pathophysiology in CDH 344Diagnosis of CDH 344Pre- and Postnatal Management of CDH 344Outcome and Prognosis of CDH 345

Eventration and Diaphragm Paresis 345Eventration 345Epidemiology of Eventration and Acquired Diaphragm Paralysis 345Pathogenesis of Eventration 345Clinical Features of Eventration 346Diagnosis of Eventration 346Management of Eventration 346

Congenital Cystic Adenomatoid Malformation of the Lung (Congenital Pulmonary AirwayMalformation) 346

References 347

57 Persistent Pulmonary Hypertension of the Newborn 350Amish Jain and Mark K. FriedbergCirculatory Pathophysiology 350Diagnostic Work-up 350Management Considerations 350Conclusions 351References 358

58 Hydrops Fetalis 359James C. HuhtaPresentation 359Causes 359Evaluation 360

Evaluation for Immune Hydrops 360Evaluation for Non-immune Hydrops 360

Treatment 361Maternal Complications 361Prognosis 361Fetal Hydrops for the Perinatal Cardiologist 362Counseling 363Antepartum 363Delivery 363CV Profile Score in Hydrops 363

Umbilical and Ductus Venous Doppler 363Cardiomegaly 365Abnormal Myocardial Function 365Arterial Doppler Redistribution of Fetal Cardiac Output 366Cardiovascular Profile Score 367Conclusions 367References 367

xviii Contents

Part V Rhythm Disturbances in the Newborn 369

59 Structural, Metabolic, and Genetic Abnormalities Affecting the Neonatal Conduction System 371Supaluck Kanjanauthai and Ira ShettyReferences 379

60 Tachydysrhythmias 380Barbara J. DealSupraventricular Tachycardia 380

Evaluation and Treatment 383Ventricular Tachydysrhythmias 383

Evaluation and Treatment 384References 384

61 Bradydysrhythmias 386Gregory Webster and Barbara J. DealDiagnosis of Abnormal Atrioventricular Conduction 386Etiologies of Abnormal Atrioventricular Conduction 388Inherited Causes of Bradycardia 389Non-Cardiac Causes of Bradycardia 390Evaluation 390Management 390Conclusions 391References 392

62 Atrial and Ventricular Ectopies 394Sabrina Tsao and Barbara J. DealPremature Atrial Contractions 394Clinical Presentation 394Evaluation 395Management 396Premature Ventricular Contractions 396Clinical Presentation 397Evaluation 397Management 397References 399

Part VI Special Issues in the Newborn 401

63 Balloon Atrial Septostomy 403Neil D. Patel and Damien KennyReferences 405

64 Interventional Therapeutic Procedures in the Newborn 406Salwa M. Gendi, Qi-Ling Cao, and Ziyad M. HijaziOpening of Atrial Communication 407

Atrial Septostomy 407Blade Atrial Septostomy and Static Balloon Dilation 408

Atrial Septal Stenting 408Transcatheter Balloon Dilation of Cardiac Valves 408

Pulmonary Valvuloplasty 408Aortic Valvuloplasty 409Mitral Balloon Valvuloplasty 411

Balloon Angioplasty and/or Stent Placement 411

Contents xix

Native Coarctation of the Aorta and Recoarctation 411Pulmonary Artery Stenosis 413Systemic and Pulmonary Veins Balloon Angioplasty 413

PDA Stenting 413Transcatheter Vascular Occlusion 413

PDA Occlusion 413Aortopulmonary Collateral Vessels 413

Closure of Intracardiac Communications (ASD, VSD) 415References 418

65 The Hybrid Procedure 419Ralf J. HolzerHybrid Palliation of Hypoplastic Left Heart Syndrome 419

Background 419Stage I Hybrid Palliation 419

Creating a Non-Restrictive Atrial Communication 419The Interstage Period 421

Perventricular Closure of Ventricular Septal Defects 426Hybrid Balloon Aortic or Pulmonary Valvuloplasty 428References 429

