Pediatric practice cardiology

PEDIATRIC PRACTICE Cardiology NOTICE Medicine is an ever-changing science As new research and clinical experience broaden our knowledge, changes in treatment and drug therapy are required The authors and the publisher of this work have checked with sources believed to be reliable in their efforts to provide information that is complete and generally in accord with the standards accepted at the time of publication However, in view of the possibility of human error or changes in medical sciences, neither the authors nor the publisher nor any other party who has been involved in the preparation or publication of this work warrants that the information contained herein is in every respect accurate or complete, and they disclaim all responsibility for any errors or omissions or for the results obtained from use of the information contained in this work Readers are encouraged to confirm the information contained herein with other sources For example and in particular, readers are advised to check the product information sheet included in the package of each drug they plan to administer to be certain that the information contained in this work is accurate and that changes have not been made in the recommended dose or in the contraindications for administration This recommendation is of particular importance in connection with new or infrequently used drugs PEDIATRIC PRACTICE Cardiology Marie Murphy Gleason Division of Cardiology The Children’s Hospital of Philadelphia Philadelphia, Pennsylvania Jack Rychik Division of Cardiology The Children’s Hospital of Philadelphia Philadelphia, Pennsylvania Robert Shaddy Division of Cardiology The Children’s Hospital of Philadelphia Philadelphia, Pennsylvania New York Chicago San Francisco Lisbon London Madrid Mexico City Milan New Delhi San Juan Seoul Singapore Sydney Toronto Copyright © 2012 by The McGraw-Hill Companies, Inc All rights reserved Except as permitted under the United States Copyright Act of 1976, no part of this publication may be reproduced or distributed in any form or by any means, or stored in a database or retrieval system, without the prior written permission of the publisher ISBN: 978-0-07-176970-9 MHID: 0-07-176970-6 The material in this eBook also appears in the print version of this title: ISBN: 978-0-07-176320-2, MHID: 0-07-176320-1 All trademarks are trademarks of their respective owners Rather than put a trademark symbol after every occurrence of a trademarked name, we use names in an editorial fashion only, and to the benefit of the trademark owner, with no intention of infringement of the trademark Where such designations appear in this book, they have been printed with initial caps McGraw-Hill eBooks are available at special quantity discounts to use as premiums and sales promotions, or for use in corporate training programs To contact a representative please e-mail us at bulksales@mcgraw-hill.com TERMS OF USE This is a copyrighted work and The McGraw-Hill Companies, Inc 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is dedicated to the patients and families who deal with cardiac disease every day We also dedicate it to the medical professionals in all areas who contribute to their care, no matter how big or small This page intentionally left blank Contents CONTRIBUTORS / ix PREFACE / xiii ACKNOWLEDGMENTS / xv Conotruncal Abnormalities 179 Lindsay S Rogers and Chitra Ravishankar 10 Single-Ventricle Congenital Heart Disease 195 Normal Cardiac Physiology and the Cardiac Exam Michael V Di Maria, Jeffrey R Boris and Victoria L Vetter Cardiac Testing 23 Brooke T Davey, R Lee Vogel, Meryl S Cohen, Mark A Fogel and Stephen M Paridon Fetal Cardiology 61 Donna Goff, Michael Quartermain and Anita Szwast The Genetics of Abnormal Cardiac Development 77 Lisa C.A D’Alessandro, Shabnam Peyvandi, Susan Schachtner and Elizabeth Goldmuntz Evaluation and Therapy: Neonatal Critical Heart Disease 95 Shyam K Sathanandam, Stephanie Fuller and Matthew J Gillespie Care of the Postprocedural Patient 117 Javier J Lasa, Roxanne Kirsch, Stephanie Fuller and Jonathan J Rome Acyanotic Shunt Lesions 135 Shari Wellen, Andrew C Glatz and Shobha Natarajan Acyanotic Heart Disease: Valves, Outflow Tracts, and Vasculature 155 Matthew C Schwartz, Joseph W Turek, Therese M Giglia, Stephanie Fuller and Andrew C Glatz Matthew J O’Connor, David J Goldberg and Jack Rychik 11 Neurodevelopment in Children with Complex Congenital Heart Disease 215 Marianne Glanzman, Daniel Licht and Gil Wernovsky 12 Cardiac Arrhythmias 231 Akash Patel, William Bonney and Maully Shah 13 Heart Failure, Cardiomyopathy, and Pulmonary Hypertension 249 Kimberly Lin, Thomas Bernadzikowski, Stephen Walker, Brian Hanna and Beth Kaufman 14 Acquired Heart Disease 265 Katherine E Bates, Anirban Banerjee and Marie M Gleason 15 Preventive Cardiology 289 Aimee Parnell, Aaron Dorfman and Julie Brothers 16 Pediatric Issues for Patients with Congenital Heart Disease 315 Camila Londono-Obregon, Lisa M Montenegro, Therese Giglia, V Ramesh Iyer and Paul Farrell Jr 17 Survival to Adulthood 335 Alex Davidson, Yuli Y Kim, Stephanie Fuller and David Drajpuch INDEX / 353 This page intentionally left blank Contributors Anirban Banerjee, MD Clinical Associate Professor of Pediatrics The University of Pennsylvania School of Medicine Attending Cardiologist The Children’s Hospital of Philadelphia Philadelphia, Pennsylvania Julie Brothers, MD Assistant Professor of Pediatrics The University of Pennsylvania School of Medicine Director, Lipid Heart Clinic, Division of Cardiology The Children’s Hospital of Philadelphia Philadelphia, Pennsylvania Katherine Bates, MD Instructor in Pediatrics The University of Pennsylvania School of Medicine Fellow in Cardiology The Children’s Hospital of Philadelphia Philadelphia, Pennsylvania Meryl Cohen, MD Medical Director Echocardiography Laboratory Associate Director, Cardiology Fellowship Program The Children’s Hospital of Philadelphia Associate Professor of Pediatrics University of Pennsylvania School of Medicine Philadelphia, Pennsylvania Thomas Bernadzikowski, MS, MSN, CRNP Certifed Registered Nurse Practitioner Cardiology, Cardiomyopathy and Heart Failure Program The Children’s Hospital of Philadelphia Philadelphia, Pennsylvania William Bonney, MD Assistant Professor of Pediatrics The University of Pennsylvania School of Medicine Staff Cardiologist Section of Electrophysiology Division of Cardiology The Children’s Hospital of Philadelphia Philadelphia, Pennsylvania Jeffrey R Boris, MD Clinical Associate Professor of Pediatrics The University of Pennsylvania School of Medicine Staff Cardiologist The Children’s Hospital of Philadelphia Philadelphia, Pennsylvania Lisa C.A D’Alessandro, MD Instructor in Pediatrics The University of Pennsylvania School of Medicine Fellow in Cardiology The Children’s Hospital of Philadelphia Philadelphia, Pennsylvania Brooke Davey, MD Cardiology Fellow The Children’s Hospital of Philadelphia Instructor in Pediatrics University of Pennsylvania School of Medicine Philadelphia, Pennsylvania Alex Davidson, MD Professor of Clinical Pediatrics and Medicine The University of Pennsylvania School of Medicine Staff Cardiologist Philadelphia Adult Congenital Cardiac Center The Children’s Hospital of Philadelphia and Hospital of the University of Pennsylvania Philadelphia, Pennsylvania Index Q definition, 239 management, 240 Atrial flutter in adult congenital heart disease, 338 causes, 239 clinical features, 239 definition, 239 management, 239 neonatal, 239 postoperative, 239 Atrial septal defects (ASDs), 138–140 Amplatzer septal occluder, 127, 128f, 130f clinical presentation of, 139 physical examination, 139 symptoms, 139 definition, 138 embryology, 138 epidemiology, 138–139 Helex device in, 127, 129f–130f pathogenesis, 139 summary of, 141t treatment, 140 catheter-based intervention, 140 medical management, 140 postoperative concerns, 140 postoperative follow-up, 140 surgical intervention, 140 