Moss & Adams' Heart Disease in Infants, Children, and Adolescents

Name: Moss & Adams' Heart Disease in Infants, Children, and Adolescents
Brand: Wolters Kluwer Health
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Robert E. Shaddy(Autor*in)

Wolters Kluwer Health (Verlag)

1. Auflage

Erschienen am 19. Februar 2021

1912 Seiten

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Cover
Title Page
Copyright
Dedication
Contributors
Preface to the Previous Edition
Preface
Previous Editors
Contents
Section I: From the Gene to the Neonate
1 Development of the Heart: Morphogenesis, Growth, and Molecular Regulation of Differentiation
From Cardiac Embryology to Understanding the Development of Congenital Heart Defects
From Cardiac Embryology to Regenerating the Failing Heart
Experimental Animal Models and Tools Used in Cardiac Embryology Research
FORMATION AND LOOPING OF THE HEART TUBE
Formation of the Embryo
Rising the Heart: The Primary Field of Cardiac Progenitors
Formation of the Primitive Heart Tube
Growth of the Primitive Heart Tube and Role of the Secondary Heart Field
CHAMBER FORMATION AND VENTRICULAR SEPTATION
Development of the Basic Building Plan of the Mammalian Heart
Growth and Differentiation of the Left and Right Ventricular Chambers
Ventricular Septation
DEVELOPMENT OF THE VENOUS POLE OF THE HEART
Morphogenesis and Differentiation of the Atria
Atrial Septation
Development of the Systemic and Pulmonary Veins
DEVELOPMENT OF THE ATRIOVENTRICULAR JUNCTION
Formation of the AV Endocardial Cushions
Development and Maturation of the AV Valves
Atrioventricular Septation
Formation of the AV Plane of Fibrous Insulation
DEVELOPMENT OF THE ARTERIAL POLE OF THE HEART
Cardiac Neural Crest
Formation and Septation of the Cardiac Outflow Tract
Formation of the Intrapericardial Arterial Trunks
Development and Maturation of the Arterial Valves
Transformation of the Pharyngeal Arch Arterial System
Development of the Subclavian and Pulmonary Arteries
DEVELOPMENT OF THE EPICARDIUM AND CORONARY ARTERIES
Formation of the Epicardium
Development of the Coronary Arteries
2 Epidemiology and Prevention of Congenital Heart Defects
FROM EPIDEMIOLOGY TO PREVENTION
Why Epidemiology Matters: The Cycle from Assessment to Prevention
OUTCOMES AS INCENTIVE FOR ACTION: COMMON, COSTLY, AND CRITICAL
How Common Are Congenital Heart Defects?
How Costly Are Congenital Heart Defects?
How Critical Are Heart Defects?
Outcomes: Gaps, Needs, Trends
EVIDENCE FOR ACTION: EPIDEMIOLOGY OF RISK AND CAUSES
What Does It Mean to Characterize Modifiable Causes?
Candidates for Prevention: Review of Selected Risk Factors
STRATEGIES: EPIDEMIOLOGY OF PREVENTION
Integrating Interventions
When to Start Preventing?
What Proportion of Heart Defects Can Be Prevented?
CONCLUDING COMMENTS
3 Genetic Aspects of Congenital Heart Defects
METHODS AND MECHANISMS
Genetic Testing
Investigative Approaches
PATTERNS OF INHERITANCE AND FAMILIAL RISKS
GENETIC SYNDROMES
Syndromes Associated with Chromosome Abnormalities
Change in Chromosome Number (Aneuploidy)
Deletion-Duplication Syndromes
Genetic Syndromes Caused by Variants in Single Genes
Other Complex Genetic Conditions Associated with CHD
Genetics of Isolated Congenital Heart Defects
Candidate Approach to Gene Discovery
Next-Generation Sequencing Approach to Gene Discovery
GENETIC TESTING FOR CHD
GENETIC EVALUATION AND COUNSELING
Importance of Identifying a Genetic Syndrome or Genomic Imbalance
When to Refer the Cardiac Patient for a Genetic Evaluation
The Genetic Evaluation
Genetic Testing
CONCLUSIONS
4 Development of Myocardial Structure and Function