66 Neonatal Cardiac Surgical Procedures 430Harold M. BurkhartHypoplastic Left Heart Syndrome (Figure 66.1) 430

Procedure: Norwood Procedure with Sano Modification (Figures 66.2, 66.3,and 66.4) 431

Transposition of the Great Arteries (Figure 66.5) 432Surgical Correction (Figures 66.6, 66.7, and 66.8) 432Procedure 433

Systemic to Pulmonary Artery Shunt (Figure 66.9) 434Procedure 434

Neonatal Repair of Coarctation 434Extended End-To-End Resection and Anastomosis (Figures 66.10, 66.11, 66.12, and 66.13) 435

Total Anomalous Pulmonary Venous Connection 436Surgical Correction of Supracardiac TAPVC (Figures 66.14, 66.15, and 66.16) 436Infracardiac TAPVC (Figures 66.17, 66.18, and 66.19) 438

Interrupted Aortic Arch (Figures 66.20 and 66.21) 438Truncus Arteriosus (Figures 66.22, 66.23, 66.24, and 66.25) 439Patent Ductus Arteriosus (Figures 66.26 and 66.27) 440

Indications 440Procedure 442

67 Extracorporeal Membrane Oxygenation and Ventricular Assist Devices 443Vikas Sharma, Gregory J. Schears, and Joseph A. DearaniIndications for Circulatory Support 443Types of Mechanical Circulatory Support Devices 443

Cannulation Techniques and Circuit Designs 445Management During Circulatory Support 445

Anticoagulation 446Nutrition and Fluid/Electrolyte Management 446Complications 448

Experience with Berlin Heart EXCOR 448Conclusions 449The Future 449References 449

xx Contents

68 Neonatal Cardiac Transplantation 451Stephen Pophal, Justin Ryan, and John J. NigroHistory 451Demographics of Transplant 451Epidemiology 451Post-Transplant Survival 451Quality of Life 453Immunosuppression 453Morbidity 453Waitlist Mortality 453Benefits 454Transplantation and Hypoplastic Left Heart Syndrome 454Heart Transplant Technique 454Conclusions 454References 454

69 Postoperative Care of the Newborn 456Anthony F. Rossi and Enrique Oliver AregullinGeneral Principles 456Inotropic Support of the Postoperative Patient 463Mechanical Support of the Failing Heart After Surgery 464Sedation, Analgesia, and Neuromuscular Blockade 465Postoperative Arrhythmias 465Special Considerations 466

Patients With Pulmonary Hypertension 466Preterm or Low Birth Weight Children 466

Issues on Postoperative Care in the Newborn for Specific Lesions (see also chapters on specific cardiacdefects) 467

Surgery for Hypoplastic Left Heart Syndrome 467Timing of Surgery 467Norwood Operation 467Sano Modification 468Hybrid Procedure (see also Chapter 65) 468Heart Transplantation (see also Chapter 68) 468

Surgery for Complete Transposition of the Great Arteries 469Timing of Surgery 469

Neonatal Tetralogy of Fallot Repair 469Timing of Surgery 469

Part VII Neonatal Formulary 471

70 Drugs 473Enrique Oliver Aregullin and Anthony F. RossiFurther Reading 482

71 Nutrition 483Nicole SuttonStress Response to Surgery or Shock 483Metabolic Needs in Uncorrected CHD 483Feeding 484References 487

Index 489

xxi

Preface

Majority of cardiac defects manifest themselves in theneonate. The uniqueness of their cardiovascular issuesand the complexities of the spectra of cardiac defectsin this age group necessitate that this teaching mediumbe made available. This book is the product of thecumulative efforts of 103 pediatric cardiologists, car-diac surgeons, pathologists, radiologists, sonographers,scientists, and others who are authorities in the care ofnewborns with heart disease.

This book discusses all aspects of neonatal heartdisease in a comprehensive, clear, and succinct way.Each section will be valuable, not only for its textualcontent but also for the use of figures, charts, plates,graphs, illustrations, and tables. The use of these visualaids will make it easier for the reader to understand thecorresponding topic. In many sections, the written textmay be juxtaposed with illustrations, photographs ofa patient, chest roentgenograms, electrocardiograms,echocardiograms, angiograms, computed tomography,magnetic resonance imaging, pathologic specimens, andother relevant visual aids.