Atrioventricular canal defect See Common atrioventricular canal defects Atrioventricular re-entrant tachycardia (AVRT) hypertrophic cardiomyopathy, 244 supraventricular tachycardia, 236 Wolff-Parkinson-White syndrome, 237–238 Atrioventricular valves mitral valve, 165–169 prolapse, 168–169 regurgitation, 167–168 rheumatic heart disease, 169 stenosis, 165–167 tricuspid valve, 169–172 Ebstein anomaly, 170–172 regurgitation, 172 stenosis, 169–170 Attention-deficit hyperactivity disorder (ADHD), 323–325, 324t Auscultation click, heart sounds in, knock, patient in, rub, 7–8 snaps, Austin Flint murmur(s), 161 AV nodal re-entrant tachycardia (AVNRT), 236 AV septal defects (AVSDs) See Common atrioventricular canal defects AVMs See Arteriovenous malformations AVNRT (AV nodal re-entrant tachycardia), 236 AVRT See Atrioventricular re-entrant tachycardia B Baffle leaks, 184 Balloon angioplasty in aortic coarctation, 110, 128–129, 174 in branch pulmonary artery stenosis, 128–129 Balloon aortic valvuloplasty, 129–130 Balloon atrial septostomy, 180, 182 Balloon dilation, 129 for dysplastic valve, 163 353 for mitral valve, 166 for Noonan syndrome, 163 in peripheral pulmonary stenosis, 177 for valvar pulmonary stenosis, 163 Balloon pulmonary arterioplasty, 129 Balloon valvuloplasty in mitral stenosis, 166 in pulmonary regurgitation, 165 in pulmonary stenosis, 108, 108f in valvar aortic stenosis, 159 in valvar pulmonary stenosis, 163, 163f Barrier methods, 320 Barth syndrome, 258 Bazett formula, 26 Beery-Buktenica developmental test of visual motor integration (Beery VMI), 220–221 Benign syncope, 243 Benzathine penicillin G, for acute rheumatic fever, 275, 277 Benzodiazepine, 330 Bernoulli equation, 39 β-Blockers for arrhythmia, 241, 244 for atrial fibrillation, 240 for catecholaminergic polymorphic ventricular tachycardian, 244 for ectopic atrial tachycardia, 238 for heart failure, 251 for hypertrophic cardiomyopathy, 256 for long QT syndrome, 244 for monomorphic ventricular tachycardia, 241 for paroxysmal supraventricular tachycardia, 237 for torsade de pointes, 242 Bicuspid aortic valve, 89 Bile acid sequestrants, 309 Birth history, 3–4 Blalock-Taussig shunt, 70–71 antithrombotic prophylaxis, 326 pulmonary atresia with intact ventricular septum, 209 in single-ventricle congenital heart disease, 202–203, 203f, 204, 208 in tetralogy of Fallot, 100f Blood pressure body mass index, 297 high See also Hypertension classifications of, 294, 297 definition of, 294–297 measurement of, 4–5, 294–299 selection of cuff in, 295, 297f sphygmomanometer in, 295 systolic and diastolic percentile values for, 296–297, 301 Blowing murmur(s), 168 BNP (B-type natriuretic peptide), 251 Body mass index (BMI), 289–294 Bone morphogenic protein receptor (BMPR2), 259 Bradyarrhythmias, 15, 231–235 ectopic atrial rhythm, 232 heart block, 233–235 junctional bradycardia, 232 premature atrial contractions, 233 premature ventricular contraction, 233 sinus bradycardia, 231–232 wandering atrial pacemaker, 232–233 Bradycardia, junctional, 232 sinus, 231–232 Branch pulmonary artery stenosis, 128–129 Bruce protocol, 34, 35f Brugada syndrome, 27, 27f, 245 B-type natriuretic peptide (BNP), 251 354 Q Index C Calcium channel blocker, 302 for arrhythmia, 244 for atrial fibrillation, 240 for ectopic atrial tachycardia, 238 for hypertension, 302 for hypertrophic cardiomyopathy, 256 Cardiac anatomy, 1, 2f Cardiac arrhythmias See Arrhythmias Cardiac asthma, 165 Cardiac catheterization Amplatzer septal occluder, 127, 128f in aortic coarctation, 173 basics, 127 complications, 130–131 in dilated cardiomyopathy, 255 in heart failure, 251 Helex device, 127, 129f–130f interventional procedures, 127–130 in peripheral pulmonary stenosis, 177 in pulmonary atresia with intact ventricular septum, 209 in pulmonary hypertension, 260 in restrictive cardiomyopathy, 257 in single-ventricle congenital heart disease, 201, 204–205 in subvalvar aortic stenosis, 159 in subvalvar pulmonary stenosis, 164 in supravalvar aortic stenosis, 160 in supravalvar pulmonary stenosis, 164 transesophageal echocardiography, 46 in valvar aortic stenosis, 158 in valvar pulmonary stenosis, 163 Cardiac cycle, 1, 2f Cardiac evaluation cardiac magnetic resonance, 46–56 in congenital heart disease, 47–54 future developments, 55–56 indications of, 54–55 overview, 46–47 cardiopulmonary stress in/exercise testing in, 30–37 echocardiography imaging in, 37–46 pediatrics, 41–46 principles, 37–41 transesophageal, 46 electrocardiography in cardiac rhythm detection, 27–30 pediatric electrocardiogram, 23–27 Cardiac index, Cardiac magnetic resonance (CMR) for anatomy, 47–49, 47f–49f in aortic coarctation, 174f in aortic regurgitation, 161 in arrhythmogenic right ventricular cardiomyopathy, 258 for blood flow, 49–53 in chest pain, 53–54 in congenital heart disease, 46–56 in dilated cardiomyopathy, 255 future developments, 55–56 in hypertrophic cardiomyopathy, 256 indications for, 54–55 anatomy, 54t physiology/function, 55t tissue characterization, 55t in mitral regurgitation, 168 in myocarditis, 279 overview, 46–47 in pulmonary regurgitation, 165 in restrictive cardiomyopathy, 257 in single-ventricle congenital heart disease, 201, 202f, 204–205 for tissue characterization, 53–54 in vascular rings, 176 for ventricular function, 49 Cardiac murmur(s), 164 Cardiac output, 2, 20 cardiac magnetic resonance, 53 exercise testing, 31 Cardiac physiology, 1–3 Cardiac resynchronization therapy, 252–253 Cardiac rhythm detection, 30t additional testing, 29 external cardiac ambulatory telemetry, 28–29, 29f fifteen-lead ECG with rhythm strip, 27 Holter monitoring, 27, 28f insertable cardiac monitors, 29 transtelephonic event recorders, 27–28, 28f Cardioinhibitory syncope, 15 Cardiomyopathy, 254–258 arrhythmogenic right ventricular, 258 in children, 254–258 definition, 254–255 dilated, 255 epidemiology, 254–255 hypertrophic, 255–257 left ventricular noncompaction, 258 restrictive, 257–258 secondary, 255 Cardiopulmonary bypass in children, 117–118 in infant, 117–118 in neonates, 117–118 ultrafiltration, 118 Cardiopulmonary interactions, postoperative period, 121 Cardiopulmonary stress testing See Exercise stress testing Cardiovascular system fetal circulation in, 61, 62f extracardiac anomalies and, 67–69 Catecholaminergic medications hemodynamic monitoring, 119–120 side effects, 120t Catecholaminergic polymorphic ventricular tachycardia (CPVT), 244 Catheter ablation in arrhythmogenic right ventricular dysplasia/cardiomyopathy, 245 in atrial fibrillation, 240 in atrial flutter, 239 in ectopic atrial tachycardia, 238–239 in paroxysmal supraventricular tachycardia, 237 CAVCD See Common atrioventricular canal defects Cavopulmonary anastomosis, 326–328 CCAM (Congenital cystic adenomatoid malformation), 67–68 CCHB (Congenital complete heart block), 73–74 cCHD See Complex congenital heart disease CDH (Congenital diaphragmatic hernia), 68, 68f Central nervous system injuries, 123–124 Char syndrome, 147 CHARGE syndrome, 86 CHD See Congenital heart disease Chest pain alphabetical pneumonic for evaluation, 18t cardiac causes, 19 cardiac magnetic resonance, 53–54 clinical presentation, 17 definition of, 17 diagnosis, 19, 20f differential diagnosis, 19 epidemiology, 17 gastrointestinal causes, 18 Index Q guidelines, 20 inspection of, musculoskeletal causes, 17 in pericarditis, 280 physical examination in, 17 psychogenic causes, 18 pulmonary causes, 17–18 treatment, 20 Chest radiograph (CXR) in aortic coarctation, 173 in aortic regurgitation, 161 in arteriovenous malformations, 151 in atrial septal defects, 139 in common atrioventricular canal defects, 146 in dextro-transposition of the great arteries, 102, 102f, 181t in dilated cardiomyopathy, 255 in heart failure, 251 in Kawasaki disease, 268 in levo-transposition of the great arteries, 181t in mitral regurgitation, 168 in mitral stenosis, 166 in myocarditis, 278 in patent ductus arteriosus, 148 in pericarditis, 281 in pulmonary regurgitation, 165 in restrictive