Moss and Adams' Heart Disease in Infants, Children, and Adolescents, Including the Fetus and Young Adult

Author: Hugh Allen, MD, FAHA, FACC, FAAP, Robert Shaddy, MD, Daniel Penny, MD, Timothy Feltes, MD, Frank Cetta, MD, FACC, FASE
Affiliation:
Publisher: Lippincott Williams & Wilkins
Publication Date: 2016
ISBN 10: 1496300246
ISBN 13: 9781496300249
eISBN: 9781496300263
Edition: 9th

Description:

The 9th edition of the gold standard in pediatric cardiology offers up-to-date, comprehensive coverage of this challenging and rapidly changing field. New chapters based on reader requests, two new associate editors, new contributing authors, and an enhanced digital version of the text make Moss and Adams’ Heart Disease in Infants, Children, and Adolescents the reference you’ll turn to when caring for patients. From cover to cover, this award-winning two-volume resource provides the authoritative, state-of-the-art information you need when caring for young patients with heart disease. New, reader-requested chapters cover Development of Myocardial Structure and Function, Global Challenge of Heart Disease, Impact of Congenital Heart Disease on Other Organ Systems, Non-Compaction Cardiomyopathy, Beyond the Cardiac Device, and Transition to Adult Care. All chapters have been updated and revised, including vital new information on genetics and embryo development. Two new associate editors, Drs. Daniel J. Penny and Frank Cetta, offer fresh perspectives in their areas of expertise. Enhanced digital access includes the full text; bonus question and answer sections similar to those on the pediatric cardiology board examination; videos of echoes, angiograms, aortic arch anomalies, open-heart operations, and more; and phonocardiograms with accompanying audio of various heart sounds and murmurs. Now with the print edition, enjoy the bundled interactive eBook edition, which can be downloaded to your tablet and smartphone or accessed online and includes features like: Complete content with enhanced navigation Powerful search tools and smart navigation cross-links that pull results from content in the book, your notes, and even the web Cross-linked pages, references, and more for easy navigation Highlighting tool for easier reference of key content throughout the text Ability to take and share notes with friends and colleagues Quick reference tabbing to save your favorite content for future use

Table of Contents

Front Matter

  • ABOUT
  • DEDICATION
  • CONTRIBUTORS
  • PREFACE
  • TRIBUTE TO MOSS, ADAMS, & EMMANOUILIDES
  • HISTORY OF PEDIATRIC CARDIOLOGY
  • THE DEVELOPMENT OF PEDIATRIC CARDIOLOGY: HISTORICAL MILESTONES

SECTION I From the Gene to the Neonate

    1: Development of the Heart: Morphogenesis, Growth, and Molecular Regulation of Differentiation

    • INTRODUCTION
    • FORMATION AND LOOPING OF THE HEART TUBE
    • CHAMBER FORMATION AND VENTRICULAR SEPTATION
    • DEVELOPMENT OF THE VENOUS POLE OF THE HEART
    • DEVELOPMENT OF THE ATRIOVENTRICULAR JUNCTION
    • DEVELOPMENT OF THE ARTERIAL POLE OF THE HEART
    • DEVELOPMENT OF THE EPICARDIUM AND CORONARY ARTERIES
    • REFERENCES

    2: Epidemiology and Prevention of Congenital Heart Defects

    • FROM EPIDEMIOLOGY TO PREVENTION
    • OUTCOMES AS INCENTIVE FOR ACTION: COMMON, COSTLY, AND CRITICAL
    • EVIDENCE FOR ACTION: EPIDEMIOLOGY OF RISK AND CAUSES
    • STRATEGIES: EPIDEMIOLOGY OF PREVENTION
    • CONCLUDING COMMENTS
    • REFERENCES

    3: Genetic Aspects of Congenital Heart Defects

    • METHODS AND MECHANISMS
    • PATTERNS OF INHERITANCE AND FAMILIAL RISKS
    • GENETIC SYNDROMES
    • GENETICS OF SPECIFIC CARDIAC LESIONS
    • GENETIC EVALUATION AND COUNSELING
    • CONCLUSIONS
    • REFERENCES

    4: Development of Myocardial Structure and Function

    • INTRODUCTION
    • MYOCARDIAL STRUCTURE
    • CARDIAC FIBROBLASTS AND THE EXTRACELLULAR MATRIX
    • CARDIAC MYOCYTES
    • MITOCHONDRIA AND METABOLISM
    • CELL–EXTRACELLULAR MATRIX AND CELL–CELL INTERACTIONS
    • CORONARY VASCULATURE
    • MYOCARDIAL GROWTH AND REMODELING
    • MYOCARDIAL FUNCTION
    • LV CHAMBER FUNCTION
    • PRESSURE–VOLUME LOOP ASSESSMENT OF CARDIAC FUNCTION
    • EMERGING CONCEPTS AND CONCLUSION
    • REFERENCES

    5: Fetal and Perinatal Cardiology

    • INTRODUCTION
    • IMPLICATIONS OF FETAL DIAGNOSIS OF CARDIOVASCULAR DISEASE
    • FETAL CARDIOVASCULAR CIRCULATION
    • EARLY GESTATION FETAL CARDIAC EVALUATION AND SCREENING FOR FETAL CARDIOVASCULAR DISEASE
    • INDICATIONS FOR FETAL ECHOCARDIOGRAPHY
    • THE FETAL ECHOCARDIOGRAM
    • ASSESSMENT OF CARDIOVASCULAR PERFORMANCE
    • CONGENITAL HEART LESIONS
    • OTHER FETAL CARDIOVASCULAR CONDITIONS
    • EXTRACARDIAC CONDITIONS WITH CARDIAC MANIFESTATIONS
    • FETAL ARRHYTHMIA
    • GENETIC DISORDERS AND ASSOCIATIONS WITH CHD
    • FETAL CARDIAC INTERVENTION
    • NOVEL FETAL IMAGING MODALITIES
    • PARENTAL COUNSELING
    • ACKNOWLEDGEMENTS
    • REFERENCES

SECTION II Structure and Function of the Cardiovascular System

    6: Cardiac Anatomy and Examination of Cardiac Specimens

    • MEDIASTINUM
    • PERICARDIUM
    • EXTERNAL TOPOGRAPHY
    • GREAT VEINS
    • ATRIA
    • ATRIOVENTRICULAR VALVES
    • VENTRICLES
    • SEMILUNAR VALVES
    • GREAT ARTERIES
    • CORONARY CIRCULATION
    • CARDIAC CONDUCTION SYSTEM
    • EXAMINATION OF CARDIAC SPECIMENS
    • REFERENCES

    7: Classification and Terminology of Cardiovascular Anomalies

    • PERSPECTIVES ON NOMENCLATURE
    • SEQUENTIAL SEGMENTAL ANALYSIS
    • CARDIAC POSITION AND APICAL DIRECTION
    • VISCERAL SIDEDNESS (SITUS)
    • MORPHOLOGY OF CARDIAC SEGMENTS
    • POSITIONS OF CARDIAC SEGMENTS
    • CONNECTIONS OF CARDIAC SEGMENTS
    • REFERENCES