The 71 chapters have been grouped into seven majorparts. The first part, Prenatal and Perinatal Issues,includes new principles in cardiac embryogenesis andembryopathy and topics on the fetal heart and howthey manifest in the neonatal heart. The second part,General Neonatal Issues, includes epidemiology, tran-sitional circulation, approach to history-taking andphysical examination of the newborn suspected tohave cardiac disease, and discussions on the common

manifestations – cyanosis, tachypnea, hypoperfusion,and dysmorphism. The third part, Diagnostic Proce-dures, has seven chapters that discuss the various toolsfor an accurate and comprehensive diagnosis of neonatalheart disease. The fourth part, Specific MorphologicConditions, comprises 41 chapters that provide compre-hensive discussions on the various cardiac defects thatare made easy to understand through generous use offigures and tables. The fifth part, Rhythm Disturbancesin the Newborn, provides a four-chapter discussion ofneonatal rhythm disturbances, their presentation, eval-uation, and management. The sixth part, Special Issuesin the Newborn, includes important topics presentedgenerously with visual aids. These are balloon atrialseptostomy, interventional therapeutic procedures, theHybrid procedure, neonatal cardiac surgical procedures,extracorporeal membrane oxygenation, ventricular assistdevice, cardiac transplantation, and postoperative care.The seventh and last part, Neonatal Formulary, detailsthe various cardiac medications used in the neonatal agegroup as well as provides guidance for nutritional care.

This book will hopefully be a major resource for pedi-atric cardiologists, neonatologists, residents, fellows,sonographers, nurses, and other allied professionals whotake care of newborns with heart disease.

Ernerio T. AlbolirasZiyad M. HijaziLeo LopezDonald J. Hagler

xxiii

List of Contributors

Ra-Id Abdulla, MDProfessorDepartment of PediatricsChief, Section of Pediatric CardiologyRush University Medical CenterChicago, IL, USA

Michael J. Ackerman, MD, PhDProfessor in Medicine, Pediatrics, and PharmacologyWindland Smith Rice Sudden Death GenomicsLaboratoryMayo ClinicRochester MN, USA

Hitesh Agrawal, MDFellow in Pediatric CardiologyTexas Children’s HospitalHouston, TX, USA

Ernerio T. Alboliras, MDMedical DirectorGenus Heart CenterScottsdale, AZ, USA

Zahid Amin, MDProfessor and ChiefDivision of Pediatric CardiologyChildren’s Hospital of GeorgiaAugusta UniversityAugusta, GA, USA

Robert H. Anderson, MDHonorary Visiting ProfessorInstitute of Genetic MedicineNewcastle University;Division of Biomedical SciencesSt George’s, University of LondonLondon, UK

Enrique Oliver Aregullin, MDPediatric CardiologistHelen DeVos Children’s Hospital (Congenital HeartCenter CVTS)Grand Rapids, MI, USA

Sawsan Awad, MDAssociate ProfessorSection of Pediatric CardiologyRush University Medical CenterChicago, IL, USA

Carl L. Backer, MDDivision of Cardiovascular-Thoracic SurgeryAnn & Robert H. Lurie Children’s Hospital of Chicago;Department of SurgeryNorthwestern University Feinberg School of MedicineChicago, IL, USA

Sowmya Balasubramanian, MD, MScClinical Assistant ProfessorDivision of CardiologyDepartment of PediatricsStanford Medical SchoolStanford, CA, USA

Piers C.A. Barker, MDProfessor of Pediatrics and Obstetricsand GynecologyDivision of Pediatric CardiologyDuke University Medical CenterDurham, NC, USA

Sergio Bartakian, MDAssistant Professor of Pediatrics;Director of Pediatric and Congenital CardiacCatheterization LaboratoryDivision of Pediatric CardiologyUniversity of Texas at San AntonioSan Antonio, TX, USA

Aaron Bell, MDDepartment of Paediatric CardiologyEvelina Children’s HospitalLondon, UK