cardiomyopathy, 257 in single-ventricle congenital heart disease, 201 in subvalvar pulmonary stenosis, 164 in tetralogy of Fallot, 99, 99f, 181t in total anomalous pulmonary venous connection, 105, 105f in tricuspid atresia, 106 in truncus arteriosus, 181t in valvar aortic stenosis, 157 in valvar pulmonary stenosis, 163 in vascular rings, 176 in ventricular septal defect, 143 Child health questionnaire (CHQ), 220 Children body mass index, 294 cardiomyopathy, 254–258 cholesterol, 308–309, 308t dyslipidemia in, 302–311 screening for, 306–308, 308t familial combined hyperlipidemia in, 310 familial hypercholesterolemia in, 310 heart failure in, 249–254 heterozygous familial hypercholesterolemia in, 304, 310 hypertension in, 294–302 ambulatory blood pressure monitoring, 301, 301t, 302 antihypertensive medications, 302, 303t causes of, 300 clinical findings, 302 diagnosis, 299f treatment, 301–302 LDL-C, 310t pulmonary hypertension in, 258–263, 259t Chlorothiazide, for shunt lesions, 136 Cholesterol absorption inhibitors, 311 classification of, levels, 308, 308t dietary supplements to, lower, 309t management of, 309 CHQ (Child health questionnaire), 220 Chromosomal Analysis congenital heart disease, 91 trisomy 13, 91 trisomy 18, 91 trisomy 21, 91 Turner syndrome, 91 Circulation changes after birth, 95–97 CK (Creatine kinase), 310 Clicks, in aortic stenosis, 109 in pulmonary stenosis, 107–108 Clubbing, CMR See Cardiac magnetic resonance Coarctation of aorta (COA), 69–70 clinical features, 110 clinical presentation, 173 congenital, 109–110 definition, 172 diagnosis, 173 epidemiology, 172 hemodynamics, 110 management, 110 neonatal surgical repair, 110 pathogenesis, 172–173 treatment, 173–174 Turner syndrome and, 172–173 Coeur-en-sabot, 99 Color flow Doppler echocardiography, 40, 40f in aortic regurgitation, 161 in common atrioventricular canal defects, 146 in dextro-transposition of the great arteries, 102 in Marfan syndrome, 161f in mitral regurgitation, 168 in tricuspid regurgitation, 171f Common atrioventricular canal defects (CAVCD), 145–146 clinical presentation, 145–146 embryology, 145 epidemiology, 145 physiology of, 145 summary of, 147t treatment, 146 medical management, 146 postoperative concerns, 146 postoperative follow-up, 146 surgical repair, 146 trisomy 21, 145 Complex congenital heart disease (cCHD) central nervous system abnormalities in children with, 216t clinical diagnosis for children, 224t developmental deficits in children with, 223t hypoplastic left heart syndrome, 218–219, 221–222 neurodevelopment in children with, 215–225 adolescents, 220–222 birth to three years of age, 218–219 epidemiology, 215 four to five years of age, 219–220 pathogenesis, 215–218 school-age children, 220–222 treatment, 222–225 total anomalous pulmonary venous return, 221–222 transposition of the great arteries, 218–219, 221–222 Cone reconstruction, 171–172 Congenital cardiac lesions acyanotic, 156t in children/adolescents, 157t Congenital complete heart block (CCHB), 73–74 Congenital cystic adenomatoid malformation (CCAM), 67–68 Congenital diaphragmatic hernia (CDH), 68, 68f Congenital heart defects associated syndromes or genetic defects with, 74–75, 74t left-sided obstructive lesions, 69–70, 69f pulmonary atresia with intact ventricular septum, 70–71 355 356 Q Index Congenital heart defects (Cont.): right-sided obstructive lesions, 70–71 tetralogy of Fallot, 70 tricuspid atresia/VSD, 71 Congenital heart disease (CHD) amniocentesis, 74 anesthesia and sedation, 328–330 anticoagulation issues and, 325–328 antithrombotic prophylaxis post cavopulmonary anastomosis, 326–328 post Fontan procedure, 328 post Norwood procedure, 326 post systemic-to-pulmonary shunts, 326 valve replacements with, 328 attention-deficit hyperactivity disorder and, 323–325 contraception and, 319–320 and dental care, 132, 315–316 diagnostic approach to, 91–93 chromosomal analysis, 91 FISH, 91 genome-wide microarray, 91 karyotype, 91 targeted mutation screening and sequencing, 91–92 environmental factors, 77–78 diabetes, 77–78 maternal medications, 78 pregestational diabetes, 77–78 prevention of, 78 epidemiology, 77 etiology of, genetic syndrome, 78–89 Alagille syndrome, 86 CHARGE syndrome (Hall-Hittner syndrome), 86 Costello syndrome, 86–87 Down syndrome (trisomy 21), 82–83 Holt-Oram syndrome, 86 LEOPARD syndrome, 86–87 Marfan syndrome, 87–88 Noonan syndrome, 86–87 overview, 78 22q11.2 microdeletion syndrome, 84–85 trisomy 13 syndrome, 83 trisomy 18 syndrome, 83 Turner syndrome, 83–84 VATER association, 88–89 Williams syndrome, 85–86 heritable heart disease, 89 bicuspid aortic valve, 89 hypoplastic left heart syndrome, 89 left-sided lesions, 89 nonsyndromic single-gene disorders, 89 immunization and, 132, 321–322 altered immunocompetence, 321–322 influenza vaccination, 321 respiratory syncytial virus prophylaxis, 321 infective endocarditis prophylaxis and, 316–319 maternal conditions associated with, 78t overview, 61, 77 patient approach to, 89–91 examination, 90–91 family history, 90 history, 89–90 pediatric issues for patients with, 315–332 in postnatal period, 118t postprocedural outpatient evaluation, 131–132 pregnancy and, 319–320, 346t QT interval prolongation, 322–323 recurrence risk, 78 single-ventricle defects, 195–211 survival to adulthood, 335–347 thromboembolic complications in, 327t Congenital pulmonary vein stenosis, 262 Congestive heart failure, 137t Conoseptal hypoplasia, 142 Conotruncal abnormalities clinical findings in, 181t tetralogy of Fallot, 186–190 transposition of the great arteries, 179–186 truncus arteriosus, 190–192 Conotruncal anomaly facial syndrome, 84–85 Continuous murmurs, 11 Continuous wave Doppler echocardiography, 39 Contraception barrier methods, 320 combined hormonal, 320 congenital heart disease and, 319–320 intrauterine devices, 320 progesterone-based, 320 risks and advantages of, 320t tubal ligation, 320 Contractility, Contrast echocardiography, 40–41, 41f Coronary aneurysm, 269, 269t Coronary artery patency, 183 Coronary buttons, 182, 182f Coronary sinus septal defect, 138–140 Coronary vasospasm, 18–19 Costello syndrome, 87 Costochondritis, 17 CPVT (Catecholaminergic polymorphic ventricular tachycardia), 244 C-reactive protein (CRP), 267 Creatine kinase (CK), 310 Critical aortic stenosis clinical features, 109 hemodynamics, 108–109 management, 109 neonatal catheter intervention in, 109 neonatal surgical repair of, 109 overview, 108 critical AS, 155 Critical heart disease in newborn cyanotic, 98–108 management, principles of, 97–98 as shock, 108–114 Critical PS, 162 CRP (C-reactive protein), 267 CXR See Chest radiograph Cyanosis See also Cyanotic heart diseases described, 98 in dextro-transposition of the great arteries, 181 in neonates, 96t, 98–108 in physical examination, Cyanotic heart diseases dextro-transposition of the great arteries, 101–103 pulmonary atresia with intact ventricular septum, 100–101 pulmonary stenosis, 107–108 tetralogy of Fallot, 98–100 total anomalous pulmonary venous connection, 103–106 tricuspid atresia, 106–107 D Damus-Kaye-Stansel procedure, 106–107 DCM See Dilated cardiomyopathy Dental home, 316 Dexmedetomidine (Precedex), 330–331 Index Q Dextro-transposition of the great arteries arterial switch operation, 182–183 cardiac defects associated with, 180f characteristics, 71–72 clinical features, 102 clinical presentation, 181 definition, 179 diagnosis, 72 epidemiology, 179 hemodynamics, 101–102 long-term management of, 185 management, 103 neonatal interventions, 103 neonatal surgical repair, 103 outpatient management of, 183–184 overview, 101 pathophysiology, 179–180 physical examination, 181t postoperative management, 103 Diaphoresis, 5–6 Diastolic dysfunction, 2, 45 Diastolic murmurs, 11, 20, 161, 165, 168 Dietary interventions for dyslipidemia, 308–309 adjunctive dietary therapy, 309, 309t individual-based approach, 308–309 population-based approach, 308 DiGeorge syndrome, 74, 84–85 Digoxin for atrial fibrillation, 240 for ectopic atrial