    8: Cardiovascular Physiology

    • INTRODUCTION
    • VENTRICULAR FUNCTION
    • THE TRANSLATION OF VENTRICULAR PRESSURE-WORK TO FLOW-WORK IN THE GREAT ARTERIES
    • CONTROL OF FLOW IN THE SYSTEMIC CIRCULATION
    • CONTROL OF FLOW IN THE PULMONARY CIRCULATION
    • COUPLING BETWEEN THE CIRCULATION AND TISSUE METABOLISM
    • THE PHYSIOLOGY OF THE DEVELOPING CIRCULATION
    • ACKNOWLEDGMENT
    • REFERENCES

SECTION III Diagnostic and Therapeutic Methods

    9: History and Physical Examination

    • HISTORY
    • PHYSICAL EXAMINATION
    • OBSERVATION/INSPECTION
    • PALPATION
    • AUSCULTATION
    • INNOCENT MURMURS
    • REFERENCES

    10: Exercise Screening and Sports Participation

    • PHYSICAL ACTIVITY AND EXERCISE
    • PREPARTICIPATION SCREENING FOR UNDIAGNOSED CARDIAC CONDITIONS
    • SPECIFIC CONGENITAL CARDIAC LESIONS
    • DEFECTS ASSOCIATED WITH SUDDEN CARDIAC DEATH IN ATHLETES
    • REFERENCES

    11: Exercise Testing

    • BASIC EXERCISE PHYSIOLOGY
    • EXERCISE TESTING METHODS
    • MAXIMAL AEROBIC POWER
    • CARDIAC RESPONSES TO ACUTE BOUTS OF EXERCISE
    • REGULATION OF CIRCULATION DURING EXERCISE
    • VENTILATORY RESPONSE TO EXERCISE
    • TECHNIQUES OF SPECIFIC MEASUREMENTS
    • REFERENCES

    12: Echocardiography: Basic Principles and Imaging

    • HISTORY
    • PHYSICS OF 2-D ECHOCARDIOGRAPHY
    • THE EXAMINATION
    • TRANSESOPHAGEAL ECHOCARDIOGRAPHY
    • STRESS ECHOCARDIOGRAPHY
    • THREE-DIMENSIONAL ECHOCARDIOGRAPHY
    • ECHOCARDIOGRAPHIC EVALUATION OF INTERVENTIONAL PROCEDURES
    • CONTRAST ECHOCARDIOGRAPHY
    • RESEARCH ECHOCARDIOGRAPHY
    • QUALITY ASSURANCE
    • THE FUTURE
    • ACKNOWLEDGMENTS
    • REFERENCES

    13: Echocardiographic Assessment of Cardiac Dimensions, Cardiac Function, and Valve Function

    • QUANTIFICATION OF CHAMBER DIMENSIONS AND CARDIAC STRUCTURES
    • QUANTIFICATION OF THE LEFT VENTRICLE
    • QUANTIFICATION OF THE RIGHT VENTRICLE
    • QUANTIFICATION OF THE UNIVENTRICULAR HEART
    • EVALUATION OF SYSTOLIC VENTRICULAR FUNCTION
    • ASSESSMENT OF VALVE FUNCTION
    • DIASTOLIC VENTRICULAR FUNCTION
    • ABNORMALITIES OF LEFT VENTRICULAR DIASTOLIC FUNCTION
    • EVALUATION OF VENTRICULAR SYNCHRONY
    • ECHOCARDIOGRAPHIC ASSESSMENT OF THE ATRIOVENTRICULAR DELAY
    • ASSESSMENT OF INTERVENTRICULAR DYSSYNCHRONY
    • ASSESSMENT OF INTRAVENTRICULAR DYSSYNCHRONY
    • CORONARY ARTERY PHYSIOLOGY
    • REFERENCES

    14: Magnetic Resonance Imaging

    • INDICATION FOR MAGNETIC RESONANCE IMAGING EVALUATION OF CONGENITAL HEART DISEASE
    • MAGNETIC RESONANCE IMAGING TECHNIQUES
    • SEDATION AND MONITORING
    • PATIENT PREPARATION
    • SAFETY
    • SUPERVISION AND INTERPRETATION OF MRI EXAMINATION
    • CLINICAL APPLICATIONS
    • EXAMPLES OF CLINICAL APPLICATIONS OF CMR
    • REFERENCES

    15: Cardiac Computed Tomography in Children with Congenital Heart Disease

    • INTRODUCTION
    • OVERVIEW OF CT TECHNOLOGY
    • CTA TECHNIQUES AND PROTOCOLS
    • PATIENT PREPARATION
    • INTRAVENOUS CONTRAST AGENTS AND INJECTION PROTOCOL
    • IMAGE POST-PROCESSING
    • ARTIFACTS IN CARDIAC CT
    • RADIATION REDUCTION ON CT
    • CHOICE OF CT VERSUS MR FOR PEDIATRIC CARDIAC IMAGING
    • CLINICAL APPLICATIONS OF PEDIATRIC CARDIAC CT
    • CONCLUSION
    • REFERENCES

    16: Cardiac Catheterization and Angiography

    • DIAGNOSTIC CARDIAC CATHETERIZATION AND ANGIOGRAPHY
    • ANGIOGRAPHY
    • ACKNOWLEDGMENTS
    • REFERENCES

    17: Therapeutic Cardiac Catheterization

    • INTRODUCTION
    • ADVERSE EVENTS AND QUALITY IMPROVEMENT
    • THE INTERVENTIONAL ARMAMENTARIUM
    • HOW TO CREATE, ENLARGE, AND MAINTAIN INTRA-ATRIAL COMMUNICATIONS
    • BALLOON AORTIC VALVULOPLASTY
    • BALLOON PULMONARY VALVULOPLASTY
    • PERFORATION OF THE ATRETIC PULMONARY VALVE
    • TRANSCATHETER TREATMENT OF STENOTIC AV VALVES
    • TRANSCATHETER MANAGEMENT OF COARCTATION, RE-COARCTATION, AND AORTIC ARCH OBSTRUCTIONS
    • REHABILITATION OF (BRANCH) PULMONARY ARTERY STENOSIS
    • TRANSCATHETER MANAGEMENT OF PULMONARY VEIN STENOSES
    • TRANSCATHETER MANAGEMENT OF SYSTEMIC VEIN STENOSES
    • TRANSCATHETER THERAPY AFTER “ATRIAL SWITCH”
    • DEVICE CLOSURE OF SEPTAL DEFECTS
    • TRANSCATHETER OCCLUSION OF PATENT DUCTUS ARTERIOSUS
    • CLOSURE OF OTHER PERSISTENT OR ABNORMAL VASCULAR COMMUNICATIONS
    • RETRIEVAL OF FOREIGN BODIES
    • TRANSCATHETER PULMONARY VALVE REPLACEMENT
    • HYBRID PROCEDURES
    • SUMMARY
    • REFERENCES