Hannah Bellsham-Revell, MDDepartment of Paediatric CardiologyEvelina Children’s HospitalLondon, UK

xxiv List of Contributors

Darren P. Berman, MDCo-Director of Cardiac Catheterization andInterventional TherapyDivision of CardiologyNationwide Children’s HospitalColumbus, OH, USA

Rebecca S. Beroukhim, MDDirector of Pediatric Echocardiography and FetalCardiologyMassachusetts General Hospital for ChildrenBoston, MA, USA

Deepti Bhat, MDPediatric CardiologistCardon Children’s HospitalMesa, AZ, USA

John W. Bokowski, PhDInstructor, Section of Pediatric CardiologyRush Center for Congenital and Structural Heart DiseaseChicago, IL, USA

William L. Border, MBChBDirector of Noninvasive Cardiac ImagingChildren’s Healthcare of Atlanta Sibley Heart Center;Associate Professor of PediatricsEmory University School of MedicineAtlanta, GA, USA

J. Martijn Bos, MD, PhDAssistant Professor in PediatricsWindland Smith Rice Sudden Death GenomicsLaboratoryMayo ClinicRochester, MN, USA

Jessica Bowman, MDPediatric CardiologistNationwide Children’s HospitalColumbus, OH, USA

David W. Brown, MDPediatric Cardiologist, Director of Clinical TrainingProgramDepartment of CardiologyBoston Children’s Hospital;Associate Professor of PediatricsHarvard Medical SchoolBoston, MA, USA

Nigel A. Brown, MDProfessorDivision of Biomedical SciencesSt George’s, University of LondonLondon, UK

Sujatha Budde, MD, MSPediatric CardiologistSeattle Children’s Hospital;Assistant ProfessorDepartment of PediatricsUniversity of Washington School of MedicineSeattle, WA, USA

Harold M. Burkhart, MDProfessor of Surgery and ChiefDivision of Cardiovascular and Thoracic SurgeryUniversity Health Sciences CenterOklahoma City, OK, USA

Allison K. Cabalka, MDProfessor of PediatricsDivision of Pediatric CardiologyMayo ClinicRochester, MN, USA

Bryan Cannon, MDAssociate Professor of Pediatrics;Director, Pediatric Arrhythmia and Pacing ServiceMayo ClinicRochester, MN, USA

Qi-Ling Cao, MDMedical Director Echo and Research LaboratorySidra Cardiovascular Center of ExcellenceSidra Medical and Research CenterDoha, Qatar

Frank Cetta, MDProfessor of Medicine and PediatricsDivision of Pediatric CardiologyMayo ClinicRochester, MN, USA

Sarah Chambers Gurson, MDPediatric Cardiology Associates PCFairfax, VA, USA

Grace Choi, MDAnn & Robert H. Lurie Children’s Hospital of ChicagoNorthwestern University Feinberg School of MedicineChicago, IL, USA

Nadine Choueiter, MDAssistant Professor in PediatricsAlbert Einstein College of Medicine;Director of Non-Invasive ImagingChildren’s Hospital at MontefioreBronx, NY, USA

List of Contributors xxv

Meryl S. Cohen, MDAttending Cardiologist, Professor of PediatricsPerelman School of Medicine at the University ofPennsylvania;Director, Cardiology Fellowship Training ProgramThe Children’s Hospital of PhiladelphiaPhiladelphia, PA, USA

Timothy M. Cordes, MDDirector of Pediatric Echocardiography Laboratory;Associate Professor of PediatricsRiley Children’s Hospital,Indiana University School of MedicineIndianapolis, IN, USA

Clifford L. Cua, MDPediatric CardiologistNationwide Children’s HospitalColumbus, OH, USA

Barbara J. Deal, MDDivision Head, CardiologyAnn & Robert H. Lurie Children’s Hospital;Getz Professor of CardiologyNorthwestern University Feinberg School of MedicineChicago, IL, USA

Joseph A. Dearani, MDProfessor of SurgeryDivision of Cardiovascular SurgeryMayo ClinicRochester, MN, USA