tachycardia, 238 for fetal supraventricular tachycardia, 73 in heart failure management, 252, 252t for paroxysmal supraventricular tachycardia, 237 for ventricular tachycardia, 241 Dilated cardiomyopathy (DCM) arrhythmias and, 245–246 clinical presentation, 255 described, 255 diagnosis, 245, 255 differential diagnosis, 255 etiologies of, 252t implantable cardioverterdefibrillator in, 246 management, 246 pathogenesis, 255 treatment, 255 DILV See Double-inlet left ventricle 2-Dimensional echocardiography in acute rheumatic fever, 274 in left ventricular hypertrophy, 300–301 in myocarditis, 278 in single-ventricle congenital heart disease, 201 Discharge medical management, 131–132 preparation for, 126–127 Diuretics for hypertension, 302 in mitral stenosis, 166 shunt lesions, 136–137 Doppler echocardiography in aortic coarctation, 173 in arteriovenous malformations, 151 color flow, 40 in common atrioventricular canal defects, 146 continuous wave, 39 in diastolic dysfunction, 45 in fetal, 199 in Kawasaki disease, 268 357 in mitral stenosis, 166 in myocarditis, 278 in pericarditis, 281 in peripheral pulmonary stenosis, 177 in pulmonary regurgitation, 165 pulsed wave, 38–39, 64, 72 restrictive cardiomyopathy, 257 in single-ventricle congenital heart disease, 64–67 in subvalvar pulmonary stenosis, 164 in supravalvar pulmonary stenosis, 164 in valvar pulmonary stenosis, 163 in ventricular septal defect, 143 Double-inlet left ventricle (DILV) echocardiography, 210f in single-ventricle congenital heart disease, 209 Double-switch operation, 186 Down syndrome, 82–83 Ductus arteriosus, 66–67 Ductus venosus, 64–65, 65f Dynamic exercise, 341 Dyslipidemia clinical presentation, 304–306 definition, 302 dietary interventions, 308–309 adjunctive dietary therapy, 309, 309t individual-based approach, 308–309 population-based approach, 308 differential diagnosis, 306 epidemiology, 304–306 elevated lipoprotein (a), 306 familial combined hyperlipidemia, 304–305 familial dysbetalipoproteinemia, 305–306 familial hypertriglyceridemia, 305 heterozygous familial hypercholesterolemia, 304 Fredrickson classification of, 306t genetic causes of, 306t obesity and, 309 overweight and, 309 pathogenesis, 302–304 pharmacologic therapy, 309–311 bile acid sequestrants, 309 cholesterol absorption inhibitors, 311 hydroxy-3-methyl-glutaryl–coenzyme A (HMG-CoA) reductase inhibitors, 309–311 screening for, 306–308, 308t secondary, causes of, 307t treatment, 308–311 Dysplastic valve, 162–163 E Ebstein anomaly clinical presentation, 170 definition, 170 diagnosis, 170–171 epidemiology, 170 pathogenesis, 170 treatment, 171–172 Wolff-Parkinson-White syndrome, 170 ECAT (External cardiac ambulatory telemetry), 28–29, 29f ECG See Electrocardiogram Echocardiography in aortic coarctation, 173 in aortic regurgitation, 161 in aortic stenosis, 109 in arrhythmogenic right ventricular cardiomyopathy, 258 in atrial septal defects, 139–140 in bicuspid aortic valve, 158f in cardiomyopathy, 254 358 Q Index Echocardiography (Cont.): color flow, 40, 40f contrast, 40–41, 41f in dextro-transposition of the great arteries, 181 in dilated cardiomyopathy, 255 Doppler, 38–39 in double-inlet left ventricle, 210f in Ebstein anomaly, 171, 171f fetal described, 62 in hypoplastic left heart syndrome, 199f indications for, fetal, 62, 62t indications for, maternal, 62, 62t in single-ventricle congenital heart disease, 198, 199f, 201 in fetal arrhythmia, 72 in heart failure, 251–253 history, 37 in HLHS syndrome, 69, 112, 204f in hypertrophic cardiomyopathy, 256 in interrupted aortic arch, 174 in Kawasaki disease, 268 in levo-transposition of the great arteries, 185 in left ventricular hypertrophy, 300, 301f in Marfan syndrome, 169f in mitral regurgitation, 168 in mitral stenosis, 166 in mitral valve prolapse, 169, 169f M-mode, 37–38, 37f–38f in myocarditis, 278–279 pediatric, 41–46 in pericarditis, 281 in peripheral pulmonary stenosis, 177 in premature ventricular contraction, 233 in pulmonary atresia with intact ventricular septum, 100, 209 in pulmonary hypertension, 260 in pulmonary regurgitation, 165 in pulmonary stenosis, 108 in restrictive cardiomyopathy, 257 in sacrococcygeal teratoma, 67 in subvalvar aortic stenosis, 159, 159f in supravalvar aortic stenosis, 160, 160f in supravalvar pulmonary stenosis, 164 in tetralogy of Fallot, 70, 187 three-dimensional, 41 tissue Doppler, 40, 41f transesophageal, 46 in tricuspid atresia, 106, 208f in tricuspid regurgitation, 171f in truncus arteriosus, 191 two-dimensional, 38 in valvar aortic stenosis, 157, 158f in valvar pulmonary stenosis, 163 in vascular rings, 176 in Williams syndrome, 160f ECMO (Extracorporeal membrane oxygenation), 120 Ectopia cordis, 114 Ectopic atrial rhythm, 232 Ectopic atrial tachycardia causes, 238 clinical features, 238 definition, 238 management, 238–239 Edema, Edler, Inge, 37 Edward syndrome, 83 Ehlers-Danlos syndrome, 88 Eisenmenger syndrome, 3, 142 Ejection fraction, 43–44 Ejection murmurs, 8–10 Electrocardiogram (ECG) in aortic coarctation, 173 in aortic regurgitation, 161, 161f in aortic stenosis, 109 in arrhythmogenic right ventricular cardiomyopathy, 258 in arteriovenous malformations, 151 in atrial septal defects, 139 in attention-deficit hyperactivity disorder, 324, 326t in benign syncope, 243 in cardiomyopathy, 254 in chest pain, 18–19 in coarctation of aorta, 110 in common atrioventricular canal defects, 146 in dextro-transposition of the great arteries, 102, 181t in dilated cardiomyopathy, 255 in Ebstein anomaly, 170–171 in heart failure, 251–253 in hypertrophic cardiomyopathy, 256 in interrupted aortic arch, 111 in levo-transposition of the great arteries, 181t in mitral regurgitation, 168, 168f in mitral stenosis, 166, 166f in myocarditis, 19 in palpitations, 12 in patent ductus arteriosus, 148 in peripheral pulmonary stenosis, 177 in pulmonary atresia with intact ventricular septum, 100 in pulmonary regurgitation, 165 in pulmonary stenosis, 108 in restrictive cardiomyopathy, 257 in single-ventricle congenital heart disease, 201 in subvalvar aortic stenosis, 159 in subvalvar pulmonary stenosis, 164 in supravalvar aortic stenosis, 160 in supravalvar pulmonary stenosis, 164 in syncope, 16, 243 in tetralogy of Fallot, 99 in total anomalous pulmonary venous connection, 105 in tricuspid atresia, 106 in truncus arteriosus, 181t in valvar aortic stenosis, 157, 157f in valvar pulmonary stenosis, 163 in ventricular septal defect, 143 Electrolyte abnormalities, 124 Enalapril, for shunt lesions, 136–137 Endocarditis, 283–287 American heart association recommendations, 287 antibiotic prophylaxis regimens, 287t definition, 283 diagnosis, 285 differential diagnosis, 285, 285t epidemiology, 283 etiologic agents, 284t infective, 283 pathogenesis, 283–284 peripheral blood cultures, 285–286 prevention, 287 symptoms and signs, 284t treatment of, 286–287 Epoprostenol, 261 Ergometers, 33, 34t ESR (erythrocyte sedimentation rate), 267 Euro heart survey, 336–337 Exercise for adult congenital heart disease, 338–341 dynamic (isotonic), 341 static (isometric), 341 Index Q Exercise stress testing American Heart Association guidelines, 34t contraindications to, 36–37, 37t in dilated cardiomyopathy, 255 environment, 32 equipment, 33–34 heart, 31–32 in hypertrophic cardiomyopathy, 256 indications for, 34–36, 36t lung, 31–32 overview, 30 physiology, 30–31 protocols, 34 respiratory exchange ratio, 30–31 safety precautions, 32–33 External cardiac ambulatory telemetry (ECAT), 28–29, 29f Extracorporeal membrane oxygenation (ECMO), 120 Ezetimibe, for dyslipidemia, 311 F Familial broad β disease, 305–306 Familial combined hyperlipidemia (FCHL), 304–305, 310 Familial dysbetalipoproteinemia (FDBL), 305–306 Familial hypertriglyceridemia (FHTG), 305 Family history, Fasting lipid profile (FLP), 308–309 FBN1 gene, 87 FCHL (Familial combined hyperlipidemia), 304–305, 310 FDBL (Familial dysbetalipoproteinemia), 305–306 Fetal echocardiography, 62–63 in dextro-transposition of the great arteries, 