MYOCARDIAL STRUCTURE
CARDIAC FIBROBLASTS AND THE ECM
FUNCTIONAL STRUCTURE OF CARDIAC MYOCYTES
Plasma Membrane
T-Tubules
Sarcomere
MITOCHONDRIA AND METABOLISM
CELL-ECM AND CELL-CELL INTERACTIONS
Costameres
Dystrophin-Associated Glycoprotein Complexes
CORONARY VASCULATURE
MYOCARDIAL GROWTH AND REMODELING
Cell Death and Myocardial Structure
MYOCYTE CONTRACTILE FUNCTION
Regulation of E-C Coupling
Intrinsic Regulation
Autonomic Innervation
Thyroid Hormone
LV CHAMBER FUNCTION
Systole
Diastole
Alterations in Pump Function at Birth
PRESSURE-VOLUME LOOP ASSESSMENT OF CARDIAC FUNCTION
EMERGING CONCEPTS AND CONCLUSION
5 Fetal and Perinatal Cardiology
IMPLICATIONS OF FETAL DIAGNOSIS OF CARDIOVASCULAR DISEASE
Parental Preparation
Morbidity and Mortality
FETAL CARDIOVASCULAR CIRCULATION
EARLY GESTATION FETAL CARDIAC EVALUATION AND SCREENING FOR FETAL CARDIOVASCULAR DISEASE
INDICATIONS FOR FETAL ECHOCARDIOGRAPHY
Fetal Indications
Maternal Indications
Family History of Congenital Heart Disease
THE FETAL ECHOCARDIOGRAM
Timing of the Fetal Echocardiogram
Fetal Views
ASSESSMENT OF CARDIOVASCULAR PERFORMANCE
CONGENITAL HEART LESIONS
Ventricular Septal Defects
Atrioventricular Septal Defects
Transposition of the Great Arteries
Congenitally Corrected Transposition of the Great Arteries
Tetralogy of Fallot and Pulmonary Atresia with VSD
Truncus Arteriosus
Double-Outlet Right Ventricle
Aortopulmonary Window
Pulmonary Atresia with Intact Ventricular Septum
Aortic Arch Hypoplasia and Coarctation of the Aorta
Hypoplastic Left Heart Syndrome, Hypoplastic Left Heart Complex, and Fetal Aortic Stenosis
Other Single-Ventricle Lesions
Tricuspid Valve Disorders
Anomalous Pulmonary Venous Connection
Heterotaxy
OTHER FETAL CARDIOVASCULAR CONDITIONS
Cardiomyopathy
Tumors
Pericardial Effusions
EXTRACARDIAC CONDITIONS WITH CARDIAC MANIFESTATIONS
Twin-Twin Transfusion Syndrome
Twin Reversed Arterial Perfusion
Congenital Diaphragmatic Hernia
Sacrococcygeal Teratoma/Vascular Malformation
Ectopia Cordis/Pentalogy of Cantrell
Omphalocele and Gastroschisis
FETAL ARRHYTHMIA
Irregular Rhythm
Bradycardia
Tachycardia
GENETIC DISORDERS AND ASSOCIATIONS WITH CHD
FETAL CARDIAC INTERVENTION
Pharmacologic
Ultrasound
Invasive Procedures
NOVEL FETAL IMAGING MODALITIES
Fetal Cardiac Magnetic Resonance Imaging
Myocardial Deformation Imaging
3D and 4D Echocardiography
PARENTAL COUNSELING
ACKNOWLEDGMENTS
Section II: Structure and Function of the Cardiovascular System
6 Cardiac Anatomy and Examination of Cardiac Specimens
MEDIASTINUM
General Features
Cardiac Size
Cardiac Position
PERICARDIUM
General Features
Parietal Pericardium
Visceral Pericardium (Epicardium)
Pericardial Reflection
EXTERNAL TOPOGRAPHY
General Features
Base-Apex Characteristics
External Landmarks
Chambers and Great Vessels
GREAT VEINS
Superior Vena Cava
Inferior Vena Cava
Coronary Sinus
Pulmonary Veins
ATRIA
General Features
Right Atrium
Left Atrium
Comparison of the Atria
ATRIOVENTRICULAR VALVES
General Features
Tricuspid Valve
Mitral Valve
Comparison of AV Valves
VENTRICLES
General Features
Right Ventricle
Left Ventricle
Comparison of Ventricles
SEMILUNAR VALVES
General Features
Pulmonary Valve
Aortic Valve
Comparison of Semilunar Valves
Base of the Heart
GREAT ARTERIES
General Features
Pulmonary Arteries
Aorta
Ductus Arteriosus
CORONARY CIRCULATION
Coronary Arteries
Coronary Veins
Cardiac Lymphatics
CARDIAC CONDUCTION SYSTEM
General Features
Sinus Node
Internodal Tracts
Atrioventricular Node
Atrioventricular (His) Bundle
Bundle Branches
Cardiac Innervation
EXAMINATION OF CARDIAC SPECIMENS