Karim A. Diab, MDAssociate ProfessorDirector of Echocardiography LaboratoryRush Center for Congenital Heart DiseaseChicago, IL, USA

Adam L. Dorfman, MDProfessor of PediatricsDivision of Pediatric CardiologyUniversity of Michigan Congenital Heart CenterC.S. Mott Children’s HospitalAnn Arbor, MI, USA

Howaida El-Said, MD, PhDDirector of the Cardiac Catheterization LaboratoryRady Children’s Hospital, San Diego;Clinical Professor of Pediatrics at UC San DiegoSan Diego, CA, USA

Osama Eltayeb, MDAssistant Professor of SurgeryDepartment of Surgery, Northwestern UniversityFeinberg School of Medicine;Division of Cardiovascular-Thoracic SurgeryAnn & Robert H. Lurie Children’s Hospital of ChicagoChicago, IL, USA

Lowell Frank, MDAttending Cardiologist and Director of CardiologyFellowship Training ProgramChildren’s National Medical Center;Assistant Professor of PediatricsGeorge Washington University School of MedicineWashington, DC, USA

Mark K. Friedberg, MDAssistant Professor, Department of PediatricsUniversity of Toronto;Co-Director Echo Research LaboratoryDivision of Pediatric CardiologyThe Labatt Family Heart CenterHospital for Sick ChildrenToronto, Canada

Miwa K. Geiger, MDAssistant Professor, PediatricsDivision of Pediatric Cardiology;Director, Fetal Heart ProgramMount Sinai HospitalNY, USA

Salwa Gendi, MDAssistant Professor of PediatricsUniversity of Mississippi Medical CenterJackson, MS, USA

Tal Geva, MDCardiologist-in-ChiefDepartment of CardiologyBoston Children’s Hospital;Professor of PediatricsHarvard Medical SchoolBoston, MA, USA

Donald J. Hagler, MDProfessor of Pediatrics and MedicineDivision of Pediatric CardiologyMayo ClinicRochester, MN, USA

Denise A. Hayes, MDAssistant ProfessorHofstra Northwell School of Medicine;Pediatric CardiologistCohen Children’s Medical CenterQueens, NY, USA

Camden L. Hebson, MDAssistant Professor of MedicineDivision of CardiologyDepartment of MedicineEmory University School of MedicineAtlanta, GA, USA

xxvi List of Contributors

Ziyad M. Hijazi, MD, MPHActing Chief Medical Officer - Chair of the Departmentof Pediatrics & DirectorSidra Cardiovascular Center of ExcellenceDoha, Qatar

Ralf J. Holzer, MDChief, Division of Pediatric CardiologyNew York-Presbyterian/Weill Cornell Medical Center;Director of Pediatric Cardiac CatheterizationThe Komansky Children’s HospitalNew York, NY, USA

Lisa Hornberger, MDProfessor of Pediatrics and Obstetrics and GynecologyUniversity of Alberta;Director of Fetal and Neonatal Cardiology Program,Section Head of Pediatric EchocardiographyStollery Children’s HospitalEdmonton, Alberta, Canada

James C. Huhta, MDPediatric Cardiology AssociatesSt. Petersburg, FL, USA;Professor of Pediatrics, Adjunct ProfessorInstitute of Clinical MedicineUniversity of Tromso, Norway

Darren Hutchinson, MBBSPaediatric and Fetal CardiologistDepartment of CardiologyRoyal Children’s Hospital MelbourneVictoria, Australia

Amish Jain, MDDivision of NeonatologyDepartment of PediatricsMount Sinai Hospital;Department of PediatricsUniversity of TorontoToronto, Canada

Jonathan N. Johnson, MDAssistant Professor of PediatricsDivision of Pediatric CardiologyMayo ClinicRochester, MN, USA

Supaluck Kanjanauthai, MDAdvocate Heart Institute for ChildrenDepartment of Pediatric CardiologyAdvocate Children’s HospitalOak Lawn CampusOak Lawn, IL, USA

Deepak Kaura, MDExecutive ChairFoundation Medical ServicesSidra Medical and Research CenterDoha, Qatar