181 in single-ventricle congenital heart disease, 198–200 in tetralogy of Fallot, 187 Fetus arrhythmia, 72–74 cardiovascular imaging described, 62–64 of ductus arteriosus, 66–67 of ductus venosus, 64–65 of middle cerebral artery, 65–66 of umbilical artery and vein, 64 circulation in, 61, 62f, 95, 96f, 218 dextro-transposition of the great arteries with, 71–72 left-sided obstructive lesions with, 69–70 myocardium, 61–62 right-sided obstructive lesions with, 70–71 FHTG (Familial hypertriglyceridemia), 305 Fick equation, 97–98 Fifteen-lead surface electrocardiogram, 27, 123f FISH (Fluorescent in situ hybridization), 91 FLP (Fasting lipid profile), 308–309 Fluorescent in situ hybridization (FISH), 91 Fontan procedure in antithrombotic prophylaxis, 328 in hypoplastic left heart syndrome, 205–206 in lesions with obstruction to systemic blood flow, 205–206 in single-ventricle congenital heart disease, 202–203 in tricuspid atresia, 207–208 Four-dimensional flow imaging, 53 Friedewald formula, 308 Functional Independence Measure for Children (WeeFIM), 219–220 Furosemide, for shunt lesions, 136 G Gadolinium, 53 GAS (group A β-hemolytic streptococcal) infections See Acute rheumatic fever Gastroesophageal reflux, 18 359 GATA4 gene, 89 Genetic syndrome Alagille syndrome, 86 cardiac disease associated with, 79t–81t CHARGE syndrome, 86 Costello syndrome, 86–87 Down syndrome, 82–83 Holt-Oram syndrome, 86 LEOPARD syndrome, 86–87 Marfan syndrome, 87–88 Noonan syndrome, 86–87 22q11.2 microdeletion syndrome, 84–85 trisomy 13 syndrome, 83 trisomy 18 syndrome, 83 Turner syndrome, 83–84 VATER association, 88–89 Williams syndrome, 85–86 Genome-wide microarray, 91 Glenn procedure, 205, 326–328 GORE HELEX Septal Occluder, 141f Great artery(ies) dextro-transposition of, 101–103, 179–185 levo-transposition of, 185–186 Grooved pegboard test, 221 H Hall-Hittner syndrome, 86 HCM See Hypertrophic cardiomyopathy HDL-C (High-density lipoprotein cholesterol), 302–312 Heart block, 233–235 Heart exercise testing, 31–32 Heart failure, 249–254 ACE inhibitors, 251, 252t aldosterone antagonists, 251, 252t β-blockers for, 251, 252t cardiac resynchronization therapy, 252–253 definition, 249 diuretics, 252, 252t International society of heart and lung transplant practice guidelines, 252 left, 251–254 New York Heart Association classification, 250t outpatient, medications, 252t pathophysiology, 249–251 staging guidelines, 250t ventricular assist devices in, 253, 254f “Heart hand” syndrome, 86, 138 Heart murmurs characteristics, continuous, 11 diastolic, 11 grading of, 10 innocent, 8, 9t location, 9–10 maneuvers, 10, 11t patent ductus arteriosus, 11 pathologic, quality, 10 systolic, 8–10 timing, Heart rate, 4, 24, 338 Heart sound(s), 7, 8t Heart transplantation, 253, 254t HeFH (Heterozygous familial hypercholesterolemia), 304, 307f, 310 Helex device, 127, 129f–130f Hemi-Fontan procedure, 205 Hemodynamic monitoring, 118–121 360 Q Index Heritable heart disease bicuspid aortic valve, 89 hypoplastic left heart syndrome, 89 left-sided lesions, 89 nonsyndromic single-gene disorders, 89 Heterotaxy syndrome, 196, 198, 200 Heterozygous familial hypercholesterolemia (HeFH), 304, 307f, 310 High-density lipoprotein cholesterol (HDL-C), 302–312 History See Patient history HLHS See Hypoplastic left heart syndrome Holosystolic murmurs, 9–10, 168, 256 Holter, Jefferis, 27 Holt-Oram syndrome, 86, 138 Hormonal contraception, combined, 320 Hybrid procedure, 206 Hydroxy-3-methyl-glutaryl–coenzyme A (HMG-CoA) reductase inhibitors, 309–311, 311f Hypercyanotic spells, 99, 187–188 Hyperoxia test, 201 Hypertension antihypertensive medications, 302, 303t causes of, 299–300 clinical evaluation, 300–301 definition, 294–297 diagnosis, 298, 299f differential diagnosis, 298–300, 299t obesity and, 297, 302 overview, 294 primary, 297 secondary, 297–298, 298t treatment, 301–302 Turners syndrome, 298, 300 Williams syndrome, 298, 300 Hypertensive urgency, 297 Hypertrophic cardiomyopathy (HCM) arrhythmias and, 244 atrioventricular re-entrant tachycardia, 244 β-blockers for, 256 calcium channel blockers for, 256 cardiac magnetic resonance in, 53–54 clinical presentation, 256 diagnosis, 243, 256 differential diagnosis, 256 etiologies of, 252t exercise stress testing in, 256 management, 244 myomectomy in, 257 overview, 255–256 pathogenesis, 256 sudden cardiac death, 256–257 treatment of, 256–257 Hypoplastic left heart syndrome (HLHS), 65, 69, 69f, 75, 89, 218–219, 221–222 approach of high-risk neonates with, 113–114 balancing pulmonary and systemic blood flow, 112–113 classifications, 111–112 fetal circulation, 218f first stage of palliation, 205f Fontan procedure in, 205–206 Glenn procedure in, 205 Hemi-Fontan procedure in, 205 hemodynamics, 112 management, 112 Norwood procedure in, 203–206 restrictive/intact atrial septum with, 113 second stage of palliation, 206f single-ventricle congenital heart disease, 195–210 third stage of palliation, 207f Hypoplastic right heart syndrome, 208–209 Hypoxic spells, 99 I IAA See Interrupted aortic arch IART (Intra-atrial re-entrant tachycardia), 239 ICD See Implantable cardioverterdefibrillator ICMs (Insertable cardiac monitors), 29 IDEA (Individuals with disabilities education act), 222–225 Idiopathic chest wall pain, 17 IEP (Individualized education plan), 225 Immunization for congenital heart disease, 321–322 altered immunocompetence, 321–322 general rules on, 322 influenza vaccination, 321 respiratory syncytial virus prophylaxis, 321 Implantable cardioverterdefibrillator (ICD) in arrhythmogenic right ventricular dysplasia/cardiomyopathy, 244–245 in Brugada syndrome, 245 in dilated cardiomyopathy, 246 in monomorphic ventricular tachycardia, 241 Individualized education plan (IEP), 225 Individuals with disabilities education act (IDEA), 222–225 Indomethacin, 148 Infective endocarditis, 283 antibiotic prophylaxis for, 316–319 dental procedures and drug regimens, 317, 318t fundamental principles, 316 guidelines, 316–317 other comorbidities, 319 recommended interventions for, 318–319 cardiac surgery and, prophylactic drug regimens for, 319 dental or respiratory tract procedures and, prophylactic drug regimens for, 318 genitourinary tract procedures and, prophylactic drug regimens for, 318 infected skin or musculoskeletal tissue procedures and, prophylactic drug regimens for, 318–319 Infliximab, for Kawasaki disease, 270 Influenza vaccine in congenital heart disease, 321 Kawasaki disease and, 270 Innocent murmurs, 8, 9t Insertable cardiac monitors (ICMs), 29 Insurance, for adult congenital heart disease, 346 Interrupted aortic arch (IAA) clinical features, 111 clinical presentation, 174 definition, 174 diagnosis, 174–175 echocardiography, 174–175 epidemiology, 174 hemodynamics, 111 management, 111 neonatal surgical repair, 111 pathogenesis, 174 treatment, 175 types, 111 Intra-atrial re-entrant tachycardia (IART), 239 Intrathoracic pressure, 121 Intrauterine devices, 320 Intravenous immunoglobulin (IVIG), 268 Isometric exercise, 341 Isoproterenol Index Q for pulmonary hypertension, 261 for torsade de pointes, 242 Isotonic exercise, 341 IVIG (intravenous immunoglobulin), 268 J JET See Junctional ectopic tachycardia Jones criteria, 273t Jugular venous distention, Junctional bradycardia, 232 Junctional ectopic tachycardia (JET), 122 causes, 242 clinical features, 242 definition, 242 management, 242–243 postoperative, 242 K Karyotype analysis in congenital heart disease, 91 in Down syndrome, 83 in trisomy 13 syndrome, 83 in trisomy 18 syndrome, 83 in Turner syndrome, 84 Kawasaki disease, 265–270 atypical, 266–267 clinical criteria, 266t clinical presentation, 266–267 atypical disease, 266–267 C-reactive protein, 267 diagnosis, 267–268 epidemiology, 265 erythrocyte sedimentation rate, 267 laboratory findings, 268t noncardiac issues, 269–270 antipyretics, 270 infliximab treatment, 270 influenza vaccine, 270 vaccinations, 269–270 overview, 265 pericardial effusion, 269f prevalence, 265 RNA virus theory, 266 superantigen