SECTION IV Electrophysiology

    18: Development and Functional Maturation of the Cardiac Conduction System

    • RECOGNITION OF THE CONDUCTION TISSUES IN THE POSTNATAL HEART
    • THE SINUS NODE
    • THE AV CONDUCTION AXIS AND VENTRICULAR CONDUCTION SYSTEM
    • DEVELOPMENTAL ASPECTS OF CARDIAC ELECTROPHYSIOLOGY
    • DEVELOPMENT AND MODULATION OF CARDIAC AUTOMATICITY AND CONDUCTION BY AUTONOMIC NERVOUS SYSTEM
    • THE CONDUCTION SYSTEM IN CONGENITALLY MALFORMED HEARTS
    • REFERENCES

    19: The Normal Electrocardiogram

    • THE HISTORY OF ELECTROCARDIOGRAPHY
    • PRINCIPLES AND TECHNICAL CONSIDERATIONS IN RECORDING THE ELECTROCARDIOGRAM
    • HOW TO READ A PEDIATRIC ELECTROCARDIOGRAM
    • THE NORMAL ELECTROCARDIOGRAM
    • GUIDELINES AND INDICATIONS
    • REFERENCES

    20: Cardiac Channelopathies, Syncope, and Sudden Death

    • THE PREVALENCE AND CAUSES OF SUDDEN DEATH IN THE YOUNG
    • AUTOPSY-NEGATIVE SUDDEN UNEXPLAINED DEATH
    • THE CARDIAC CHANNELOPATHIES
    • DISTINGUISHING FEATURES OF “COMMON YET BENIGN” SYNCOPE FROM “SUDDEN DEATH WARNING SIGN” SYNCOPE
    • REFERENCES

    21: Electrophysiology Studies and Electrophysiologic Therapeutic Catheterization

    • ELECTROPHYSIOLOGIC STUDIES: DIAGNOSTIC
    • PLANNING THE STUDY
    • INTRACARDIAC TECHNIQUE
    • TRANSESOPHAGEAL TECHNIQUE
    • OBJECTIVES OF DIAGNOSTIC ELECTROPHYSIOLOGIC STUDIES AND COMPARISON OF INTRACARDIAC AND TRANSESOPHAGEAL TECHNIQUES
    • ELECTROPHYSIOLOGIC STUDIES: THERAPEUTIC CATHETERIZATION
    • THE DECISION TO ABLATE: SAFETY VERSUS EFFICACY
    • ENERGY SOURCES
    • PROCEDURE: GENERAL
    • PROCEDURE: SUBSTRATE SPECIFIC
    • COMPLICATIONS AND FOLLOW-UP
    • SPECIAL CONSIDERATIONS FOR PEDIATRICS
    • SUMMARY
    • REFERENCES

    22: Disorders of Cardiac Rhythm and Conduction

    • SINUS NODE
    • ANATOMY OF THE ATRIOVENTRICULAR NODE
    • TACHYCARDIA
    • NARROW COMPLEX ARRHYTHMIAS
    • TREATMENT
    • BRADYCARDIA
    • PACEMAKERS AND IMPLANTABLE CARDIOVERTER DEFIBRILLATORS
    • SUMMARY
    • REFERENCES

SECTION V Pediatric Cardiac Intensive Care

    23: Physiology of the Preterm and Term Infant

    • INTRODUCTION
    • TRANSITIONAL CIRCULATION
    • PATENT DUCTUS ARTERIOSUS
    • LUNG DEVELOPMENT
    • PULMONARY HYPERTENSION
    • RESPIRATORY PHYSIOLOGY
    • CARDIOPULMONARY INTERACTION
    • HYPERTROPHIC CARDIOMYOPATHY IN INFANTS OF DIABETIC MOTHERS
    • RESPIRATORY SYNCYTIAL VIRUS
    • NEUROLOGY
    • GASTROINTESTINAL SYSTEM
    • RENAL
    • CARDIAC INTERVENTION IN THE PREMATURE OR LOW BIRTH WEIGHT NEONATE
    • INTERDISCIPLINARY APPROACH
    • REFERENCES

    24: Cardiovascular Intensive Care

    • INTRODUCTION
    • ORGANIZATION OF CARDIAC INTENSIVE CARE SERVICES
    • EVALUATION AND MANAGEMENT OF THE CARDIOVASCULAR SYSTEM
    • ARRHYTHMIAS
    • PULMONARY HYPERTENSION
    • THE PULMONARY SYSTEM, VENTILATION, AND AIRWAY MANAGEMENT
    • GENERAL POSTOPERATIVE CARE
    • PERIOPERATIVE CONSIDERATIONS FOR SPECIFIC LESIONS AND PROCEDURES
    • CENTRAL NERVOUS SYSTEM ISSUES
    • THE RENAL SYSTEM
    • FLUIDS, ELECTROLYTES, AND NUTRITION
    • INFECTION IN THE CVICU
    • ENDOCRINE ISSUES
    • CPR AND ECPR IN THE CVICU
    • THE CATHETERIZATION LABORATORY AND THE CVICU
    • MEDICAL CONDITIONS IN THE CVICU
    • THE LOW BIRTH WEIGHT INFANT
    • THE ADULT WITH CONGENITAL HEART DISEASE
    • CONCLUSIONS
    • REFERENCES

    25: Mechanical Circulatory Support in Children

    • INTRODUCTION
    • INDICATIONS FOR MECHANICAL CIRCULATORY SUPPORT
    • CONTRAINDICATIONS TO MECHANICAL CIRCULATORY SUPPORT
    • CANDIDATE SELECTION
    • SHORT-TERM MCS EITHER AS BRIDGE TO RECOVERY OR BRIDGE TO TRANSPLANTATION
    • LONG-TERM MCS SUPPORT IN CHILDREN
    • SPECIAL CONSIDERATIONS
    • REFERENCES

    26: Cardiopulmonary and Right–Left Heart Interactions

    • COMPARISON OF RIGHT AND LEFT HEART HEMODYNAMICS
    • CARDIOPULMONARY INTERACTIONS IN THE NORMAL CIRCULATION
    • CARDIOPULMONARY INTERACTIONS AND THE ABNORMAL RIGHT HEART
    • CARDIOPULMONARY INTERACTIONS AND THE LEFT HEART
    • RIGHT–LEFT HEART INTERACTIONS
    • CONCLUSIONS
    • REFERENCES

    27: Cardiac Trauma

    • BLUNT CARDIAC INJURY
    • PENETRATING CARDIAC INJURY
    • ELECTRICAL INJURY
    • REFERENCES

SECTION VI Congenital Cardiovascular Malformations

    PART A Septal Defects

      28: Atrial Septal Defects

      • INTRODUCTION
      • INCIDENCE
      • GENETIC AND ENVIRONMENTAL RISK FACTORS
      • PATHOGENESIS AND ANATOMIC FEATURES
      • ASSOCIATED CARDIOVASCULAR ANOMALIES
      • PATHOPHYSIOLOGY
      • CLINICAL PRESENTATION
      • DIAGNOSTIC EVALUATION
      • NATURAL HISTORY OF ASDS
      • MANAGEMENT
      • REFERENCES