Damien Kenny, MB, MDPediatric CardiologistOur Lady’s Children’s HospitalCrumlin, Dublin, Ireland

H. Helen Ko, BSTechnical Director/Operations Manager of PediatricEchocardiographyDivision of Pediatric CardiologyMount Sinai Medical CenterNew York, NY, USA

Peter Koenig, MDAnn & Robert H. Lurie Children’s Hospital of ChicagoNorthwestern University Feinberg School of MedicineChicago, IL, USA

Wyman W. Lai, MD, MPHMedical Director, Echocardiography and Co-MedicalDirectorHeart Institute, Children’s Hospital of Orange CountyOrange, CA, USA

Leo Lopez, MDDirector of Cardiac Non-Invasive ImagingNicklaus Children’s Hospital;Clinical Professor of PediatricsHerbert Wertheim College of MedicineFlorida International UniversityMiami, FL, USA

Irene D. Lytrivi, MDAssociate Professor of PediatricsDivision of Pediatric CardiologyMount Sinai Medical CenterNew York, NY, USA

Kiran K. Mallula, MDAssistant Professor of Clinical PediatricsDivision of CardiologyLouisiana State University School of Medicine;Interventional CardiologistChildren’s Hospital of New OrleansNew Orleans, LA, USA

Samer Masri, MDPediatric CardiologistAmerican University of BeirutBeirut, Lebanon

List of Contributors xxvii

Marie McDonald, MDProfessor of PediatricsDivision of Medical GeneticsDepartment of PediatricsDuke University Medical CenterDurham, NC, USA

Timothy J. Mohun, PhDDevelopmental Biology DivisionThe Francis Crick Institute Mill Hill LaboratoryLondon, UK

Michael C. Mongé, MDDivision of Cardiovascular-Thoracic SurgeryAnn & Robert H. Lurie Children’s Hospital of ChicagoDepartment of SurgeryNorthwestern University Feinberg School of MedicineChicago, IL, USA

Anita J. Moon-Grady, MDProfessor, Clinical Pediatrics;Director, Fetal Cardiovascular ProgramUniversity of California at San FranciscoSan Francisco, CA, USA

Gareth J. Morgan, MDPediatric CardiologistChildren’s Hospital of Colorado;Associate Professor of PediatricsUniversity of Colorado School of MedicineAurora, CO, USA

Shaine A. Morris, MD, MPHPediatric Cardiology and Cardiac Non-Invasive ImagingTexas Children’s Hospital;Assistant Professsor – Pediatrics-CardiologyBaylor College of MedicineHouston, TX, USA

Brieann Muller, MDAssistant Professor of PediatricsSection of Pediatric CardiologyRush University Medical CenterChicago, IL, USA

John J. Nigro, MDChief of Cardiac SurgeryRady Children’s Hospital;Director, Rady Children’s Heart InstituteRady Children’s Hospital-San DiegoSan Diego, CA, USA

Patrick W. O’Leary, MDConsultant, Division of Pediatric Cardiology;Professor of Pediatrics, College of MedicineMayo ClinicRochester, MN, USA

Neil D. Patel, MDFellow in Pediatric CardiologyChildren’s Hospital of Los AngelesLos Angeles, CA, USA

Andrada R. Popescu, MDDivision of Cardiovascular-Thoracic SurgeryAnn & Robert H. Lurie Children’s Hospital of Chicago;Assistant Professor of RadiologyDepartment of SurgeryNorthwestern University Feinberg School of MedicineChicago, IL, USA

Stephen Pophal, MDDivision of CardiologyPhoenix Children’s HospitalPhoenix, AZ, USA

Rajesh Punn, MDClinical Assistant ProfessorDivision of Pediatric CardiologyDepartment of PediatricsStanford Medical SchoolStanford, CA, USA

Robert Puntel, MDDivision of CardiologyPhoenix Children’s HospitalPhoenix, AZ, USA

Michael D. Quartermain, MDDivision of CardiologyThe Children’s Hospital of Philadelphia and PerelmanSchool of MedicineUniversity of PennsylvaniaPhiladelphia, PA, USA

