Common Cardiac Issues in Pediatrics

Common Cardiac Issues in Pediatrics
Common Cardiac
Issues in Pediatrics
Common
Editors
Jonathan N. Johnson, MD, FACC, FAAP, and
Cardiac Issues
in Pediatrics
Deepak M. Kamat, MD, PhD, FAAP
Learn from leading experts the latest information on cardiac

issues that present most often in a pediatric office. The
scope of focus includes signs and symptoms, management
of patients with acquired or congenital heart disease, cardiac
implications of common infectious diseases, preventive
cardiology, and other issues that are frequently managed or
evaluated by the primary care pediatrician.
Topics include
• Electrocardiography
• Heart Murmur
• Syncope
• Cardiomyopathies and Channelopathies
• Rheumatic Heart Disease
• Endocarditis
JOHNSON ∞ KAMAT
• Cardiac Pharmacology
• Transition From Pediatric to Adult-Centered
Cardiac Care
Editors
For other pediatric resources, visit the
Jonathan N. Johnson, MD, FACC, FAAP
American Academy of Pediatrics
at shop.aap.org. Deepak M. Kamat, MD, PhD, FAAP
ISBN 978-1-61002-144-9
90000>
From Common Cardiac Issues in Pediatrics: , 9 781610 021449 From Common Cardiac Issues in Pediatrics: ,
Johnson, Jonathan N.;Kamat, Deepak M. AAP Johnson, Jonathan N.;Kamat, Deepak M.
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Common
Cardiac Issues
in Pediatrics
Editors
Deepak M. Kamat, MD, PhD, FAAP
From Common Cardiac Issues in Pediatrics: ,

Johnson, Jonathan N.;Kamat, Deepak M.
Downloaded on May 14, 2021, 6:04 PM at 185.89.158.94
Published by AAP, 2018. All rights reserved.
CCIP.indb 1 3/13/18 4:18 PM

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COMMON CARDIAC ISSUES IN PEDIATRICS
Editors
Jonathan N. Johnson, MD, FACC, FAAP Deepak M. Kamat, MD, PhD, FAAP
Associate Professor of Pediatrics Professor of Pediatrics
Chair, Division of Pediatric Cardiology Vice Chair for Education
Department of Pediatric and Adolescent The Carman and Ann Adams Department
Medicine of Pediatrics
Mayo Clinic Wayne State University
Rochester, MN Designated Institutional Official
Children’s Hospital of Michigan
Detroit, MI
Contributing Authors
Sanjeev Aggarwal Heather Anderson, MD, FAAP
Professor of Pediatrics Fellow
Division of Pediatric Cardiology Division of Pediatric Cardiology
Children’s Hospital of Michigan Mayo Clinic
Wayne State University Rochester, MN
Detroit, MI Ch 19: Congenital Heart Lesions
Ch 8: Chest Pain Kristin Anton, RN, MSM, CPNP-AC
Huzaifa Ahmad, MBBS Children’s Health
MBBS Class of 2018 Dallas, TX
The Aga Khan University Ch 44: Pulmonary Hypertension
Karachi, Pakistan Neha Bansal, MD
Ch 14: Neonatal Screening for Heart Disease Fellow Pediatric Cardiology
Kiona Y. Allen, MD Children’s Hospital of Michigan
Assistant Professor of Pediatrics Detroit, MI
Northwestern University Feinberg School Ch 8: Chest Pain
of Medicine Dianna M. E. Bardo, MD, FSCCT, FNASCI
Ann and Robert H. Lurie Children’s Hospital Director, Body MRI
of Chicago Co-Director, 3D Innovation Lab
Chicago, IL Department of Medical Imaging
Ch 39: Neurodevelopmental and Psychosocial Phoenix Children’s Hospital
Outcomes in Children With Congenital Clinical Professor of Radiology and
Heart Disease Child Health
Carolyn A. Altman, MD, FAAP University of Arizona
Associate Chief, Pediatric Cardiology Phoenix, AZ
Professor, Pediatrics Ch 5: Chest Radiography
Baylor College of Medicine Sarosh P. Batlivala, MD, MSCI, FAAP
Texas Children’s Hospital Associate Professor, Pediatric Cardiology
Houston, TX Associate Program Director, Pediatric
Ch 31: Prevention of Bacterial Endocarditis Cardiology Fellowship
Ch 33: Pericardial Diseases Director, Pediatric Fellowship Training
Ahdi Amer, MD Programs
Associate Professor of Pediatrics The Children’s Heart Center
The Carman and Ann Adams Department Blair E. Batson Hospital for Children
of Pediatrics University of Mississippi Medical Center
Wayne State University School of Medicine Jackson, MS
Detroit, MI Ch 19: Congenital Heart Lesions
Ch 28: Vaccines for Patients With Cardiac
Conditions
Published by AAP, 2018. All rights reserved. iii
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Allison K. Black, MD Kanupriya Chaturvedi, MD, FAAP