      29: Atrioventricular Septal Defects

      • INTRODUCTION AND NOMENCLATURE
      • DEMOGRAPHICS
      • EMBRYOGENESIS
      • PARTIAL ATRIOVENTRICULAR SEPTAL DEFECT
      • COMPLETE ATRIOVENTRICULAR SEPTAL DEFECT
      • INITIAL SURGICAL TREATMENT OF AVSD
      • REOPERATION AFTER REPAIR OF AVSD
      • POSTOPERATIVE ECHOCARDIOGRAPHIC ASSESSMENT
      • THE ADULT WITH ATRIOVENTRICULAR SEPTAL DEFECT
      • CONCLUSIONS
      • ACKNOWLEDGMENTS
      • REFERENCES

      30: Ventricular Septal Defects

      • EPIDEMIOLOGY
      • ETIOLOGY
      • MORPHOLOGY
      • ASSOCIATED LESIONS
      • PHYSIOLOGY
      • CLINICAL FEATURES AND INVESTIGATIONS
      • NATURAL HISTORY
      • DIFFERENTIAL DIAGNOSIS
      • MEDICAL MANAGEMENT
      • INTERVENTIONAL MANAGEMENT
      • OUTCOMES
      • YOUNG ADULTS
      • REFERENCES

    PART B Arterial Abnormalities

      31: Patent Ductus Arteriosus and Aortopulmonary Window

      • PATENT DUCTUS ARTERIOSUS
      • AORTOPULMONARY WINDOW
      • REFERENCES

      32: Congenital Anomalies of the Coronary Vessels and the Aortic Root

      • CORONARY VASCULAR ANOMALIES
      • ANOMALIES OF CORONARY ARTERIES IN THE ABSENCE OF STRUCTURAL HEART DISEASE
      • CORONARY ARTERY FISTULAE
      • RARE CORONARY ANOMALIES
      • MISCELLANEOUS ANOMALIES: MYOCARDIAL BRIDGES
      • CORONARY ARTERY PATTERNS WITH CONGENITAL HEART DEFECTS
      • CONGENITAL ANOMALIES OF THE AORTIC ROOT
      • REFERENCES

      33: Aortic Arch and Vascular Anomalies

      • INTRODUCTION
      • ANOMALIES OF THE AORTIC ARCH
      • MALFORMATIONS OF THE SYSTEMIC VASCULATURE
      • PULMONARY ARTERIOVENOUS MALFORMATIONS
      • ACKNOWLEDGMENTS
      • ONLINE 3-D MODELS
      • REFERENCES

      34: Abnormalities of the Aortic Root

      • INTRODUCTION
      • MARFAN SYNDROME
      • BICUSPID AORTIC VALVE
      • TETRALOGY OF FALLOT
      • COARCTATION OF THE AORTA
      • TRANSPOSITION OF THE GREAT ARTERIES
      • OTHER LESIONS
      • CONCLUSIONS
      • REFERENCES

    PART C Venous Abnormalities

      35: Anomalies of the Pulmonary Veins

      • ANATOMY
      • EMBRYOLOGY
      • PARTIALLY ANOMALOUS PULMONARY VENOUS CONNECTIONS
      • TOTALLY ANOMALOUS PULMONARY VENOUS CONNECTION OR DRAINAGE
      • ATRESIA OF THE COMMON PULMONARY VEIN AFTER THE PULMONARY SYSTEMIC CONNECTIONS ARE OBLITERATED
      • STENOSIS OF THE COMMON PULMONARY VEIN
      • ABNORMAL ABSORPTION OF THE COMMON PULMONARY VEIN INTO LEFT ATRIUM
      • REFERENCES

      36: Abnormal Systemic Venous Connections

      • EMBRYOLOGY
      • ANOMALIES OF THE SUPERIOR VENAE CAVAE
      • ANOMALIES OF THE CORONARY SINUS
      • ANOMALIES OF THE INFERIOR VENA CAVA
      • ANOMALIES OF THE DUCTUS VENOSUS
      • PERSISTENT VALVES OF THE SINUS VENOSUS
      • REFERENCES

      37: Vascular Tumors and Anomalies

      • INTRODUCTION
      • EMBRYOLOGY AND GENETICS
      • CLASSIFICATION
      • CLINICAL MANIFESTATIONS AND TREATMENT
      • PHARMACOTHERAPY OF LYMPHATIC AND MIXED VASCULAR MALFORMATIONS
      • REFERENCES

    PART D The Right Ventricle

      38: Tricuspid Valve Disorders: Atresia, Dysplasia, and Ebstein Anomaly

      • TRICUSPID VALVE ATRESIA
      • EBSTEIN ANOMALY
      • TRICUSPID VALVE REGURGITATION: CONGENTIAL AND ACQUIRED
      • UHL ANOMALY
      • ACKNOWLEDGMENTS
      • REFERENCES

      39: Pulmonary Stenosis

      • ISOLATED VALVAR PULMONARY STENOSIS
      • PULMONARY STENOSIS ASSOCIATED WITH SYSTEMIC DISEASES
      • PULMONARY VALVE STENOSIS IN YOUNG ADULTS
      • SUBVALVULAR RIGHT VENTRICULAR OUTFLOW TRACT OBSTRUCTION
      • RIGHT VENTRICULAR OBSTRUCTION
      • DOUBLE-CHAMBERED RIGHT VENTRICLE IN ADULTS
      • PERIPHERAL PULMONARY ARTERY STENOSIS WITH INTACT VENTRICULAR SEPTUM
      • PULMONARY ARTERY STENOSIS IN YOUNG ADULTS
      • REFERENCES

      40: Pulmonary Atresia and Intact Ventricular Septum

      • ROBERT MARK FREEDOM 1941–2005
      • EPIDEMIOLOGY
      • DEFINITION
      • MORPHOGENESIS
      • PATHOLOGY AND PHYSIOLOGY
      • CLINICAL FEATURES
      • TREATMENT
      • TREATMENT OF THE ADULT
      • SUMMARY
      • REFERENCES

      41: Tetralogy of Fallot with Pulmonary Stenosis, Pulmonary Atresia, and Absent Pulmonary Valve

      • EPIDEMIOLOGY
      • ETIOLOGY (GENETIC AND ENVIRONMENTAL FACTORS)
      • ANATOMY (MORPHOLOGIC, ECHOCARDIOGRAPHIC, AND ANGIOGRAPHIC CORRELATIONS)
      • CLINICAL FEATURES AND INVESTIGATION
      • MEDICAL TREATMENT
      • PERCUTANEOUS PALLIATION
      • THE EVOLUTION OF SURGICAL TREATMENT
      • POSTOPERATIVE PATHOPHYSIOLOGY
      • OTHER ISSUES FOR ADULT LIFE
      • REFERENCES

      42: Truncus Arteriosus

      • EMBRYOLOGY
      • PATHOLOGY
      • MANIFESTATIONS
      • DIFFERENTIAL DIAGNOSIS
      • NATURAL HISTORY
      • TREATMENT
      • SURGICAL CORRECTION
      • CARE OF THE ADULT PATIENT WITH REPAIRED TRUNCUS ARTERIOSUS
      • LONG-TERM ISSUES
      • REFERENCES