Muhammad Yasir Qureshi, MBBSDivision of Pediatric CardiologyMayo ClinicRochester, MN, USA

Shakeel A. Qureshi, MDProfessor and Consultant Paediatric CardiologistEvelina London Children’s HospitalLondon, UK

Randy Richardson, MDDepartment of RadiologySt. Joseph’s Hospital and Medical CenterCreighton University School of MedicinePhoenix, AZ, USA

Anthony F. Rossi, MDDirector of Cardiac Critical CareThe Heart ProgramNicklaus Children’s HospitalMiami, FL, USA

xxviii List of Contributors

Hyde M. Russell, MDDepartment of SurgeryNorthwestern University Feinberg School of MedicineDivision of Cardiovascular-Thoracic SurgeryAnn & Robert H. Lurie Children’s Hospital of ChicagoChicago, IL, USA

Justin Ryan, PhDDivision of CardiologyPhoenix Children’s HospitalPhoenix, AZ, USA

Sameh M. Said, MDInstructor of SurgeryDivision of Cardiovascular SurgeryMayo ClinicRochester, MN, USA

Vikas Sharma, MBBSAssistant Professor of Surgery, Thoracic and CardiacSurgeryUniversity of Utah Health Care-Hospital and ClinicsSalt Lake City, UT, USA

Gregory J. Schears, MDProfessor of AnesthesiologyMayo ClinicRochester, MN, USA

Ira Shetty, MDDepartment of Pediatric CardiologyAdvocate Heart Institute for ChildrenAdvocate Children’s HospitalOak Lawn CampusOak Lawn, IL, USA

Timothy C. Slesnick, MDDirector of Pediatric Cardiac Magnetic ResonanceImaging;Pediatric Cardiologist, Sibley Heart CenterChildren’s Healthcare of Atlanta;Associate Professor of PediatricsEmory UniversityAtlanta, GA, USA

Shubhika Srivastava, MBBSProfessor of Pediatrics;Director of Echocardiography LaboratoryDivision of Pediatric CardiologyMount Sinai Medical CenterNew York, NY, USA

Nicole Sutton, MDAssistant Professor of PediatricsAlbert Einsein College of Medicine;Pediatric CardiologistChildren’s Hospital at MontefioreBronx, NY, USA

Nathaniel W. Taggart, MDAssistant Professor of PediatricsDivision of Pediatric CardiologyMayo ClinicRochester, MN, USAGregory H. Tatum, MDAssociate Professor of PediatricsDivision of Pediatric CardiologyDuke University Medical CenterDurham, NC, USADarshit Thakrar, MDAssistant Professor of RadiologyUniversity of PittsburghPittsburgh, PA, USASabrina Tsao, MBBSDirector, ElectrophysiologyDivision of CardiologyAnn & Robert H. Lurie Children’s Hospital;Assistant Professor of Pediatrics (Cardiology)Feinberg School of MedicineNorthwestern University Feinberg School of MedicineChicago, IL, USAGregory Webster, MD, MPHDirector, Electrocardiogram Laboratory, CardiologyAnn & Robert H. Lurie Children’s Hospital;Assistant Professor of PediatricsNorthwestern University Feinberg School of MedicineChicago, IL, USAStephanie Burns Wechsler, MDAssociate Professor of PediatricsDivisions of Pediatric Cardiology and Medical GeneticsDuke University Medical CenterDurham, NC, USAMark Wylam, MDAssociate Professor of Pediatrics and MedicineDivision of Pediatric Pulmonology and Critical CareMedicineDepartment of Pediatric and Adolescent MedicineMayo ClinicRochester, MN, USAEvan M. Zahn, MDDirectorCongenital Heart Program and Division of PediatricCardiologyCedars-Sinai Medical CenterLos Angeles, CA, USAMark V. Zilberman, MDDirector, Pediatric Echocardiology and Fetal Cardiology;Pediatric Cardiologist, Boston Floating Children’sHospital;Associate Professor of PediatricsTufts University School of MedicineBoston, MA, USA