Pediatric Cardiology Fellow Fellow, Pediatric Cardiology
University of Pittsburgh The Congenital Heart Collaborative
Pittsburgh, PA University Hospitals, Rainbow Babies and
Ch 38: Autonomic Dysfunction Children’s Hospital
Jessica Bowman, MD Cleveland, OH
Attending Pediatric Cardiologist Ch 36: Cardiac Screening Prior to ADHD
Nationwide Children’s Hospital Treatment
Assistant Professor of Pediatrics Sharon Chen, MD, MPH
The Ohio State University Clinical Assistant Professor
Columbus, OH Pediatric Heart Failure and Transplantation
Ch 40: Lifestyle Counseling Lucile Packard Children’s Hospital at Stanford
Harold M. Burkhart, MD University
Professor and Chief Palo Alto, CA
Department of Surgery Ch 43: Cardiac Transplantation
Division of Thoracic and Cardiovascular Surgery Devyani Chowdhury, MD, FAAP, FACC
University of Oklahoma Health Sciences Center Director, Cardiology Care for Children
Oklahoma City, OK Affiliate Faculty Children’s Hospital
Ch 16: Office Care of the Child After Cardiac of Philadelphia
Surgery Lancaster, PA
Ryan Butts, MD Ch 14: Neonatal Screening for Heart Disease
Assistant Professor of Pediatrics Jason Cole, MD
Division of Pediatric Cardiology PGY-3 Pediatric Resident
University of Texas Southwestern UN Nationwide Children’s Hospital/Ohio State
Dallas, TX University
Ch 32: Myocarditis Columbus, OH
Michael U. Callaghan, MD Ch 40: Lifestyle Counseling
Associate Professor of Pediatrics Michael Colon, MD
Children’s Hospital of Michigan Clinical Associate Professor of Pediatrics
Detroit, MI Division of Pediatric Cardiology
Ch 27: Cardiotoxicity Among Survivors of Albany Medical College
Childhood Cancer Albany, NY
Bryan Cannon, MD, FACC, FHRS, FAAP Ch 29: Lyme Carditis
Director, Pediatric Electrophysiology John Colquitt, MD, FAAP
Director, Pediatric Cardiology Fellowship Assistant Professor of Pediatrics
Program Division of Pediatric Cardiology
Associate Professor of Pediatrics Baylor College of Medicine
Mayo Clinic Texas Children’s Hospital
Rochester, MN Houston, TX
Ch 37: Cardiac Screening for Athletic Participation Ch 31: Prevention of Bacterial Endocarditis
Chesney Castleberry, MD Bibhuti B. Das, MD, FAAP, FACC
Assistant Professor, Pediatric Cardiology Pediatric Heart Failure and Transplant
School of Medicine, Washington University in Cardiologist
St Louis Joe DiMaggio Children’s Hospital
St Louis Children’s Hospital Memorial Health Care
St Louis, MO Hollywood, FL
Ch 22: Restrictive Cardiomyopathy Ch 44: Pulmonary Hypertension