    PART E Left Ventricular Inflow and Outflow Abnormalities

      43: Anatomical and Functional Mitral Valve Abnormalities in the Pediatric Population

      • INTRODUCTION
      • MITRAL VALVE DEVELOPMENT
      • INCIDENCE OF MITRAL VALVE ABNORMALITES
      • MITRAL VALVE MORPHOLOGY AND FUNCTION
      • MITRAL VALVE PATHOLOGY: AN INTEGRATED MORPHOLOGIC AND HEMODYNAMIC APPROACH
      • HEMODYNAMIC ASSESSMENT OF THE MITRAL VALVE
      • ASSESSMENT OF MITRAL VALVE REGURGITATION
      • QUANTITATIVE ASSESSMENT OF MITRAL REGURGITATION BY DOPPLER
      • MITRAL VALVE PROLAPSE
      • CLINICAL PRESENTATION
      • ELECTROCARDIOGRAPHY
      • RADIOGRAPHY
      • MAGNETIC RESONANCE IMAGING
      • HEMODYNAMIC EVALUATION
      • MANAGEMENT AND PROGNOSIS OF CONGENITAL MITRAL VALVE STENOSIS
      • MANAGEMENT AND PROGNOSIS OF CONGENITAL MITRAL REGURGITATION
      • MANAGEMENT AND PROGNOSIS OF MITRAL VALVE PROLAPSE
      • REFERENCES

      44: Aortic Stenosis

      • INTRODUCTION
      • EPIDEMIOLOGY
      • EMBRYOLOGY AND PATHOLOGY
      • MOLECULAR GENETICS
      • PHYSIOLOGY
      • CLINICAL FEATURES AND DIAGNOSTIC METHODS
      • NATURAL HISTORY
      • MEDICAL MANAGEMENT
      • THERAPEUTIC CARDIAC CATHETERIZATION
      • SURGICAL MANAGEMENT
      • REFERENCES

      45: Coarctation of the Aorta

      • PREVALENCE AND ETIOLOGY
      • EMBRYOLOGY
      • MORPHOLOGY
      • HEMODYNAMICS
      • CLINICAL FEATURES
      • NATURAL HISTORY AND MANAGEMENT
      • OUTCOMES
      • YOUNG ADULT ISSUES
      • REFERENCES

      46: Hypoplastic Left Heart Syndrome

      • EPIDEMIOLOGY AND ETIOLOGY
      • THE DEVELOPING FETUS: ECHOCARDIOGRAPHY AND INTERVENTION
      • ANATOMY
      • PRESENTATION, DIAGNOSIS, AND ECHOCARDIOGRAPHIC IMAGING
      • PHYSIOLOGY, MONITORING, AND STABILIZATION
      • STAGED PALLIATION
      • OUTCOMES FOR STAGED PALLIATION
      • SUMMARY
      • ACKNOWLEDGMENT
      • REFERENCES

    PART F Abnormalities of the Origin of the Great Arteries

      47: Transposition of the Great Arteries

      • INTRODUCTION
      • EPIDEMIOLOGY (INCIDENCE, PREVALENCE)
      • ETIOLOGY
      • MORPHOLOGY
      • PHYSIOLOGY OF TRANSPOSITION OF THE GREAT ARTERIES
      • CLINICAL FEATURES AND INVESTIGATION
      • NATURAL HISTORY AND MANAGEMENT
      • OPERATIVE AND INTERVENTIONAL CATHETERIZATION APPROACHES
      • LONG-TERM OUTCOMES AND YOUNG ADULT ISSUES
      • REFERENCES

      48: Congenitally Corrected Transposition of the Great Arteries (Atrioventricular and Ventriculoarterial Discordance)

      • PREVALENCE, ETIOLOGY, AND MORPHOGENESIS
      • MORPHOLOGY
      • ASSOCIATED LESIONS
      • VENTRICULAR SEPTAL DEFECT
      • PULMONARY OUTFLOW OBSTRUCTION
      • LESIONS OF THE MORPHOLOGIC TRICUSPID VALVE
      • CORONARY ARTERY ANATOMY
      • SPECIALIZED CONDUCTION TISSUES
      • CLINICAL FEATURES AND PHYSICAL EXAMINATION
      • ELECTROCARDIOGRAPHIC FEATURES
      • RADIOGRAPHIC FEATURES
      • ECHOCARDIOGRAPHY
      • MAGNETIC RESONANCE IMAGING
      • CARDIAC CATHETERIZATION
      • ANGIOCARDIOGRAPHY
      • NATURAL HISTORY AND MANAGEMENT
      • MEDICAL MANAGEMENT
      • PACING
      • UNOPERATED NATURAL HISTORY
      • PHYSIOLOGIC SURGICAL REPAIR AND OUTCOMES
      • RIGHT VENTRICULAR DYSFUNCTION AND TRICUSPID REGURGITATION
      • ANATOMIC SURGICAL REPAIR AND OUTCOMES
      • QUALITY OF LIFE
      • PREGNANCY
      • CONCLUSION
      • ACKNOWLEDGMENTS
      • REFERENCES

      49: Double-Outlet Right Ventricle

      • DEFINITION
      • INCIDENCE
      • MORPHOLOGY
      • CLINICAL FEATURES: COMMON VARIANTS
      • HISTORY OF SURGICAL REPAIR OF DOUBLE-OUTLET RIGHT VENTRICLE
      • ESSENTIAL DETERMINANTS OF SURGICAL CORRECTION AND TIMING OF THE OPERATION
      • TYPES OF SURGERY AND SURGICAL STRATEGY
      • SURGICAL OUTCOMES
      • REINTERVENTION AND REOPERATION
      • MANAGEMENT OF THE ADULT WITH DOUBLE-OUTLET RIGHT VENTRICLE
      • ACKNOWLEDGMENT
      • REFERENCES

    PART G Complex Cardiac Abnormalities

      50: Univentricular Atrioventricular Connection

      • NOMENCLATURE
      • PATHOLOGIC ANATOMY
      • COMMON FORMS OF UNIVENTRICULAR ATRIOVENTRICULAR CONNECTION
      • REFERENCES

      51: Cardiac Malpositions and Abnormalities of Atrial and Visceral Situs

      • DEFINITIONS
      • SEQUENTIAL SEGMENTAL APPROACH TO CARDIAC DIAGNOSIS
      • VISCERAL AND ATRIAL SITUS
      • ATRIOVENTRICULAR CONNECTIONS AND VALVE MORPHOLOGY
      • VENTRICULAR POSITIONS AND MORPHOLOGY
      • GREAT ARTERY RELATIONS
      • CARDIAC IMAGING: USE OF THE SEGMENTAL APPROACH
      • DEXTROCARDIA
      • LEVOCARDIA (ISOLATED LEVOCARDIA)
      • MESOCARDIA
      • SITUS AMBIGUUS
      • ASPLENIA
      • POLYSPLENIA
      • TREATMENT STRATEGIES
      • CONGENITAL PERICARDIAL DEFECTS
      • ECTOPIA CORDIS
      • ACKNOWLEDGMENTS
      • REFERENCES