theory, 265–266 treatment acute therapy, 268 outpatient follow up, 268–269 Ketamine, 262 sedation and analgesia with, 330 in tetralogy of Fallot, 99 Korotkoff sounds, 295–296 L Lactate threshold, 31 LDL-C See Low-density lipoprotein cholesterol 12-lead electrocardiogram in acute rheumatic fever, 274 in Kawasaki disease, 268 in pericarditis, 281, 282f Left heart failure, 251–254 clinical presentation, 251 diagnosis, 251 differential diagnosis, 251 mechanical circulatory support, 253 prognosis, 253–254 transplantation, 253 treatment, 251–253 Left ventricular hypertrophy, 300–301, 301f Left ventricular noncompaction (LVNC), 258 Left ventricular systolic performance, 43–44 Left-sided obstructive lesions coarctation of the aorta, 69–70 hypoplastic left heart syndrome, 69 LEOPARD syndrome, 87 Lesions with obstruction to pulmonary blood flow, 196, 206–207 with obstruction to systemic blood flow, 196–197, 203–206 Fontan procedure, 205–206 Glenn procedure, 205 Hemi-Fontan procedure, 205 Norwood procedure, 203–205, 206f Levo-transposition of the great arteries cardiac defects associated with, 185t clinical presentation, 181t, 185 definition, 185 epidemiology, 185 outcome of, 186 outpatient management of, 186 physical examination, 181t treatment, 185–186 Lipid metabolism, 302–311, 305f Lipoproteins, 302–311 Liver transaminase, 310 Loeys-Dietz syndrome, 88 Long QT syndrome (LQTS), 26, 244, 322 Low cardiac output states, 118–121 Low cardiac output syndrome, 118–119 Low-density lipoprotein cholesterol (LDL-C), 302–311 medications for, lowering, 310t pharmacologic therapy, 309–311 Low-density lipoprotein receptor, 304, 310 LQTS (Long QT syndrome), 26, 244, 322 LVNC (Left ventricular noncompaction), 258 M Malalignment VSDs, 142 Malignant syncope, 243 Marfan syndrome, 87–88 aortic regurgitation, 161 Ghent criteria, 88t mitral regurgitation in, 167 MDI (Mental development index), 219 Mechanical assist devices, 120–121 Mediastinitis, 125 Mental development index (MDI), 219 Midazolam, 262, 330 Middle cerebral artery, 65–66, 66f Mitral inflow, 66 Mitral regurgitation clinical presentation, 167–168 definition, 167 diagnosis, 168 Ehlers-Danlos syndrome, 167 epidemiology, 167 hypertrophic cardiomyopathy, 256 Marfan syndrome, 167 pathogenesis, 167 treatment, 168 Mitral stenosis balloon valvuloplasty in, 166 clinical presentation, 166 definition, 165 diagnosis, 166 diuretics, 166 epidemiology, 165 361 362 Q Index Mitral stenosis (Cont.): pathogenesis, 165 treatment, 166–167 Mitral stenosis murmurs, 161 Mitral valve balloon dilation, 166 prolapse of, 168–169 regurgitation of, 167–168 rheumatic heart disease, 169 stenosis of, 165–167 Mitral valve prolapse (MVP) clinical presentation, 169 definition, 168–169 diagnosis, 169 epidemiology, 168–169 pathogenesis, 169 treatment, 169 M-mode echocardiography, 37–38, 37f–38f in fetal arrhythmia, 72 Monomorphic ventricular tachycardia causes, 240 clinical features, 240 definition, 240 implantable cardioverterdefibrillator in, 241 management, 241 MPI (Myocardial performance index), 45 Murmurs See also Heart murmurs Austin Flint, 161 blowing, 168 cardiac, 164 diastolic, 161, 165, 168 holosystolic, 9–10, 168, 256 of mitral stenosis, 161 systolic, 162, 164, 169 systolic ejection, 170, 176–177, 256 systolic regurgitant, 169 Mustard procedure, 183 MVP See Mitral valve prolapse Myocardial bridging, 18 Myocardial deformation imaging, 45–46 Myocardial iron assessment, 54 Myocardial ischemia, 18 Myocardial performance index (MPI), 45 Myocardial scarring, 53–54 Myocarditis arrhythmias and, 245 cardiac magnetic resonance in, 53–54 chest pain in, 19 chest x-ray in, 278 clinical presentation, 278 collagen vascular disease, 245 definition, 277 diagnosis, 245, 278, 279f differential diagnosis, 278t 2-dimensional echocardiogram in, 278 Doppler echocardiogram in, 278 electrocardiogram in, 278 epidemiology, 277 magnetic resonance imaging in, 279 management, 245 pathogenesis, 278 prognosis, 280 symptoms and signs, 278t transthoracic echocardiography in, 278–279 treatment, 279–280 Myocardium, fetal, 61–62 N Naxos disease, 258 Neoaorta, 204 Neoaortic root dilation, 183 Neonatal atrial flutter, 239 Neonatal Ross procedure, 109 Neurally mediated syncope, 14 Newborns aortic coarctation, 172–174 aortic regurgitation, 160–162 cardiopulmonary bypass in, 117–118 critical heart disease cyanotic, 96t, 98–108 management, principles of, 97–98 as shock, 108–114 Ebstein anomaly, 170–172 hypertension in, 299–300 interrupted aortic arch, 174–175 mitral regurgitation, 167–168 mitral stenosis, 165–167 mitral valve prolapse, 168–169 peripheral pulmonary stenosis, 176–177 pulmonary regurgitation, 164–165 subvalvar aortic stenosis, 159–160 subvalvar pulmonary stenosis, 163–164 supravalvar aortic stenosis, 160 supravalvar pulmonary stenosis, 164 valvar aortic stenosis, 155–159 valvar pulmonary stenosis, 162–163 Nitric oxide, inhaled, 122 NKX2.5 gene in atrial septal defects, 138 in HLHS, 198 in nonsyndromic single-gene disorders, 89 Nonsyndromic single-gene disorders, 89 Noonan syndrome, 87 balloon dilation, 163 valvar pulmonary stenosis with, 162 Norwood procedure antithrombotic prophylaxis and, 326 for hypoplastic left heart syndrome, 203–205, 206f O Obesity assessment of risk factors, 293f body mass index, 289–294 percentile values for, 294 clinical presentation, 290 definition, 289–290 diagnosis, 294 dyslipidemia and, 309 epidemiology, 290 hypertension and, 297, 302 prevalence, 290, 290f prevention, 290–294 related disorders, history and physical examination, 295t related disorders, screening and additional testing, 296t screening, 290–294 treatment, 294 Ohm’s law, Ostium primum defect, 138 Ostium secundum ASDs, 138, 140 Oxygen, in neonatal with critical heart disease management, 97 Oxygen saturation, Oxygen therapy, 137 Index Q P PAC (Premature atrial contractions), 73, 233, 233t Pain chest See Chest pain management, 124–125 PA/IVS See Pulmonary atresia with intact ventricular septum Palpation, Palpitations clinical presentation, 12 definition of, 12 diagnosis, 12, 13f differential diagnosis, 13t epidemiology, 12 guidelines, 13 pathogenesis, 12 treatment, 13 Paroxysmal supraventricular tachycardia catheter ablation, 237 causes, 236 clinical features, 236–237 definition, 236 management, 237 vagal maneuvers in children, 237t Past medical history, Patau syndrome, 83 Patent ductus arteriosus (PDA), 146–149 Amplatzer duct occluder, 128, 131f Char syndrome, 147 clinical presentation, 148 definition, 146 epidemiology, 146–147 evaluation, 148 pathogenesis, 147–148 summary, 150t treatment, 148–149 catheter-based intervention in, 148–149 medical management, 148 postoperative complications, 149 postoperative follow-up, 149 surgical intervention, 148–149 Patent foramen ovale, 180 Pathologic murmurs, Patient history birth, 3–4 family, past medical, present illness, PDA See Patent ductus arteriosus PDI (Psychomotor development indices), 219 Peabody developmental motor scale, 219 Pediatric echocardiography See also Echocardiography indications for referral, 41, 43t left ventricular systolic performance, 43–44 measurement adjustments for growth, 42 myocardial deformation imaging, 45–46 myocardial performance index, 45 right ventricular systolic performance, 44–45 standard views for, 41–42 Pediatric electrocardiogram See also Electrocardiogram basic concepts and approach to, 23–27 heart rate, 24 leads, 23–24, 24f limb leads, 25f overview, 23 P wave, 24–25 PR interval, 25–26 QRS axis, 24 QT interval, 26 ST segment, 26–27 T wave, 26–27 Penicillin V, for acute rheumatic fever, 275, 277 Percussion, 6–7 Percutaneous balloon valvuloplasty, 109, 110f Pericarditis, 280–283 acute, 280 chest pain in, 18 chronic, 280 clinical presentation, 280 constrictive, 280 definition, 280 diagnosis, 280–282, 282f differential diagnosis, 280, 281t epidemiology, 280 etiologies, 281t medications, 283f pathogenesis, 280 pericardiocentesis, 283 recurrent, 280 symptoms and signs, 281t treatment, 282–283 Peripheral blood cultures, 