Published byivAAP, 2018. All rights reserved.
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Pirooz Eghtesady, MD, PhD, FAAP Julie Glickstein, MD, FACC, FAAP
Professor and Emerson Chair Professor of Pediatrics at Columbia University
Department of Surgery Medical Center
Chief, Pediatric Cardiac Surgery Director, Pediatric Cardiology Fellowship
Washington University in St Louis Program
Co-Director of the Heart Center Department of Pediatrics/Division of Pediatric
St Louis Children’s Hospital Cardiology
St Louis, MO Morgan Stanley Children’s Hospital of New
Ch 15: Surgical Procedures for Congenital York Presbyterian
Heart Disease New York, NY
Noelle Andrea V. Fabie, MD Ch 12: Evaluation of the Neonate With Congenital
Medical Biochemical Fellow Heart Disease
Division of Genetic, Genomic, and Metabolic David J. Goldberg, MD
Disorders Assistant Professor
Children’s Hospital of Michigan Department of Pediatrics
Detroit, MI The Perelman School of Medicine at The
Ch 17: Common Syndromes Associated With University of Pennsylvania
Cardiac Lesions Division of Cardiology
Swati Garekar, BC The Children’s Hospital of Philadelphia
Director, Pediatric Cardiology Program Philadelphia, PA
Fortis Hospital, Mulund Ch 7: Laboratory Studies
Mumbai, India Pooja Gupta, MD, FASE, FACC
Ch 26: Acute Rheumatic Fever and Rheumatic Assistant Professor of Pediatrics
Heart Disease Division of Pediatric Cardiology
Bessey Geevarghese, DO Children’s Hospital of Michigan
Assistant Professor Wayne State University
Northwestern University Feinberg School Detroit, MI
of Medicine Ch 9: Syncope
Attending Physician, Division of Infectious Caitlin Haxel, MD
Disease Chief Fellow, Pediatric Cardiology
Ann and Robert H. Lurie Children’s Hospital Division of Pediatric Cardiology
of Chicago New York Presbyterian—Morgan Stanley
Chicago, IL Children’s Hospital
Ch 25: Kawasaki Disease Columbia University College of Physicians
Miwa Geiger, MD and Surgeons
Assistant Professor of Pediatrics New York, NY
Division of Pediatric Cardiology Ch 12: Evaluation of the Neonate With Congenital
Director, Fetal Heart Program Heart Disease
Icahn School of Medicine at Mount Sinai Gurumurthy Hiremath, MD, FACC
New York, NY Assistant Professor of Pediatrics
Ch 41: Cardiac Pharmacology Division of Pediatric Cardiology
Samuel S. Gidding, MD University of Minnesota
Cardiology Division Head Minneapolis, MN
Nemours Cardiac Center Ch 1: Clinical History and Physical Examination
Professor of Pediatrics Ch 11: Heart Murmur
Sidney Kimmel Medical College of Thomas Whitnee Hogan, MD
Jefferson University Pediatric Cardiology Fellow
Wilmington, DE Department of Pediatrics
Ch 34: Dyslipidemia University of Utah
Salt Lake City, UT
Ch 20: Hypertrophic Cardiomyopathy
Published by AAP, 2018. All rights reserved. v
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Kelley K. Hutchins, DO, MPH Sonya Kirmani, MD

Pediatric Hematology/Oncology Fellow Instructor, Pediatric Heart Failure/Transplant
Children’s Hospital of Michigan Division of Pediatric Cardiology
Detroit, MI Duke University Medical Center
Ch 27: Cardiotoxicity Among Survivors of Durham, NC
Childhood Cancer Ch 22: Restrictive Cardiomyopathy
Anitha S. John, MD, PhD Stacie Knutson, MD
Director, Washington Adult Congenital Assistant Professor of Pediatrics
Heart Program Division of Pediatric Cardiology
Children’s National Medical Center and University of Minnesota
Washington Hospital Center Minneapolis, MN
Assistant Professor of Pediatrics Ch 1: Clinical History and Physical Examination
George Washington University Ch 11: Heart Murmur
Washington, DC William Buck Kyle, MD, FAAP
Ch 18: Adults With Congenital Heart Disease Assistant Professor of Pediatrics
Jonathan N. Johnson, MD, FACC, FAAP Division of Pediatric Cardiology
Associate Professor of Pediatrics Baylor College of Medicine/Texas Children’s
Chair, Division of Pediatric Cardiology Hospital
Department of Pediatric and Adolescent Houston, TX
Medicine Ch 19: Congenital Heart Lesions
Mayo Clinic Timothy S. Lancaster, MD
Rochester, MN Fellow in Cardiothoracic Surgery
Ch 4: Basics of Exercise Testing Barnes-Jewish/St Louis Children’s Hospitals
Joyce Johnson, MD, MS, FAAP Washington University School of Medicine
Assistant Professor, Pediatric Cardiology St Louis, MO
Ann & Robert H. Lurie Children’s Hospital Ch 15: Surgical Procedures for Congenital
of Chicago Heart Disease
Northwestern University Feinberg School of Diego Lara, MD, MPH
Medicine Pediatric and Fetal Cardiology
Chicago, IL Ochsner Hospital for Children
Ch 19: Congenital Heart Lesions Senior Lecturer
Beth D. Kaufman, MD University of Queensland-Ochsner Clinical
Clinical Associate Professor of Pediatrics School
Director, Pediatric Cardiomyopathy Program New Orleans, LA
Lucile Packard Children’s Hospital, Stanford Ch 33: Pericardial Diseases
University Shannon Lyon, DO
Palo Alto, CA University of Louisville
Ch 43: Cardiac Transplantation Department of Pediatrics
Angela M. Kelle, MD, FACC, FAAP Louisville, KY
Assistant Professor of Pediatrics Ch 21: Dilated Cardiomyopathy
Penn State Children’s Hospital Nicolas L. Madsen, MD, MPH
Hershey, PA Medical Director, Inpatient Cardiology
Ch 3: The Role of Echocardiography Assistant Professor of Pediatrics
Steven J. Kindel, MD University of Cincinnati College of Medicine
Assistant Professor of Pediatrics Cincinnati Children’s Hospital Medical Center
Medical Director of Advanced Heart Failure Cincinnati, OH
and Heart Transplantation Ch 19: Congenital Heart Lesions
Herma Heart Center
Children’s Hospital of Wisconsin
Medical College of Wisconsin
Milwaukee, WI
From Common Cardiac
Ch 23: IssuesCardiomyopathy
Noncompaction in Pediatrics: ,
Published byviAAP, 2018. All rights reserved.
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Bradley S. Marino, MD, MPP, MSCE, FAAP Mark D. Norris, MD, MS