SECTION VII Diseases of the Endocardium, Myocardium, and Pericardium

    52: Hypertrophic Cardiomyopathy

    • DEFINITION AND NOMENCLATURE
    • PREVALENCE
    • MORPHOLOGY
    • PATHOPHYSIOLOGY
    • GENETICS
    • CLINICAL SCREENING STRATEGIES IN FAMILIES
    • CLINICAL FEATURES
    • HCM AND ATHLETE’S HEART
    • PREPARTICIPATION SCREENING FOR HCM IN ATHLETIC POPULATIONS
    • CLINICAL COURSE
    • RISK STRATIFICATION AND SUDDEN CARDIAC DEATH
    • MANAGEMENT
    • REFERENCES

    53: Dilated Cardiomyopathy

    • INTRODUCTION
    • ETIOLOGIES
    • PATHOPHYSIOLOGY
    • CLINICAL EVALUATION
    • TREATMENT
    • ACKNOWLEDGMENT
    • REFERENCES

    54: Left Ventricular Noncompaction Cardiomyopathy

    • INTRODUCTION
    • DEFINITION
    • HISTORY
    • NORMAL VENTRICULAR MYOCARDIAL DEVELOPMENT
    • VENTRICULAR HYPERTRABECULATION
    • GENETIC CAUSES OF LVNC
    • CHROMOSOMAL CONDITIONS ASSOCIATED WITH LVNC
    • CLINICAL PRESENTATION
    • UNDULATING PHENOTYPE
    • PRENATAL DIAGNOSIS
    • LVNC WITH NORMAL EJECTION FRACTION
    • NONCARDIAC MANIFESTATIONS OF LVNC
    • ELECTROCARDIOGRAM FINDINGS
    • ECHOCARDIOGRAPHIC ASSESSMENT OF LVNC
    • COMPUTED TOMOGRAPHY
    • CARDIAC MAGNETIC RESONANCE IMAGING
    • MEDICAL MANAGEMENT
    • LVNC AND CONGENITAL HEART DISEASE
    • PREGNANCY AND LVNC
    • EMBOLIC EVENTS IN LVNC
    • DIFFERENTIATION OF LVNC FROM DCM
    • ATHLETIC HYPERTRABECULATION
    • PROGNOSIS
    • CONTENTIOUS ISSUES
    • REFERENCES

    55: Myocarditis

    • INTRODUCTION
    • EPIDEMIOLOGY
    • ETIOLOGY
    • CLINICAL FEATURES AND INVESTIGATION
    • ENDOMYOCARDIAL BIOPSY
    • TREATMENT
    • CONCLUSION
    • REFERENCES

    56: Restrictive Cardiomyopathy

    • EPIDEMIOLOGY
    • GENETICS
    • ETIOLOGIES
    • PATHOLOGY
    • PATHOPHYSIOLOGY
    • CLINICAL PRESENTATION
    • PHYSICAL EXAMINATION
    • DIAGNOSTIC STUDIES
    • DIFFERENTIAL DIAGNOSIS
    • OUTCOME
    • MANAGEMENT
    • REFERENCES

    57: The Heart in Muscular Dystrophies

    • MUSCULAR DYSTROPHIES WITH CLINICAL CARDIAC DISEASE
    • ASSESSMENT TOOLS
    • MUSCULAR DYSTROPHIES WITH CARDIAC INVOLVEMENT
    • MYOTONIC MUSCULAR DYSTROPHY
    • EMERY–DREIFUSS MUSCULAR DYSTROPHY
    • FACIOSCAPULOHUMERAL MUSCULAR DYSTROPHY
    • LIMB-GIRDLE MUSCULAR DYSTROPHIES
    • FRIEDREICH’S ATAXIA
    • REFERENCES

    58: Kawasaki Disease (Mucocutaneous Lymph Node Syndrome)

    • EPIDEMIOLOGIC FEATURES
    • ETIOLOGY AND PATHOGENESIS
    • PATHOLOGY
    • CLINICAL FEATURES: SYSTEMIC
    • RECURRENCE AND RECRUDESCENCE
    • DIAGNOSIS AND DIFFERENTIAL DIAGNOSIS
    • ALGORITHM FOR CLINICAL EVALUATION AND TREATMENT OF CHILDREN WITH SUSPECTED KAWASAKI DISEASE
    • TREATMENT: ACUTE PHASE
    • TREATMENT: CHRONIC PHASE
    • SUMMARY
    • REFERENCES

    59: Rheumatic Fever and Rheumatic Heart Disease

    • EPIDEMIOLOGY
    • PATHOGENESIS
    • PATHOLOGY
    • CLINICAL MANIFESTATIONS
    • CHRONIC RHEUMATIC HEART DISEASE
    • TREATMENT
    • SURGICAL AND CATHETER-BASED TREATMENT
    • PREVENTION AND PROPHYLAXIS
    • SUMMARY
    • REFERENCES

    60: Inflammatory Noninfectious Cardiovascular Diseases

    • INTRODUCTION
    • JUVENILE IDIOPATHIC ARTHRITIS
    • SYSTEMIC LUPUS ERYTHEMATOSUS
    • TAKAYASU ARTERITIS
    • JUVENILE IDIOPATHIC INFLAMMATORY MYOPATHIES
    • BEHÇET DISEASE
    • SCLERODERMA
    • SARCOID
    • POLYARTERITIS NODOSA
    • REFERENCES

    61: Pericardial Diseases

    • ANATOMY AND PHYSIOLOGY
    • ACUTE PERICARDITIS
    • ETIOLOGY
    • CONSTRICTIVE PERICARDITIS
    • REFERENCES

    62: Infective Endocarditis and Prevention

    • DEFINITION
    • EPIDEMIOLOGY
    • MICROORGANISMS
    • PATHOGENESIS
    • CLINICAL FEATURES
    • LABORATORY DIAGNOSIS
    • ECHOCARDIOGRAPHY
    • DIAGNOSTIC CRITERIA
    • ANTIMICROBIAL THERAPY
    • PREVENTION
    • REFERENCES

    63: Myocardial Ischemia in the Pediatric Population

    • INTRODUCTION
    • DEFINITION
    • HISTORY
    • DIFFERENTIAL DIAGNOSIS
    • CONGENITAL ANOMALIES OF THE CORONARY ARTERIES
    • ANOMALOUS ORIGIN OF LEFT CORONARY ARTERY FROM THE PULMONARY ARTERY (ALCAPA)
    • TETRALOGY OF FALLOT
    • OTHER CARDIAC DEFECTS
    • EXAMINATION
    • ELECTROCARDIOGRAM
    • BIOMARKERS OF MYOCARDIAL INJURY
    • CARDIAC IMAGING AND STRESS TESTING
    • TREATMENT
    • SUMMARY
    • REFERENCES