General Features
Inflow-Outflow Method
Base-of-Heart Method
Window Method
Tomographic Method
Photography of Cardiac Specimens
7 Classification and Terminology of Cardiovascular Anomalies
PERSPECTIVES ON NOMENCLATURE
Diversity of Terminology
Unity from Diversity
SEQUENTIAL SEGMENTAL ANALYSIS
CARDIAC POSITION AND APICAL DIRECTION
Location in the Chest
Orientation in the Chest
VISCERAL SIDEDNESS (SITUS)
Isomerism and Splenic Anomalies
Cardiac Sidedness (Situs)
Pulmonary Sidedness (Situs)
Abdominal Sidedness (Situs)
MORPHOLOGY OF CARDIAC SEGMENTS
Superior Vena Cava
Inferior Vena Cava
Coronary Sinus
Pulmonary Veins
Atria
Atrioventricular Valves
Ventricles
Great Arteries
POSITIONS OF CARDIAC SEGMENTS
Atria
Ventricles
Great Arteries
CONNECTIONS OF CARDIAC SEGMENTS
Venoatrial Connections
Atrioventricular Connections
Ventriculoarterial Connections
Overriding and Straddling Valves
8 Cardiovascular Physiology
VENTRICULAR FUNCTION
THE TRANSLATION OF VENTRICULAR PRESSURE WORK TO FLOW WORK IN THE GREAT ARTERIES
CLINICAL APPLICATIONS
CONTROL OF FLOW IN THE SYSTEMIC CIRCULATION
Central Neural Control
Central Hormonal Control
Local Control by the Endothelium
Local Control by Metabolism
Local Control by Red Blood Cells
Local Control by Autoregulation
Flow to Specific Vascular Beds
CONTROL OF FLOW IN THE PULMONARY CIRCULATION
Pulmonary Arterial Load, Compliance, and Resistance
Passive Regulation of Pulmonary Vascular Tone and Vessel Recruitment
The Role of Intrathoracic Pressure
Local Control of Pulmonary Flow by the Endothelium
Central Neural and Hormonal Control
Oxygen Tension
COUPLING BETWEEN THE CIRCULATION AND TISSUE METABOLISM
The Relationship between Systemic O2 Delivery and Consumption
The Relationship between Systemic O2 Delivery and Consumption after Cardiac Surgery
Systemic O2 Delivery and Consumption in Patients with One Functional Ventricle
Changes in Systemic O2 Delivery during Catecholamine Infusions
THE PHYSIOLOGY OF THE DEVELOPING CIRCULATION
The Central Circulation
Developmental Changes in the Systemic Circulation
Developmental Changes in the Pulmonary Circulation
Physiologic Regulation of the Developing Pulmonary Circulation
ACKNOWLEDGMENT
Section III: Diagnostic and Therapeutic Methods
9 History and Physical Evaluation
CYANOTIC HEART DISEASE
ACYANOTIC HEART DISEASE
ACYANOTIC OR CYANOTIC HEART DISEASE WITH POOR SYSTEMIC PERFUSION
HISTORY
PHYSICAL EXAMINATION
Blood Pressure
Inspection
Palpation
Percussion
Auscultation
MISCELLANEOUS BUT INTERESTING FINDINGS
10 Exercise Screening and Sports Participation
PHYSICAL ACTIVITY AND EXERCISE
Types of Physical Activity
Promoting Physical Activities and Exercise in Patients with Congenital Heart Disease
PREPARTICIPATION SCREENING FOR UNDIAGNOSED CARDIAC CONDITIONS
Incidence of Sudden Cardiac Death
Purpose of Preparticipation Screening
Current Preparticipation Screening Recommendations
Benefits of Mandatory Preparticipation Electrocardiogram Screening
Concerns About Mandatory Preparticipation Electrocardiogram Screening
Electrocardiogram Analysis
Economic Consequence of Preparticipation Screening
SPECIFIC CONGENITAL CARDIAC LESIONS
Shunt Lesions
Left-Sided Obstructive Lesions
Bicuspid Aortic Valves
Coarctation of the Aorta
Right-Sided Obstructive Lesions
DEFECTS ASSOCIATED WITH SUDDEN CARDIAC DEATH IN ATHLETES
Congenital Coronary Artery Abnormalities
Acquired Coronary Disease
Hypertrophic Cardiomyopathy
Other Cardiomyopathies
Arrhythmias and Cardiac Channelopathies
Systemic Hypertension
Shared Decision Making
11 Exercise Stress Testing
BIOCHEMISTRY, PHYSIOLOGY, AND CARDIAC INTERACTIONS DURING EXERCISE