Professor Pediatrics and Medical Social Sciences Medical Co-Director Adult Congenital Heart
Northwestern University Feinberg School Program
of Medicine University of Michigan
Heart Center Co-Director, Research and Ann Arbor, MI
Academic Affairs Ch 42: Transition and Transfer From Pediatric to
Divisions of Cardiology and Critical Care Adult-Centered Cardiac Care
Medicine Todd T. Nowlen, MD, FACC
Ann & Robert H. Lurie Children’s Hospital Director Ambulatory Cardiology
of Chicago Phoenix Children’s Hospital
Chicago, IL Phoenix, AZ
Ch 39: Neurodevelopmental and Psychosocial Ch 5: Chest Radiography
Outcomes in Children With Congenital
Brandon Lane Phillips, MD, FAAP
Heart Disease
Department of Pediatrics
Daniel Mauriello, MD Ochsner Health Center for Children
Assistant Professor of Pediatrics Monroe, Ochsner Clinic Foundation
Division of Pediatric Cardiology West Monroe, LA
Johns Hopkins All Children’s Hospital Ch 1: Clinical History and Physical Examination
St Petersburg, FL
Adam Putschoegl, DO, FAAP
Ch 2: Electrocardiography
Pediatric Cardiology Fellow
Shaji C. Menon, MD, FACC, FASE, FAAP Division of Pediatric Cardiology
Associate Professor of Pediatrics Mayo Clinic
Adjunct Associate Professor of Radiology Rochester, MN
Medical Director, Single Ventricle Survivorship Ch 19: Congenital Heart Lesions
Program Ch 42: Transition and Transfer From Pediatric to
University of Utah Adult-Centered Cardiac Care
Salt Lake City, UT
Muhammad Yasir Qureshi, MBBS, FASE
Ch 6: Cardiac MR Imaging and CT
Senior Associate Consultant
James H. Moller, MD, FAAP Pediatric Cardiology
Professor Emeritus and Former Head of Assistant Professor of Pediatrics
Pediatrics Mayo Clinic College of Medicine
Adjunct Professor of Pediatrics Rochester, MN
Minneapolis, MN Ch 3: The Role of Echocardiography
Ch 13: Fetal and Newborn Transitional
Hari Rajagopal, MD
Circulations
Pediatric Cardiology Advanced Imaging Fellow
Brandon Morrical, MD, FAAP Division of Pediatric Cardiology
Instructor of Pediatrics Icahn School of Medicine at Mount Sinai
Division of Pediatric Cardiology New York, NY
Mayo Clinic Ch 41: Cardiac Pharmacology
Rochester, MN
Rini Sahewalla, MD
Ch 4: Basics of Exercise Testing
Fellow, Pediatric Cardiology
Kathleen A. Mussatto, PhD, RN Children’s Hospital of Michigan
Co-Director of Cardiac Research Detroit, MI
Associate Clinical Professor of Surgery Ch 17: Common Syndromes Associated With
Children’s Hospital of Wisconsin Cardiac Lesions
Milwaukee, WI
Amy H. Schultz, MD, MSCE, FACC, FAAP
Ch 39: Neurodevelopmental and Psychosocial
Attending Cardiologist
Outcomes in Children With Congenital
Seattle Children’s Hospital
Heart Disease
Associate Professor of Pediatrics
Division of Cardiology
From Common Cardiac Issues in Pediatrics: , University of Washington School of Medicine
Seattle, WA
Ch 30: Endocarditis
Published by AAP, 2018. All rights reserved. vii
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Kristen Sexson Tejtel, MD, PhD, MPH, Joshua D. Sparks, MD