285–286 Peripheral pulmonary stenosis clinical presentation, 176–177 definition, 176 diagnosis, 177 epidemiology, 176 pathogenesis, 176 treatment, 177 PGE1 See Prostaglandin E1 Pharmacologic therapy bile salt sequestrants, 309 cholesterol absorption inhibitors, 311 hemodynamic monitoring, 118–120 HMG-CoA reductase inhibitors, 309–311 for torsade de pointes, 242 Phase velocity mapping, 49, 51f–53f, 52–53 Physical examination auscultation in, 7–8 blood pressure, 4–5 in dextro-transposition of the great arteries, 102 heart murmurs in continuous, 11 diastolic, 11 systolic, 8–10 inspection, 5–6 palpation in, percussion, 6–7 in total anomalous pulmonary venous connection, 105 Pleural effusions, 121 Point of maximal impulse (PMI), 5–6 Polymorphic ventricular tachycardia causes, 241 clinical features, 241 definition, 241 management, 241 Postcatheterization patient, care basics, 127 complications, 127–131 interventional procedures, 127–130 Postoperative infection, 125 Postsurgical patient, care for acute renal failure, 124 for arrhythmias, 122–123 cardiopulmonary bypass, 117–118 for cardiopulmonary interactions, 121 for central nervous system injuries, 123–124 363 364 Q Index Postsurgical patient, care (Cont.): hemodynamic monitoring, 118–121 low cardiac output states, 118–121 pain management, 124–125 postoperative infection, 125 preoperative stabilization, 117 preparation for discharge, 126–127 for pulmonary hypertension, 121–122 for respiratory distress, 121 sedation, 124–125 transfer to the general care ward, 125–126 Postural orthostatic tachycardia syndrome (POTS), 15 POTS (Postural orthostatic tachycardia syndrome), 15 PR interval, 25–26 Precedex (Dexmedetomidine), 330–331 Pregestational diabetes, 77–78 Preload, Premature atrial contractions (PAC), 73, 233, 233 Premature ventricular contraction (PVC), 233 Preoperative planning, 337–338 Preoperative stabilization, 117 Presedation evaluation, 330 Preventive cardiology dyslipidemia, 302–311 hypertension, 294–302 obesity, 289–294 overview, 289 Primary hypertension, 297 Procainamide, 238, 240–241, 243 Progesterone-based contraception, 320 Propofol, 262, 331 Prostaglandin E1 (PGE1) in neonatal with critical heart disease management, 97 for pulmonary atresia with intact ventricular septum, 71 for pulmonic stenosis, 70 side effects, 118t Psychomotor development indices (PDI), 219 Pulmonary artery stenosis, 176–177 Pulmonary atresia with intact ventricular septum (PA/IVS), 70–71 clinical features, 100 diagnosis, 209 management, 100 neonatal catheter intervention, 100–101 overview, 100 palliative procedures, 101 in single-ventricle congenital heart disease, 208–209 treatment, 209 Pulmonary circulation (Qp), 97–98, 98t, 112–113 Pulmonary hypertension, 3, 121–122, 258–263 anesthetic agents, 262 BMPR2, 259 chest pain in, 18 classification of, 259t clinical presentation, 259–260, 260t definition, 258 diagnosis, 260, 260t emergent therapy for, 263t epidemiology, 259 incidence of, 259t pathophysiology, 259 prognosis, 262 sedatives, 262 treatment, 261–262, 262t vasodilators, 261 Pulmonary regurgitation clinical presentation, 165 definition, 164 diagnosis, 165 epidemiology, 164 pathogenesis, 164 tetralogy of Fallot with, 164 treatment, 165 ventricular arrhythmias with, 165 ventricular dilatation with, 165 Pulmonary stenosis balloon dilation, 129 clinical features, 107–108 hemodynamics, 107 management, 108 neonatal catheter intervention, 108 neonatal surgical repair, 108 overview, 107 Pulmonary valve regurgitation, 164–165 stenosis of, 162–164 Pulmonic stenosis, 70 Pulses, Pulsus paradoxus, 6, 280 PVC (Premature ventricular contraction), 233 Q 22q11.2 microdeletion syndrome, 84–85, 91 Qp (Pulmonary circulation), 97–98, 98t, 112–113 QRS axis, 24 QRS duration, 26 Qs (Systemic circulation), 97–98, 98t, 112–113 QT interval, 26 QT interval prolongation, 322–323, 323t R Rashkind procedure, 103, 103f, 180 RCM See Restrictive cardiomyopathy Regurgitation See Aortic regurgitation; Mitral regurgitation; Pulmonary regurgitation; Tricuspid regurgitation Reintervention in adult life, 336–338 RER (Respiratory exchange ratio), 30–31 Respiration, Respiratory exchange ratio (RER), 30–31 Respiratory failure, 121 Respiratory gases analyzers, 34 Respiratory rate, Respiratory syncytial virus (RSV) prophylaxis, 321 Restrictive cardiomyopathy (RCM), 257–258 clinical presentation, 257 described, 257 diagnosis, 257 differential diagnosis, 257 diuretics, 257 etiologies of, 252t pathogenesis, 257 treatment, 257–258 Rheumatic fever See Acute rheumatic fever Rhythm disturbances, 340t Ribonucleic acid (RNA) virus theory, 266 Right ventricular systolic performance, 44–45 Right-sided obstructive lesions (RSOLs), 70–71 Ross procedure, 109, 159 Rub, 7–8 S Sacrococcygeal teratoma (SCT), 67, 68f SCT (Sacrococcygeal teratoma), 67, 68f Secondary hypertension, 297–298, 298t Sedation, 124–125 cardiac catheterization, 127 in congenital heart disease Index Q goals of, 329–330 medication for, 330–331 overview, 328–329 levels of, 329t Semilunar valves aortic valve, 155–162 regurgitation, 160–162 stenosis, 155–160 pulmonary valve, 162–165 regurgitation, 164–165 stenosis, 162–164 Senning operation, 183, 183f Shock in neonates, 108–114 coarctation of aorta, 109–110 critical aortic stenosis, 108–109 hypoplastic left heart spectrum, 111–114 interrupted aortic arch, 110–111 Shortening fraction, 43 Shunt lesions arteriovenous malformations, 149–152 atrial septal defects, 138–140 clinical manifestations, 135–136 common atrioventricular canal defects, 145–146 oxygen therapy for, 137 patent ductus arteriosus, 146–149 pathophysiology of, 135 red flag symptoms, 136t treatment principles, 136–137 ventricular septal defects, 140–145 Single-ventricle congenital heart disease, 195–211 causing obstruction to pulmonary blood flow, 197t causing obstruction to systemic blood flow, 197t clinical presentation, 198–200 fetal diagnosis of, 198–200 neonatal, 200 in the older infant or young child, 200 definition, 195–196 differential diagnosis of, 200–201, 201t double-inlet left ventricle, 209 exercise capacity in patients, 210 Fontan procedure, 202–203 genetic(s), 197–198 heterotaxy syndrome, 196, 198, 200 hyperoxia test, 200–201 hypoplastic left heart syndrome, 195–210 hypoplastic right heart syndrome, 208–209 incidence of, 197 lesions with obstruction to pulmonary blood flow, 196, 206–207 lesions with obstruction to systemic blood flow, 196–197, 203–206 lesion-specific pathways, 203–210 double-inlet left ventricle, 209 lesions with obstruction to pulmonary blood flow, 206–207 lesions with obstruction to systemic blood flow, 203–206 pulmonary atresia with intact ventricular septum, 208–209 tricuspid atresia, 207–208 neurodevelopmental outcomes, 210 pathogenesis, 197–198 prevalence of, 197 quality of life, 210 signs and symptoms of, 199t treatment, 201–203 Sinus arrhythmia, Sinus bradycardia, 231–232 Sinus tachycardia, 236 Sinus venosus ASDs, 138 Social cognition, 221 Spironolactone, 136 Spontaneous pneumothorax, 17–18 Sports, 340–341, 342t–345t See also Exercise St Jude’s Regent Mechanical Heart Valve, 166–167, 167f ST segment abnormalities, 26–27 Static exercise, 341 Statins, 309–311, 311f Stenosis aortic stenosis, 155–160 of mitral valve, 165–167 of pulmonary artery, 176–177 of pulmonary valve, 162–164 of tricuspid valve, 169–170 Stethoscope, Strain imaging, 45–46 Stroke volume, 2–3 Subclavian flap aortoplasty, 110 Subvalvar aortic stenosis clinical presentation, 159 definition, 159 diagnosis, 159 epidemiology, 159 pathogenesis, 159 treatment, 160 Subvalvar pulmonary stenosis clinical presentation, 164 definition, 163–164 diagnosis, 164 epidemiology, 163–164 pathogenesis, 164 treatment, 164 Superantigen theory, 265–266 Supravalvar aortic stenosis clinical presentation, 160 definition, 160 diagnosis, 160 epidemiology, 160 pathogenesis, 160 treatment, 160 Supravalvar pulmonary stenosis, 183 clinical presentation, 164 definition, 164 diagnosis, 164 epidemiology, 164 pathogenesis, 164 treatment, 164 