Basic Exercise Physiology
Oxygen Cascade and Utilization in Exercise
Cardiac Response and Adaptation to Exercise
Ventilatory Response to Exercise
EXERCISE TESTING
The Exercise Lab
Methods
Exercise Protocols
Contraindications
APPROACH TO CARDIOPULMONARY EXERCISE TEST INTERPRETATION
Peak Exercise Parameters
Submaximal Exercise Parameters
Additional Measures of Ventilation
Electrocardiogram Assessment
CONCLUSION
12 Echocardiography: Basic Principles and Imaging
HISTORY
PHYSICS OF 2D ECHOCARDIOGRAPHY
Eight Equations That Form the Basis of 2D and Doppler Echocardiography
THE EXAMINATION
General Considerations
Defining Anatomy: Segmental Approach
TRANSESOPHAGEAL ECHOCARDIOGRAPHY
Approach
Intraoperative Transesophageal Echocardiography
STRESS ECHOCARDIOGRAPHY
THREE-DIMENSIONAL ECHOCARDIOGRAPHY
Principles of 3D Echocardiography Imaging
Anatomic Imaging in Structural Heart Disease
Quantitation of Cardiac Chamber Volumes and Function
Three-Dimensional Guidance of Percutaneous Cardiac Interventional Procedures
ECHOCARDIOGRAPHIC EVALUATION OF INTERVENTIONAL PROCEDURES
TEE Guidance for ASD Device Closure
TEE Guidance for VSD Device Closure
Intracardiac Echocardiography
CONTRAST ECHOCARDIOGRAPHY
Agitated Saline Contrast Echocardiography
Transpulmonary Contrast Echocardiography
ACKNOWLEDGMENTS
13 Echocardiographic Assessment of Cardiac Dimensions, Cardiac Function, and Valve Function
QUANTIFICATION OF CHAMBER DIMENSIONS AND CARDIAC STRUCTURES
QUANTIFICATION OF THE LEFT VENTRICLE
2D and 3D Techniques
Left Ventricular Mass
QUANTIFICATION OF THE RIGHT VENTRICLE
QUANTIFICATION OF THE UNIVENTRICULAR HEART
EVALUATION OF SYSTOLIC VENTRICULAR FUNCTION
VCF-End-Systolic Wall Stress Relationship
Doppler Indices of Ventricular Function
ASSESSMENT OF VALVE FUNCTION
Semilunar Valves and Great Vessels
Atrioventricular Valve Size and Function
Diastolic Ventricular Function
Diastolic Physiology
History of Doppler Diastolic Assessment
Echocardiographic Parameters Used in the Evaluation of Diastolic Function
Transmitral Doppler Flow Evaluation
Pulmonary Venous Doppler Flow Analysis
Doppler Myocardial Velocity Assessment
Estimation of Left Atrial Volume
Color M-mode
Deformation Imaging
Normal Echocardiographic Diastolic Function
ABNORMALITIES OF LEFT VENTRICULAR DIASTOLIC FUNCTION
Impaired Relaxation
Pseudonormal Diastolic Dysfunction
Advanced Diastolic Dysfunction: Restrictive Physiology
Constrictive Pericarditis
Assessment of Diastolic Function during Exercise
Doppler Assessment of Right Ventricular Diastolic Function
EVALUATION OF VENTRICULAR SYNCHRONY
ECHOCARDIOGRAPHIC ASSESSMENT OF THE ATRIOVENTRICULAR DELAY
ASSESSMENT OF INTERVENTRICULAR DYSSYNCHRONY
ASSESSMENT OF INTRAVENTRICULAR DYSSYNCHRONY
ASSESSMENT OF MECHANICAL DYSSYNCHRONY IN CHILDREN
CORONARY ARTERY PHYSIOLOGY
Direct Assessment of the Coronary Arteries
Coronary Perfusion
14 Magnetic Resonance Imaging
INDICATION FOR MAGNETIC RESONANCE IMAGING EVALUATION OF CONGENITAL HEART DISEASE
MAGNETIC RESONANCE IMAGING TECHNIQUES
Background
Cardiac and Respiratory Gating
SEDATION AND MONITORING
PATIENT PREPARATION
SAFETY
SUPERVISION AND INTERPRETATION OF MRI EXAMINATION
CLINICAL APPLICATIONS
CMR Examination Techniques
Assessment of Cardiovascular Anatomy
Assessment of Myocardial Function
Analysis of Regional Wall Motion
Analysis of Myocardial Strain
Stress CMR
Diastolic Function
Flow Analysis
Myocardial Perfusion and Viability
Tissue Characteristics
EXAMPLES OF CLINICAL APPLICATIONS OF CMR
Shunt Lesions
Anomalies of the Aorta
Vascular Rings and Pulmonary Artery Sling