FAAP Director, Pediatric Advanced Heart Failure
Assistant Professor of Pediatrics and Transplantation
Director, Center for Preventive Cardiology University of Louisville School of Medicine
Texas Children’s Hospital Norton Children’s Hospital
Baylor College of Medicine Louisville, KY
Houston, TX Ch 21: Dilated Cardiomyopathy
Ch 35: Hypertension Ch 38: Autonomic Dysfunction
Stanford T. Shulman, MD, FAAP Rachel Steury, MSN, CNP
Virginia H. Rogers Professor of Pediatric Washington, Adult Congenital Heart Program
Infectious Diseases Children’s National Health System
Northwestern University Feinberg School Washington, DC
of Medicine Ch 18: Adults With Congenital Heart Disease
Division Head Emeritus Infectious Diseases David W. Stockton, MD, FACMG
Ann and Robert H. Lurie Children’s Hospital Professor of Pediatrics and Internal Medicine
of Chicago Chief, Division of Genetic, Genomic and
Chicago, IL Metabolic Disorders
Ch 25: Kawasaki Disease Wayne State University and Children’s Hospital
Nikki M. Singh, MD of Michigan
Pediatric Cardiology Fellow Detroit, MI
Medical College of Wisconsin Ch 17: Common Syndromes Associated With
Children’s Hospital of Wisconsin Cardiac Lesions
Milwaukee, WI Alex J. Thompson, MD, FAAP
Ch 23: Noncompaction Cardiomyopathy Department of Pediatric and Adolescent
Lalitha Sivaswamy, MD Medicine
Associate Professor of Pediatrics and Neurology Division of Pediatric Cardiology
Carmen and Ann Adams Department Mayo Clinic
of Pediatrics Rochester, MN
Wayne State University School of Medicine Ch 19: Congenital Heart Lesions
Detroit, MI Jess Thompson, MD, MSc
Ch 9: Syncope Assistant Professor of Surgery
Melissa Smith-Parrish, MD Section of Congenital Heart Surgery
Assistant Professor of Pediatrics University of Oklahoma
Northwestern University Feinberg School Oklahoma City, OK
of Medicine Ch 16: Office Care of the Child After Cardiac
Chicago, IL Surgery
Ch 39: Neurodevelopmental and Psychosocial Bhadra Trivedi, MD
Outcomes in Children With Congenital Pediatrics, Fellow, Pediatric Cardiology
Heart Disease Department of Pediatric Cardiology
Brian S. Snarr, MD, PhD Fortis Child Heart Mission
Cardiology Fellow Fortis Hospital Mulund
Division of Cardiology Mumbai, India
The Children’s Hospital of Philadelphia Ch 26: Acute Rheumatic Fever and Rheumatic
Philadelphia, PA Heart Disease
Ch 7: Laboratory Studies Juan Villafañe, MD, FAAP
Christopher Snyder, MD, FAAP Professor of Pediatrics Cardiology
Ch 36: Cardiac Screening Prior to ADHD University of Kentucky
Treatment Lexington, KY
Ch 24: Long QT Syndrome and Other
Channelopathies

Published byviii
AAP, 2018. All rights reserved.
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Philip L. Wackel, MD Melissa Yamauchi, MD, MPH

Assistant Professor of Pediatrics University of Utah
Division of Pediatric Cardiology Salt Lake City, UT
Mayo Clinic College of Medicine Ch 6: Cardiac MR Imaging and CT
Rochester, MN
Ch 10: Palpitations and Arrhythmia
American Academy of
Josh Weldin, MD
Assistant Professor of Pediatrics
Pediatrics Reviewers
Division of Pediatric Cardiology Section on Cardiology and Cardiac Surgery
University of Washington School of Medicine Section on Clinical Pharmacology and
Seattle Children’s Hospital Therapeutics
Seattle, WA Section on Infectious Diseases
Ch 30: Endocarditis Section on Medicine—Pediatrics
Don P. Wilson, MD, FNLA

Diplomate, American Board of Clinical
Lipidology
Endowed Chair, Pediatric Cardiovascular
Health and Risk Prevention
Pediatric Endocrinology and Diabetes
Cook Children’s Medical Center
Fort Worth, TX
Ch 34: Dyslipidemia

Published by AAP, 2018. All rights reserved. ix
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Contents
Introduction.......................................................................................... xvii
Part 1: Evaluation of the Patient