    64: Pediatric Heart Transplantation

    • PRETRANSPLANT EVALUATION
    • PRETRANSPLANT MANAGEMENT
    • DONOR ISSUES
    • ABO-INCOMPATIBLE HEART TRANSPLANTATION
    • POSTOPERATIVE MANAGEMENT
    • REFERENCES

SECTION VIII Pulmonary Vascular Disease

    65: Pathophysiology of Pulmonary Hypertension

    • INTRODUCTION
    • HEART DEFECTS WITH INCREASED PULMONARY BLOOD FLOW
    • HEART DEFECTS WITH INCREASED PULMONARY VENOUS PRESSURE
    • HEART DEFECTS WITH DECREASED PULMONARY BLOOD FLOW
    • HYPOXIA-INDUCED PULMONARY VASCULAR DISEASE
    • CHRONIC HYPOXIA
    • LUNG DISEASE
    • UPPER AIRWAY OBSTRUCTION
    • DISORDERS OF THE CHEST WALL
    • PERSISTENT PULMONARY HYPERTENSION OF THE NEWBORN
    • THROMBOEMBOLIC DISEASES
    • SICKLE CELL DISEASE AND OTHER HEMAGLOBINOPATHIES
    • PORTAL HYPERTENSION
    • GRANULOMATOUS AND COLLAGEN VASCULAR DISEASES AND IMMUNE/INFECTIOUS DISEASES
    • DRUGS AND TOXINS
    • IDIOPATHIC AND HERITABLE PULMONARY HYPERTENSION
    • SUMMARY
    • FUTURE DIRECTIONS
    • REFERENCES

    66: Pediatric Pulmonary Hypertension

    • DEFINITION AND CLASSIFICATION
    • ETIOLOGY
    • EPIDEMIOLOGY AND SURVIVAL
    • CLINICAL PRESENTATION
    • SIGNS AND SYMPTOMS
    • EVALUATION
    • CHEST RADIOGRAPHY
    • ELECTROCARDIOGRAPHY
    • ECHOCARDIOGRAPHY
    • CARDIAC MRI
    • EXERCISE CAPACITY
    • CARDIAC CATHETERIZATION AND BIOMECHANICAL EVALUATION
    • LABORATORY ASSESSMENT
    • VENTILATION–PERFUSION SCINTIGRAPHY
    • PULMONARY FUNCTION TESTING/NOCTURNAL OXIMETRY
    • EVALUATION OF REFLUX AND ASPIRATION
    • BIOMARKERS AND TREATMENT GOALS
    • LUNG BIOPSY AND WEDGE ANGIOGRAPHY
    • IMPORTANT CAUSES OF PH IN CHILDREN
    • PHARMACOLOGIC THERAPY OF PAH
    • COMBINATION THERAPY
    • ATRIAL SEPTOSTOMY
    • POTTS SHUNT
    • TRANSPLANTATION
    • NOVEL THERAPIES
    • REFERENCES

SECTION IX Unique Issues Regarding the Young Adult with Congenital Heart Disease

    67: The Adolescent and Adult with Congenital Heart Disease

    • PREVALENCE OF ADULT CONGENITAL HEART DISEASE (ACHD)
    • AN ADULT CONGENITAL HEART DISEASE PROGRAM
    • TRAINING IN ADULT CONGENITAL HEART DISEASE
    • BICUSPID AORTIC VALVE
    • AORTIC VALVE REGURGITATION
    • SUBAORTIC STENOSIS
    • COARCTATION OF THE AORTA
    • SYSTEMIC HYPERTENSION
    • RECOARCTATION
    • AORTIC ANEURYSM/PSEUDOANEURYSM
    • LEFT-TO-RIGHT SHUNTING LESIONS
    • VENTRICULAR SEPTAL DEFECTS
    • ATRIOVENTRICULAR SEPTAL DEFECTS
    • ATRIAL SEPTAL DEFECTS
    • PULMONARY HYPERTENSION IN ADULTS WITH CONGENITAL HEART DISEASE
    • EISENMENGER SYNDROME
    • PATENT DUCTUS ARTERIOSUS
    • MITRAL VALVE PROLAPSE
    • PULMONARY VALVE STENOSIS
    • CYANOTIC CONGENITAL DEFECTS
    • TETRALOGY OF FALLOT
    • TRANSPOSITION OF THE GREAT ARTERIES
    • ARRHYTHMIAS
    • BAFFLE OBSTRUCTION AND LEAKS
    • BAFFLE LEAKS
    • TRICUSPID VALVE REGURGITATION
    • SYSTEMIC (RV) VENTRICULAR DYSFUNCTION
    • LATE-STAGE VENTRICULAR FAILURE
    • ARTERIAL SWITCH FOR TGA
    • EBSTEIN ANOMALY OF THE TRICUSPID VALVE
    • CONGENITALLY CORRECTED TRANSPOSITION OF THE GREAT ARTERIES
    • SINGLE VENTRICLE
    • THE FONTAN PATIENT
    • CYANOSIS
    • ARRHYTHMIAS
    • THROMBOEMBOLIC COMPLICATIONS
    • PROTEIN LOSING ENTEROPATHY
    • PLASTIC BRONCHITIS
    • HEPATIC COMPLICATIONS POST FONTAN SURGERY
    • FONTAN REVISION
    • THE CYANOTIC ACHD PATIENT
    • REFERENCES

    68: Transition to Adult Care

    • INTRODUCTION
    • WHAT IS TRANSITION AND TRANSFER?
    • WHY TRANSITION?
    • TIMING OF TRANSITION
    • COMPONENTS OF TRANSITION
    • TRANSITION TIMELINE
    • PERSONNEL REQUIRED FOR TRANSITION
    • BARRIERS TO TRANSITION
    • SUMMARY
    • REFERENCES

    69: Pregnancy in Young Women with Congenital Heart Disease

    • PHYSIOLOGIC CHANGES DURING PREGNANCY
    • CARDIAC FINDINGS IN NORMAL PREGNANCY
    • ASSESSMENT OF PREGNANCY RISK IN WOMEN WITH CONGENITAL HEART DISEASE: GENERAL CONCEPTS AND GLOBAL EVALUATION
    • LESION-SPECIFIC RISKS AND OUTCOMES
    • TRANSMISSION OF CARDIAC DISEASE TO OFFSPRING
    • MANAGEMENT ISSUES DURING PREGNANCY
    • MANAGEMENT ISSUES DURING LABOR AND DELIVERY
    • CONTRACEPTION
    • REFERENCES

SECTION X Other Special Problems and Issues

    70: Chest Pain in Children and Adolescents

    • MUSCULOSKELETAL/CHEST-WALL PAIN
    • OTHER NONCARDIAC CAUSES OF CHEST PAIN
    • CARDIAC CAUSES OF CHEST PAIN
    • MEDICAL EVALUATION
    • TREATMENT
    • OUTCOME
    • REFERENCES