Pulmonary Artery Anomalies
Systemic and Pulmonary Venous Anomalies
Tetralogy of Fallot
Transposition of the Great Arteries
Single Ventricle and Fontan
15 Cardiac Computed Tomography in Children with Congenital Heart Disease
OVERVIEW OF CT TECHNOLOGY
Electron Beam CT
Multidetector CT
New Generation CT
CT TECHNIQUES AND PROTOCOLS
CT Technique
Scanning Strategy
ECG Synchronization
PATIENT PREPARATION
Breath Holding
Premedication
Sedation
INTRAVENOUS CONTRAST AGENTS AND INJECTION PROTOCOL
Contrast Agents
Contrast Reaction
Contrast-Induced Nephropathy
Intravenous Contrast Injection
Contrast Injection Protocols
Timing of the Contrast Bolus
IMAGE POSTPROCESSING
ARTIFACTS IN CARDIAC CT
Image Noise
Pulsation Artifacts
Respiratory Artifacts
High Contrast Artifacts
Inhomogeneous Contrast
Partial Volume Effects
RADIATION REDUCTION ON CT
CHOICE OF CT VERSUS MR FOR PEDIATRIC CARDIAC IMAGING
CLINICAL APPLICATIONS OF PEDIATRIC CARDIAC CT
Systemic Veins
Pulmonary Veins
Pulmonary Arteries
Aorta
Coronary Arteries
Vascular Mediated Airway Compromise
Intracardiac Pathology and Ventricular Function
Postoperative and Postprocedural Indications for CT
Emergent Indications
CONCLUSION
16 Cardiac Catheterization and Angiography
DIAGNOSTIC CARDIAC CATHETERIZATION
Indications
Techniques
ANGIOGRAPHY
Basic Concepts
Contrast Media
Radiation Dose and Exposure
Patient Dose
Cath Lab Personnel Exposure
Angiography
Imaging of the Biventricular Heart
Specific Techniques
Complications
ACKNOWLEDGMENTS
SELECTED READINGS
17 Therapeutic Cardiac Catheterization
PROCEDURAL EFFICACY AND ADVERSE EVENTS
HOW TO CREATE, ENLARGE AND MAINTAIN INTRAATRIAL COMMUNICATIONS
Balloon Atrial Septostomy
Other Interventions to Enlarge or Maintain an Atrial Communication
Perforating an (Intact) Atrial Septum
BALLOON AORTIC VALVULOPLASTY
BALLOON PULMONARY VALVULOPLASTY
PERFORATION OF THE ATRETIC PULMONARY VALVE
STENTING OF THE PATENT ARTERIAL DUCT
TRANSCATHETER TREATMENT OF AV VALVES IN THE CONGENITAL POPULATION
Mitral Valve Interventions
Tricuspid Valve Interventions
TRANSCATHETER MANAGEMENT OF COARCTATION, RECOARCTATION 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
Devices for Occlusion of Septal Defects
Transcatheter Closure of Atrial Septal Defects
TRANSCATHETER CLOSURE OF FONTAN FENESTRATIONS
TRANSCATHETER CLOSURE OF VENTRICULAR SEPTAL DEFECTS
Background and History
Technique of Closure of Muscular VSDs Using the AMPLATZER Muscular VSD Occluder
Results of Transcatheter Closure of Muscular VSDs
TRANSCATHETER OCCLUSION OF PATENT DUCTUS ARTERIOSUS AND OTHER PERSISTENT OR ABNORMAL VASCULAR COMMUNICATIONS
Devices for PDA and Vascular Occlusion
Technique of PDA Occlusion
Complications of PDA Occlusion
CLOSURE OF OTHER PERSISTENT OR ABNORMAL VASCULAR COMMUNICATIONS
RETRIEVAL OF FOREIGN BODIES
TRANSCATHETER PULMONARY VALVE REPLACEMENT
HYBRID PROCEDURES
HYPOPLASTIC LEFT HEART SYNDROME
PERVENTRICULAR CLOSURE OF VENTRICULAR SEPTAL DEFECTS
INTRAOPERATIVE STENT PLACEMENT AND OTHER HYBRID PROCEDURES
CONCLUSION
Section IV: Electrophysiology
18 Anatomy of the Cardiac Conduction System in Congenital Heart Disease
NORMAL ANATOMY OF THE CARDIAC CONDUCTION SYSTEM
Sinoatrial Conduction System
Atrioventricular Conduction System
Ventricular Conduction
THE CARDIAC CONDUCTION SYSTEM IN CONGENITAL HEART DISEASE
Atrial Septal Defects
Ventricular Septal Defects
Atrioventricular Septal Defects
Ebstein Anomaly
Tricuspid Atresia
Congenitally Corrected Transposition of the Great Arteries
Double Inlet Left Ventricle
Heterotaxy
19 The Normal Electrocardiogram
THE HISTORY OF ELECTROCARDIOGRAPHY