1. Clinical History and Physical Examination.......................................... 3
Brandon Lane Phillips, MD, FAAP, Stacie Knutson, MD,
and Gurumurthy Hiremath, MD, FACC
2. Electrocardiography........................................................................... 13
Daniel Mauriello, MD
3. The Role of Echocardiography............................................................ 49
Angela M. Kelle, MD, FACC, FAAP, and Muhammad Yasir Qureshi,
MBBS, FASE
4. Basics of Exercise Testing................................................................... 71
Brandon Morrical, MD, FAAP, and Jonathan N. Johnson, MD,
FACC, FAAP
5. Chest Radiography............................................................................. 79
Todd T. Nowlen, MD, FACC, and Dianna M. E. Bardo,
MD, FSCCT, FNASCI
6. Cardiac MR Imaging and CT............................................................. 95
Melissa Yamauchi, MD, MPH, and Shaji C. Menon,
MD, FACC, FASE, FAAP
7. Laboratory Studies........................................................................... 109
Brian S. Snarr, MD, PhD, and David J. Goldberg, MD
Part 2: Common Signs and Symptoms

8. Chest Pain....................................................................................... 117
Neha Bansal, MD, and Sanjeev Aggarwal, MD
9. Syncope........................................................................................... 133
Lalitha Sivaswamy, MD, and Pooja Gupta, MD, FASE, FACC
10. Palpitations and Arrhythmia............................................................. 147
Philip L. Wackel, MD
11. Heart Murmur................................................................................. 161
Gurumurthy Hiremath, MD, FACC, and Stacie Knutson, MD
12. Evaluation of the Neonate With Congenital Heart Disease................ 171
Caitlin Haxel, MD, and Julie Glickstein, MD, FACC, FAAP
Published by AAP, 2018. All rights reserved. xi
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CONTENTS
Part 3: Congenital Heart Disease

13. Fetal and Newborn Transitional Circulations..................................... 187
James H. Moller, MD, FAAP
14. Neonatal Screening for Heart Disease............................................... 197
Huzaifa Ahmad, MBBS, and Devyani Chowdhury, MD, FAAP, FACC
15. Surgical Procedures for Congenital Heart Disease............................. 205
Timothy S. Lancaster, MD, and Pirooz Eghtesady, MD, PhD, FAAP
16. Office Care of the Child After Cardiac Surgery.................................. 237
Jess Thompson, MD, MSc, and Harold M. Burkhart, MD
17. Common Syndromes Associated With Cardiac Lesions..................... 245
Noelle Andrea Fabie, MD, Rini Sahewalla, MD, and
David W. Stockton, MD, FACMG
18. Adults With Congenital Heart Disease............................................. 269
Rachel Steury, MSN, CNP, and Anitha S. John, MD, PhD
19. Congenital Heart Lesions................................................................. 287
Aortic Valve Problems, Including Bicuspid Aortic Valve
and Subaortic Membranes................................................................ 287
William Buck Kyle, MD, FAAP
Atrial Septal Defects........................................................................ 292
Alex J. Thompson, MD, FAAP, and
Nicolas L. Madsen, MD, MPH
Atrioventricular Canal Defects......................................................... 296
Sarosh P. Batlivala, MD, MSCI, FAAP
Coarctation of the Aorta................................................................... 300
“Congenitally Corrected” Transposition of the Great Arteries............ 305
Mitral Valve Anomalies.................................................................... 308
Joyce Johnson, MD, MS, FAAP
Patent Ductus Arteriosus.................................................................. 311
Patent Foramen Ovale...................................................................... 314
Pulmonary Stenosis.......................................................................... 316
Heather Anderson, MD, FAAP, and
Nicolas Madsen,
From Common Cardiac Issues inMD, MPH, FAAP
Pediatrics: ,
Published byxiiAAP, 2018. All rights reserved.
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CONTENTS
Single-Ventricle Lesions................................................................... 320

Systemic and Pulmonary Vein Anomalies.......................................... 325
Tetralogy of Fallot............................................................................ 329
Nicolas L. Madsen, MD, MPH
Transposition of the Great Arteries................................................... 334
Tricuspid Valve Anomalies................................................................ 339
Truncus Arteriosus........................................................................... 343
Adam Putschoegl, DO, FAAP
Vascular Rings and Slings................................................................. 347
Ventricular Septal Defects................................................................ 353
Part 4: Cardiomyopathies and Channelopathies