Supraventricular tachycardia (SVT), 15, 73, 236 Survival to adulthood, 335–349 arrhythmias, 338 atrial fibrillation, 338 atrial flutter, 338 burden of ongoing care, 336–338 complex, 337t employment, 346 exercise, 338–341 hospitalization, 347 insurance, 346 intervention, 337 mental health, 346–347 preoperative planning, 337–338 psychological issues, 347 quality of life, 346–347 reintervention in adult life, 336–338 reproduction, 341 sexual dysfunction, 347 sexuality, 341 sports, 340–341 transitions of care, 335–336 ventricular tachycardia, 338 365 366 Q Index SVT (Supraventricular tachycardia), 15, 73, 236 Syncope benign versus malignant, 243 cardiac, 14–15 clinical presentation, 14 definition of, 14 diagnosis, 16, 16f differential diagnosis, 15t epidemiology, 14 guidelines, 17 neurally mediated, 14 noncardiac, 15 pathogenesis, 14 treatment, 16–17 Systemic circulation (Qs), 97–98, 98t, 112–113 Systolic ejection murmur(s), 157, 170, 176–177, 256 Systolic murmurs, 8–11, 169 T TA See Truncus arteriosus Tachyarrhythmias, 236–243 arterial switch operation after, 184 atrial fibrillation, 239–240 atrial flutter, 239 ectopic atrial tachycardia, 238–239 junctional ectopic tachycardia, 242–243 paroxysmal supraventricular tachycardia, 236–237 sinus tachycardia, 236 supraventricular tachycardia, 236 ventricular fibrillation, 241 ventricular tachycardias, 240–242 Wolff-Parkinson-White syndrome, 237–238, 237t Tachycardia ectopic atrial, 238–239 junctional ectopic, 242–243 paroxysmal supraventricular, 236–237 sinus, 236 supraventricular, 15 ventricular, 15, 240–242 TAPVC See Total anomalous pulmonary venous connection TAPVR (Total anomalous pulmonary venous return), 221–222 Targeted mutation screening and sequencing, 91–92 TdP See Torsades de pointes Temperature, Tet spells, 99, 187–188 Tetralogy of Fallot (TOF), 70 anatomic features of, 186, 186f associated cardiac defects in, 188, 188t chest x-ray in, 181t clinical features, 99, 181t clinical presentation, 187–188 definition, 186 electrocardiogram in, 181t epidemiology, 186–187 and genetic syndromes, 187 hemodynamics, 98–99 hypercyanotic spells in, 187–188 hypoxic spells in, 99 long-term morbidity after repair, 189t management, 99 neonatal interventions, 99 neonatal surgical repair, 99, 100f outpatient management of, 189–190 overview, 98 pathophysiology, 187 peripheral pulmonary stenosis with, 176 postoperative management, 99–100 rudimentary pulmonary valve with, 189–190 subvalvar pulmonary stenosis with, 164 treatment complete repair of, 188, 188f–189f palliative surgery, 189 surgical outcome, 189 transannular patch repair, 188 Thoracic roentgenogram, 108–111 Thrills, Tissue Doppler echocardiography, 40, 41f TOF See Tetralogy of Fallot Torsades de pointes (TdP) causes, 242 clinical features, 242 definition, 242 drugs with risk of, 323t management, 242 Total anomalous pulmonary venous connection (TAPVC), 103–106 clinical features, 105 hemodynamics, 105 management, 105–106 neonatal surgical repair, 106 postoperative management, 106 Total anomalous pulmonary venous return (TAPVR), 221–222 Total cholesterol, 302–311 Transcatheter closure, 140, 149 Transcatheter embolization, 152 Transesophageal echocardiography, 46 in atrial fibrillation, 240 in atrial flutter, 239 in cardiac catheterization lab, 46 contraindication, 46 in endocarditis, 286 indications for, 43t, 46 in mitral stenosis, 167f in operating room, 46 Transitions of care for congenital heart disease, 335–336 recommendations for, 336t Transposition of the great arteries, 179–186, 218, 218f, 219, 221–222 See also specific types Transtelephonic event recorders, 27–28, 28f Transthoracic echocardiography in arteriovenous malformations, 151 in atrial septal defects, 139–140 in common atrioventricular canal defects, 146 in endocarditis, 286 indications for, 43t in myocarditis, 278–279 in patent ductus arteriosus, 148 in pericarditis, 281, 283f in ventricular septal defect, 143 Traumatic pain, 17 Tricuspid atresia clinical features, 106 Fontan procedure, 207–208 hemodynamics, 106 management, 106 palliative procedures, 106–107 in single-ventricle congenital heart disease, 207–208 treatment, 208 ventricular septal defect with, 71 Tricuspid inflow, 66 Tricuspid regurgitation, 172 Tricuspid valve Ebstein anomaly, 170–172 regurgitation of, 172 stenosis of, 169–170 Triglycerides, 302–311 Index Q Trisomy 13 syndrome, 83 Trisomy 18 syndrome, 83 Trisomy 21 syndrome, 82–83 Truncus arteriosus (TA) cardiac defects associated with, 191t classification of, 190, 191t clinical presentation, 181t, 191 epidemiology, 190 long-term management of, 192 outpatient management of, 192 pathophysiology, 190 treatment, 191–192 TTTS (Twin-twin transfusion syndrome), 65, 68–69 Turner syndrome, 83–84 aortic coarctation, 172–173 hypertension and, 298, 300 Twin-twin transfusion syndrome (TTTS), 65, 68–69 U Ultrasonography, 121, 198 Umbilical artery, 64, 64f Umbilical vein, 64, 64f V VABS (Vineland adaptive behavior scale), 219–220, 222 VADs (Ventricular assist devices), 120–121, 253 Vagal maneuvers, 237, 237t Valvar aortic stenosis balloon valvuloplasty in, 159 clinical presentation, 157 definition, 155 diagnosis, 157–158 epidemiology, 155 pathogenesis, 155 treatment, 158–159 Valvar pulmonary stenosis balloon dilation, 163 balloon valvuloplasty in, 163, 163f clinical presentation, 162 definition, 162 diagnosis, 163 dysplastic valve, 162 epidemiology, 162 pathogenesis, 162 treatment, 163 Valvuloplasty, balloon See Balloon valvuloplasty Vascular rings, 175f clinical presentation, 176 definition, 175 diagnosis, 176 epidemiology, 175–176 pathogenesis, 176 treatment, 176 Vasculature aortic coarctation, 172–174 interrupted aortic arch, 174–175 peripheral pulmonary stenosis, 176–177 vascular rings, 175–176 Vasodepressor syncope, 14 VATER association, 88–89 367 Velocardiofacial syndrome, 74, 84–85 Ventilatory anaerobic threshold, 31 Ventricular arrhythmias arrhythmogenic right ventricular dysplasia/cardiomyopathy, 245 Brugada syndrome, 245 pulmonary regurgitation with, 165 in tetralogy of Fallot, 190 Ventricular assist devices (VADs), 120–121, 253 Ventricular diastole, Ventricular fibrillation, 241 Ventricular septal defect (VSD), 3, 140–145 clinical presentation, 143 physical examination, 143 symptoms, 143 definition, 142 dextro-transposition of the great arteries with, 179–181 diagnosis, 143 embryology, 140–141 epidemiology, 142 pathogenesis, 142 summary of, 144t treatment, 143–145 medical management, 143–144 postoperative concerns, 144–145 postoperative follow-up, 144–145, 144t surgical intervention, 144 Ventricular systole, Ventricular tachycardia, 15 in adult congenital heart disease, 338 monomorphic, 240–241 polymorphic, 241 torsade de pointes, 242 ventricular fibrillation, 241 Vineland adaptive behavior scale (VABS), 219–220, 222 Vital signs, 4–5 VSD See Ventricular septal defect W Wandering atrial pacemaker, 232–233 WeeFIM (Functional Independence Measure for Children), 219–220 White coat hypertension, 297 Williams syndrome, 85–86 hypertension and, 298, 300 supravalvar aortic stenosis with, 160 supravalvar pulmonary stenosis with, 164 Wolff-Parkinson-White (WPW) syndrome, 26–27, 185 “antidromic” reciprocating tachycardia, 238 atrial fibrillation, 238–240 atrioventricular re-entrant tachycardia, 237–238 causes, 238 clinical features, 238 definition, 237–238 management, 238 X XMR, 55 Z Z-scores, 42 ... infrequently used drugs PEDIATRIC PRACTICE Cardiology Marie Murphy Gleason Division of Cardiology The Children’s Hospital of Philadelphia Philadelphia, Pennsylvania Jack Rychik Division of Cardiology The... Division of Pediatric Cardiology Professor of Pediatrics The Children’s Hospital of Philadelphia The University of Pennsylvania School of Medicine Philadelphia, Pennsylvania Preface Pediatric cardiology. .. medications that prolong QTc Refer to pediatric cardiologist Refer to pediatric cardiologist Refer to pediatric cardiologist or pediatric cardiac electrophisiologist Refer to pediatric cardiologist Sinus