PRINCIPLES AND TECHNICAL CONSIDERATIONS IN RECORDING THE ELECTROCARDIOGRAM
The Scalar Electrocardiogram
Vectorcardiography
Body Surface Mapping and Noninvasive Electrophysiologic Imaging
Esophageal Electrocardiography
Signal-Averaged Electrocardiography
24-Hour Ambulatory Electrocardiography
Wearables
HOW TO READ A PEDIATRIC ELECTROCARDIOGRAM
Rate and Rhythm
ST Segment, J Waves, T Waves, and U Waves
Specific Abnormalities Affecting the ST Segment, T Wave, and U Wave
THE NORMAL ELECTROCARDIOGRAM
Developmental Changes
The Premature Infant
The Athlete
Gender and Racial Differences
GUIDELINES AND INDICATIONS
20 Cardiac Channelopathies, Syncope, and Sudden Cardiac Death
THE PREVALENCE AND CAUSES OF SUDDEN DEATH IN THE YOUNG
AUTOPSY-NEGATIVE SUDDEN UNEXPLAINED DEATH
Assessment of Family Members
Molecular Autopsy Investigations of SUD
THE CARDIAC CHANNELOPATHIES
Autosomal Dominant Long QT Syndrome (Romano-Ward syndrome in the Past, Now LQTS)
Autosomal Recessive LQTS (Jervell and Lange-Nielsen Syndrome or JLNS)
Multisystem or Complex LQTS
Short QT Syndrome
Catecholaminergic Polymorphic Ventricular Tachycardia
Calmodulinopathies
Triadin Knockout Syndrome
Brugada Syndrome
DISTINGUISHING FEATURES OF "COMMON YET BENIGN" SYNCOPE FROM "SUDDEN DEATH WARNING SIGN" SYNCOPE
21 Electrophysiology Studies and Electrophysiologic Therapeutic Catheterization
INTRODUCTION
ELECTROPHYSIOLOGIC STUDIES: DIAGNOSTIC
PLANNING THE STUDY
INTRACARDIAC TECHNIQUE
Educational and Emotional Preparation of Patient and Family
Sedation and Anesthesia
Preparation of the Patient in the Catheterization Laboratory
Sheath and Catheter Placement
Electrode Catheters
Recording and Stimulation Technique
Administration of Drugs
Complications
TRANSESOPHAGEAL TECHNIQUE
Educational and Emotional Preparation of Patient and Family
Sedation
Preparation of Patient in the Procedure Room
Catheter Placement
Equipment and Recording/Stimulation Technique
Administration of Drugs
Complications
OBJECTIVES OF DIAGNOSTIC ELECTROPHYSIOLOGIC STUDIES AND COMPARISON OF INTRACARDIAC AND TRANSESOPHAGEAL TECHNIQUES
ELECTROPHYSIOLOGIC STUDIES: THERAPEUTIC CATHETERIZATION
MAKING THE DECISION TO ABLATE
ENERGY SOURCES
Direct Current
Radiofrequency
Cooled-Tip Radiofrequency
Cryotherapy
Other
Summary: Energy Sources
PROCEDURE: GENERAL
Preablation
Three-Dimensional Electroanatomic Mapping
Fluoroscopic Exposure
Epicardial Ablation Techniques
PROCEDURE: SUBSTRATE SPECIFIC
Accessory Pathways
Mapping
Permanent Form of Junctional Reciprocating Tachycardia
Atrioventricular Nodal Reentrant Tachycardia
Ablation Energy for AV Node Modification: Cryoenergy versus RF
Ectopic Atrial Tachycardia
Atrial Flutter or Fibrillation in the Absence of Other Heart Disease
Intra-atrial Reentry Tachycardia in the Presence of Congenital Heart Disease
Junctional Ectopic Tachycardia
Ventricular Tachycardia
COMPLICATIONS AND FOLLOW-UP
SPECIAL CONSIDERATIONS FOR PEDIATRICS
Age (Infants)
Size
Ablation on Extracorporeal Membrane Oxygenation
Pre-excitation Syndromes in Patients with Structural Congenital Heart Disease
Atrioventricular Discordance
Twin AV Nodes
Pre-excitation and Congenital Heart Disease: Summary
SUMMARY
22 Disorders of Cardiac Rhythm and Conduction
INTRODUCTION
SINUS NODE
ANATOMY OF THE ATRIOVENTRICULAR NODE
TACHYCARDIA
Clinical Presentation
Evaluation
Characterization of Tachycardias
NARROW COMPLEX ARRHYTHMIAS
Sinus Arrhythmia
Supraventricular Tachycardias
Accessory Pathway-Mediated Tachycardia
Wolff-Parkinson-White
Concealed or Unidirectional Retrograde Accessory Pathway
Permanent Junctional Reciprocating Tachycardia
Mahaim Fibers