20. Hypertrophic Cardiomyopathy......................................................... 361
Whitnee Hogan, MD, and Shaji C. Menon, MD, FACC,
FASE, FAAP
21. Dilated Cardiomyopathy.................................................................. 377
Shannon Lyon, DO, and Joshua D. Sparks, MD
22. Restrictive Cardiomyopathy.............................................................. 387
Chesney Castleberry, MD, and Sonya Kirmani, MD
23. Noncompaction Cardiomyopathy...................................................... 395
Nikki M. Singh, MD, and Steven J. Kindel, MD
24. Long QT Syndrome and Other Channelopathies.............................. 411
Juan Villafañe, MD, FAAP
Part 5: Acquired Cardiac Diseases

25. Kawasaki Disease............................................................................. 423
Bessey Geevarghese, DO, and Stanford T. Shulman, MD, FAAP
26. Acute Rheumatic Fever and Rheumatic Heart Disease....................... 443
Swati Garekar, BC, and Bhadra Trivedi, MD
27. Cardiotoxicity
From Common Cardiac IssuesAmong Survivors
in Pediatrics: , of Childhood Cancer....................... 463
KelleyN.;Kamat,
Johnson, Jonathan K. Hutchins, DO, MPH,
Deepak M. and Michael U. Callaghan, MD
Published by AAP, 2018. All rights reserved. xiii
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CONTENTS
Part 6: Infectious Diseases

28. Vaccines for Patients With Cardiac Conditions ................................. 477
Ahdi Amer, MD
29. Lyme Carditis.................................................................................. 487
Michael Colon, MD
30. Endocarditis.................................................................................... 497
Amy H. Schultz, MD, MSCE, FAAP, FACC, and Josh Weldin, MD
31. Prevention of Bacterial Endocarditis................................................. 511
John Colquitt, MD, FAAP, and Carolyn A. Altman, MD, FAAP
32. Myocarditis...................................................................................... 517
Ryan Butts, MD
33. Pericardial Diseases.......................................................................... 529
Diego Lara, MD, MPH, and Carolyn A. Altman, MD, FAAP
Part 7: Preventive Cardiology

34. Dyslipidemia.................................................................................... 545
Don P. Wilson, MD, FNLA, and Samuel S. Gidding, MD
35. Hypertension................................................................................... 567
S. Kristen Sexson Tejtel, MD, PhD, MPH, FAAP
36. Cardiac Screening Prior to ADHD Treatment................................... 595
Kanupriya Chaturvedi, MD, FAAP, and
Christopher Snyder, MD, FAAP
37. Cardiac Screening for Athletic Participation...................................... 603
Bryan Cannon, MD, FACC, FHRS
38. Autonomic D ysfunction................................................................... 613
Alison Black, MD, and Joshua D. Sparks, MD
Part 8: General Issues in Primary Cardiac Care

39. Neurodevelopmental and Psychosocial Outcomes in Children With
Congenital Heart Disease................................................................. 627
Kiona Y. Allen, MD, Melissa Smith-Parrish, MD, Kathleen A. Mussatto,
PhD, RN, and Bradley S. Marino, MD, MPP, MSCE, FAAP
40. Lifestyle Counseling......................................................................... 643
Jessica Bowman, MD, and Jason Cole, MD
41. Cardiac Pharmacology...................................................................... 655
Miwa Geiger,
From Common Cardiac IssuesMD, and Hari Rajagopal,
in Pediatrics: , MD
Published byxiv
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CONTENTS
42. Transition and Transfer From Pediatric to Adult-Centered

Cardiac Care.................................................................................... 685
Mark D. Norris, MD, MS, and Adam Putschoegl, DO, FAAP
Part 9: Special Conditions

43. Cardiac Transplantation................................................................... 701
Sharon Chen, MD, MPH, and Beth D. Kaufman, MD
44. Pulmonary Hypertension................................................................. 713
Kristin Anton, RN, MSN, CPNP-AC, and
Bibhuti B. Das, MD, FAAP, FACC
Index..................................................................................................... 727