    71: Coronary Risk Factors in Children

    • ATHEROSCLEROSIS
    • LIPIDS AND LIPOPROTEINS
    • HYPERTENSION
    • REFERENCES

    72: Cardiac Tumors

    • HISTORY OF CARDIAC TUMORS
    • INCIDENCE
    • DIAGNOSTIC PROCEDURES
    • RHABDOMYOMAS
    • FIBROMAS
    • MYXOMAS
    • INTRAPERICARDIAL TERATOMAS
    • OTHER PRIMARY BENIGN MYOCARDIAL TUMORS
    • PRIMARY MALIGNANT MYOCARDIAL TUMORS
    • SECONDARY CARDIAC TUMORS
    • REFERENCES

    73: Chronic Heart Failure in Children

    • INTRODUCTION
    • NOMENCLATURE AND CLASSIFICATION
    • ETIOLOGY
    • NEONATES AND INFANTS
    • CHILDHOOD AND ADOLESCENCE
    • BIOMARKERS IN CHRONIC HEART FAILURE
    • MANAGEMENT OF CHRONIC HEART FAILURE
    • OUTCOMES IN PEDIATRIC HEART FAILURE
    • ACKNOWLEDGMENT
    • REFERENCES

    74: Neurodevelopmental Outcomes After Heart Surgery in Children

    • GENETIC ABNORMALITIES
    • BRAIN ANATOMY, PATHOLOGY, AND NEUROIMAGING
    • PERIOPERATIVE RISK FACTORS
    • OTHER CAUSES OF NEUROLOGIC MORBIDITY
    • LONG-TERM NEURODEVELOPMENTAL OUTCOME
    • CONCLUSION
    • REFERENCES

    75: Hematologic Aspects of Pediatric and Adolescent Heart Disease: Bleeding, Clotting, and Blood Component Abnormalities

    • BASIC PRINCIPLES OF HEMATOLOGY
    • HEMATOLOGIC DISORDERS
    • CPB, ECMO, AND VENTRICULAR ASSIST DEVICES
    • THROMBOSIS
    • REFERENCES

    76: Impact of Cardiac Disease on Other Organ Systems

    • HOW AND WHY ARE OTHER ORGAN SYSTEMS AFFECTED IN CHD?
    • IMPACT OF CHD ON THE RENAL SYSTEM
    • IMPACT OF CHD ON THE GASTROINTESTINAL SYSTEM
    • THE SPECIAL CASE OF THE FONTAN CIRCULATION
    • SUMMARY
    • REFERENCES

    77: Evaluation of Quality of Life in Children and Adolescents with Congenital and Acquired Heart Disease

    • INTRODUCTION
    • HEALTH MEASUREMENT DEFINITIONS: QOL AND HRQOL
    • INHERENT DIFFICULTIES OF HRQOL EVALUATION IN THE PEDIATRIC HD POPULATION
    • HRQOL INSTRUMENT EVALUATION
    • EXISTING GENERIC AND DISEASE-SPECIFIC HRQOL MEASURES THAT MAY BE USED TO ASSESS HRQOL IN THE PEDIATRIC HD POPULATION
    • RESEARCH ON HRQOL IN PEDIATRIC PATIENTS WITH HD: WHAT IS KNOWN?
    • SUMMARY
    • CLINICAL IMPLEMENTATION (IS HRQOL EVALUATION UTILIZED IN CLINICAL PRACTICE?)
    • RESEARCH AGENDA (WHAT IS NEEDED IN HRQOL RESEARCH IN PEDIATRIC HD PATIENTS?)
    • CLINICAL AGENDA (WHAT IS NEEDED TO HARNESS THE POTENTIAL OF HRQOL ASSESSMENT FOR CLINICAL USE IN PEDIATRIC HD PATIENTS?)
    • CONCLUSION
    • REFERENCES

    78: Beyond the Cardiac Device: Comprehensive Care of Children and Young Adults with Implantable Cardiac Devices

    • INTRODUCTION
    • MODERN USE AND MEDICAL INDICATIONS FOR DEVICE-BASED CARE
    • INDICATIONS AND UTILIZATION
    • REVIEW OF PSYCHOSOCIAL RESEARCH BY CARDIAC DEVICE: PMs, ICDs, AND VADs
    • SUCCESSFUL CLINICAL MANAGEMENT OF PSYCHOSOCIAL ISSUES IN PEDIATRIC DEVICE PATIENTS
    • CONCLUSION
    • REFERENCES

    79: Safety and Quality in the Heart Center

    • INTRODUCTION
    • I. CASE STUDY
    • II. ROOT CAUSE ANALYSIS—A ROAD TO RESOLUTION: WHAT HAPPENED AND WHY
    • III. THE IMPROVEMENT TEAM—AIM, KEY DRIVERS, AND INTERVENTIONS DEVELOPED BY THE TEAM
    • IV. DATA DRIVES CHANGE—DON’T LET DATA PARALYZE THE TEAM
    • V. HIGH RELIABILITY—DO WHAT YOU SAY AND SAY WHAT YOU MEAN
    • VI. PATIENT- AND FAMILY-CENTERED CARE
    • VII. CULTURE—AN ESSENTIAL INGREDIENT
    • REFERENCES

    80: The Global Challenge of Heart Disease

    • INTRODUCTION
    • GLOBAL BURDEN OF CARDIOVASCULAR DISEASE
    • THE CONCEPT OF THE EPIDEMIOLOGIC TRANSITION
    • THE IMPENDING GLOBAL EPIDEMIC OF ISCHEMIC CARDIOVASCULAR DISEASE
    • RHEUMATIC HEART DISEASE
    • CONGENITAL HEART DISEASE
    • CHAGAS DISEASE
    • SUMMARY
    • REFERENCES

    81: Design, Execution, and Appraisal of Clinical Trials

    • WHAT IS EVIDENCE-BASED MEDICINE AND WHY ARE CLINICAL TRIALS SO RELEVANT TO THAT PRACTICE?
    • HOW DO CLINICAL TRIALS ACHIEVE A HIGH GRADE OF EVIDENCE?
    • WHY ARE THERE NOT MORE CLINICAL TRIALS?
    • DESIGN ISSUES FOR CLINICAL TRIALS
    • ANALYSIS ISSUES FOR CLINICAL TRIALS
    • ACKNOWLEDGMENT
    • RECOMMENDED RESOURCES
    • REFERENCES

    82: Pharmacology

    • INTRODUCTION
    • COMMON DEFINITIONS OF TERMS IN CLINICAL PHARMACOLOGY
    • THE EXPOSURE–RESPONSE RELATIONSHIP
    • DEVELOPMENTAL PHARMACOKINETICS
    • DEVELOPMENTAL PHARMACODYNAMICS
    • DEVELOPMENTAL PHARMACOGENOMICS
    • CARDIOVASCULAR DRUGS
    • CARDIAC GLYCOSIDES
    • ADRENERGIC AGONISTS
    • PHOSPHODIESTERASE INHIBITORS
    • CALCIUM SENSITIZING AGENTS
    • DIURETICS
    • VASODILATORS
    • ALDOSTERONE RECEPTOR ANTAGONISTS
    • HORMONES
    • β-ADRENERGIC RECEPTOR BLOCKERS
    • PROSTAGLANDIN E 1
    • ANTIARRHYTHMIC DRUGS
    • CONCLUSIONS
    • REFERENCES