Automatic Focus (Atrial Ectopic Tachycardia)
Nonautomatic Focal Atrial Tachycardia
Chaotic Atrial Tachycardia
Junctional Ectopic Tachycardia
Atrial Flutter
Atrial Fibrillation
Intraatrial Reentrant Tachycardia
Treatment of Supraventricular Arrhythmias
Wide Complex Tachycardias
Ventricular Tachycardia
Tachycardia-Induced Cardiomyopathy
Premature Atrial Contractions
Premature Ventricular Contractions
Postoperative Arrhythmias
TREATMENT
Class I
Class II
Class III
Class IV
Class V
Adenosine
Magnesium
Other Therapies
BRADYCARDIA
Sinus Bradycardia
Treatment
Sinus Node Dysfunction
Etiology of Sinus Node Dysfunction
Treatment
Disorders of Atrioventricular Conduction
First-Degree Atrioventricular Block
Second-Degree Atrioventricular Block
Complete Atrioventricular Block
Symptoms and Treatment
Bundle Branch Block
PACEMAKERS AND IMPLANTABLE CARDIOVERTER DEFIBRILLATORS
Biventricular Pacing
Follow-Up
SUMMARY
Section V: Pediatric Cardiac Intensive Care
23 Physiology of the Preterm and Term Infant
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
24 Cardiovascular Intensive Care
ORGANIZATION OF CARDIAC INTENSIVE CARE SERVICES
Multidisciplinary Team Approach to Pediatric Cardiac Intensive Care
Relationship between the PCICU and the Cardiac OR and Catheterization Laboratory
Continuous Quality Improvement and Patient Safety in the CICU
Family-Centered Care
EVALUATION AND MANAGEMENT OF THE CARDIOVASCULAR SYSTEM
Physical Examination, Bedside, and Laboratory Data
Echocardiography
Venous Oximetry
Near-Infrared Spectroscopy
Thermodilution Cardiac Output
Lithium Dilution and Pulse Contour Analysis Cardiac Output
PREVENTION AND MANAGEMENT OF LOW CARDIAC OUTPUT SYNDROME
Determinants of Cardiac Output and Oxygen Delivery
Pharmacologic Therapy for Congenital Heart Disease
Inotropes
Chronotropes
Vasoconstrictors
Vasodilators
ß-Adrenergic Antagonists
Newer Cardiotonic and Vasoactive Agents
Mechanical Support
ARRHYTHMIAS
Etiology and Incidence
Diagnosis
Prevention and Treatment
PULMONARY HYPERTENSION
Etiologies
Diagnosis
Treatment
THE PULMONARY SYSTEM, VENTILATION, AND AIRWAY MANAGEMENT
Cardiorespiratory Interactions
Endotracheal Intubation and Airway Management
Conventional Ventilation Strategies
Weaning from Mechanical Ventilation
Alternate Ventilation Strategies-Noninvasive Ventilation
Early Tracheal Extubation
High-Frequency Oscillatory Ventilation
GENERAL POSTOPERATIVE CARE
Handoff
Effects of Cardiopulmonary Bypass
Bleeding
Delayed Sternal Closure
PERIOPERATIVE CONSIDERATIONS FOR SPECIFIC LESIONS AND PROCEDURES
Hypoplastic Left Heart Syndrome
Transposition of the Great Arteries
Truncus Arteriosus
Total Anomalous Pulmonary Venous Return
Systemic to Pulmonary Artery Shunts
Tetralogy of Fallot
Ventricular Septal Defect, Atrioventricular Septal Defect
Coarctation of the Aorta, Aortic Arch Hypoplasia, and Interruption
Bidirectional Cavopulmonary Connection
Fontan Completion
Other Left-Sided Obstructive Lesions
Other Right-Sided Obstructive Lesions
Cardiac Transplantation
CENTRAL NERVOUS SYSTEM ISSUES
Sedation and Analgesia
CNS Injury: Etiologies, Prevention, Evaluation, Treatment
THE RENAL SYSTEM
Renal Replacement Therapy
FLUIDS, ELECTROLYTES, AND NUTRITION
Glycemic Control
INFECTION IN THE CVICU
Prevention of Infection
Diagnosis and Treatment: Catheter-Related, Bloodstream, Pulmonary, Urinary, Wound Infections
ENDOCRINE ISSUES
Adrenal Insufficiency and Corticosteroids
Hypothyroidism
CPR AND ECPR IN THE CVICU
THE CATHETERIZATION LABORATORY AND THE CVICU
Care of Postcatheterization Patients
Catheterization of ICU Patients
MEDICAL CONDITIONS IN THE CVICU