Published by AAP, 2018. All rights reserved. xv
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Introduction
Though heart defects in children had been studied for many years, the field
of pediatric cardiology was essentially born in the 1930s, when Robert Gross
performed the first surgical ligation of a patent ductus arteriosus. Instead of
cardiac defects being a pathologic entity seen at autopsy, they became treatable.
Helen Taussig began training pediatricians in the new field of pediatric
cardiology in the late 1940s, and pediatric cardiology became the first sub-board
of pediatrics in 1961. The Section on Cardiology of the American Academy of
Pediatrics (AAP) was formed in 1955.
The field of pediatric cardiology has seen incredible advances in its short
period of existence. Cardiopulmonary bypass, used to repair intracardiac
defects, was first successfully used in children in 1954 and 1955. The first
echocardiograms, initially 1-dimensional, were reported in 1953, but
2-dimensional images of the moving heart were not available until the early
1970s. The arterial switch procedure, now the standard of care for most patients
with transposition of the great arteries, was first performed in 1975—only
43 years ago.
As these advances in pediatric cardiac care have occurred, the scope of
pediatric primary care exposure to patients with complex cardiac anatomy has
increased. This text was created specifically for the pediatric care professional
(pediatrician, family physician, nurse practitioner, physician assistant, and all
trainees and students) who could see a patient in the inpatient or outpatient
setting with heart disease. The content starts with the fundamentals of history
and physical examination, followed by the basics of cardiac testing, including
electrocardiography, echocardiography, and chest radiography. Specific chapters
focus on common symptoms, such as chest pain, syncope, and murmurs.
Extensive guidance is provided for each type of congenital lesion a patient
may present with in your office—each easily looked up and reviewed quickly
just prior to seeing a patient. Acquired diseases such as Kawasaki disease are
discussed, as well as infectious diseases, such as rheumatic fever, Lyme disease,
endocarditis, pericarditis, and myocarditis. Common preventive cardiology issues
are presented, including dyslipidemia, hypertension, sports clearance, screening
prior to attention-deficit/hyperactivity disorder medication administration,
and adolescent autonomic dysfunction. Finally, a robust discussion of cardiac
medications is provided, focused on the pediatric care professional.
In assembling this book, we have capitalized on the rich and unique resources
of the American Academy of Pediatrics (AAP), as well as contributions from
experts from around the globe. We thank all of our authors for their time and
expertise. The contents of this book were reviewed by experts from relevant
AAP sections, committees, and councils, including non–cardiology specialty
perspectives.
From Common CardiacThe editors
Issues and contributing
in Pediatrics: , authors are grateful for these reviewers’
expertise and generous feedback. Special thanks are due to the cardiologists
Published by AAP, 2018. All rights reserved. xvii
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INTRODUCTION
and cardiac surgeons of the Publications and Communications Workgroup

of the Section on Cardiology and Cardiac Surgery, who personally reviewed
every chapter. We want to give our sincere thanks to Chris Wiberg at the AAP,
who was the senior editor for this book. We appreciate his commitment to the
project, his editorial prowess, his constant encouragement, and his collaborative
spirit. Finally, we want to thank our families for their love, understanding, and
support while we took on this amazing endeavor.
This book is focused on the pediatric care professional for a specific reason:
You are essential partners with families and cardiology teams in the care of these
children. We hope this book will help answer questions that arise on a daily
basis and serve as an essential resource in your office, with the ultimate goal of
improving care for all children with heart disease.

Deepak Kamat, MD, PhD, FAAP

Published byxviii
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Common Cardiac Issues in Pediatrics

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Common Cardiac ­Issues in Pediatrics

Learn from leading experts the latest information on ­cardiac

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From Common Cardiac Issues in Pediatrics: ,

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Allison K. Black, MD Kanupriya Chaturvedi, MD, FAAP

From Common Cardiac Issues in Pediatrics: ,

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Kelley K. Hutchins, DO, MPH Sonya Kirmani, MD

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Bradley S. Marino, MD, MPP, MSCE, FAAP Mark D. Norris, MD, MS

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Kristen Sexson Tejtel, MD, PhD, MPH, Joshua D. Sparks, MD

From Common Cardiac Issues in Pediatrics: ,

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Philip L. Wackel, MD Melissa Yamauchi, MD, MPH

Don P. Wilson, MD, FNLA

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Part 1: Evaluation of the Patient

Part 2: Common Signs and Symptoms

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Part 3: Congenital Heart Disease

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Single-Ventricle Lesions................................................................... 320

Part 4: ­Cardiomyopathies and ­Channelopathies

Part 5: Acquired Cardiac Diseases

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Part 6: Infectious Diseases

Part 7: Preventive ­Cardiology

Part 8: General Issues in Primary Cardiac Care

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42. Transition and Transfer From ­Pediatric to Adult-Centered

Part 9: Special Conditions

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and cardiac surgeons of the Publications and Communications Workgroup

Jonathan N. Johnson, MD, FACC, FAAP

From Common Cardiac Issues in Pediatrics: ,

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Common Cardiac Issues in Pediatrics

Learn from leading experts the latest information on cardiac

Part 4: Cardiomyopathies and Channelopathies

Part 7: Preventive Cardiology

42. Transition and Transfer From Pediatric to Adult-Centered