THE LOW-BIRTH-WEIGHT INFANT
THE ADULT WITH CONGENITAL HEART DISEASE
CONCLUSIONS
25 Mechanical Circulatory Support in Children
INTRODUCTION
INDICATIONS FOR MECHANICAL CIRCULATORY SUPPORT
Bridge to Transplantation
Bridge to Recovery
CONTRAINDICATIONS TO MECHANICAL CIRCULATORY SUPPORT
CANDIDATE SELECTION
SHORT-TERM MCS EITHER AS A BRIDGE TO RECOVERY OR BRIDGE TO TRANSPLANTATION
Extracorporeal Membrane Oxygenation
Management
LONG-TERM MCS SUPPORT IN CHILDREN
SPECIAL CONSIDERATIONS
Congenital Heart Disease
MCS in Children with Cardiac Graft Dysfunction
MCS in Children with Restrictive Physiology/Cardiomyopathy
MCS Chronic (aka Destination) Therapy in Children
26 Cardiopulmonary and Right-Left Heart Interactions
COMPARISON OF RIGHT AND LEFT HEART HEMODYNAMICS
CARDIOPULMONARY INTERACTIONS IN THE NORMAL CIRCULATION
Cardiopulmonary Interactions in Congenital Heart Disease
Reduced Right Ventricular Contractile Performance
CARDIOPULMONARY INTERACTIONS AND THE ABNORMAL RIGHT HEART
Abnormal Right Ventricular Diastolic Function
Exclusion of the Right Ventricle
CARDIOPULMONARY INTERACTIONS AND THE LEFT HEART
RIGHT-LEFT HEART INTERACTIONS
The Effects of the Left Ventricle on the Right Ventricle
The Effects of the Right Ventricle on the Left Ventricle
Right-Left Heart Interactions in Congenital Heart Disease
Functional Interactions
Geometric Interactions
CONCLUSIONS
27 Cardiac Trauma
TYPES OF CARDIAC INJURY
BLUNT CARDIAC INJURY
Pericardial Injury
Myocardial Contusion
Cardiac Rupture
Septal Disruption
Ventricular Aneurysm
Injury to Heart Valves and Supporting Structures
Great Vessel Injury
Brachiocephalic Arteries
Vena Caval Injury
Coronary Artery Injury
PENETRATING CARDIAC INJURY
DIAGNOSTIC MODALITIES AND CARDIAC TRAUMA
ACKNOWLEDGMENTS
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
Lutembacher Syndrome
PATHOPHYSIOLOGY
CLINICAL PRESENTATION
History
Physical Examination
DIAGNOSTIC EVALUATION
Electrocardiogram and Electrophysiology
Chest X-Ray
Echocardiogram
Cardiac Catheterization
Other Imaging Modalities: CT/MRI
Exercise Testing
NATURAL HISTORY OF ASDs
Life Expectancy
Defect Size and Spontaneous Closure
Pulmonary Hypertension
Atrial Arrhythmias
MANAGEMENT
Secundum ASDs
Sinus Venosus ASD
Coronary Sinus ASD
Management Issues during Follow-Up
ASD in Adults
Atrial Septal Aneurysm
Patent Foramen Ovale
29 Atrioventricular Septal Defects
INTRODUCTION AND NOMENCLATURE
DEMOGRAPHICS
EMBRYOGENESIS
PARTIAL ATRIOVENTRICULAR SEPTAL DEFECT
Pathology
Clinical Manifestations
Echocardiography
Radiography
Electrocardiography and Electrophysiology
Cardiac Catheterization and Angiography
Special Forms of Partial Atrioventricular Septal Defect
COMPLETE ATRIOVENTRICULAR SEPTAL DEFECT
Pathology
Rastelli Classification for Complete AVSD
Clinical Manifestations
Echocardiography
The Concept of "Balance"
Radiography
Electrocardiography
Cardiac Catheterization and Angiography
Clinical Course
Special Forms of Complete Atrioventricular Septal Defect
INITIAL SURGICAL TREATMENT OF AVSD
Partial AVSD
Complete AVSD
Special Problems in Complete AVSD Surgery
REOPERATION AFTER REPAIR OF AVSD
Partial AVSD
Complete AVSD
POSTOPERATIVE ECHOCARDIOGRAPHIC ASSESSMENT
THE ADULT WITH ATRIOVENTRICULAR SEPTAL DEFECT
Previously Undiagnosed Adults with AVSD
Adults with Previously Repaired AVSD
Recommendations for Pregnancy
CONCLUSIONS
ACKNOWLEDGMENTS
30 Ventricular Septal Defects
EPIDEMIOLOGY
ETIOLOGY
MORPHOLOGY
Central Perimembranous VSDs
Inlet VSDs
Muscular Trabecular VSDs
Outlet VSDs
Malalignment VSDs
CONDUCTION
ASSOCIATED LESIONS
PHYSIOLOGY
CLINICAL FEATURES
Presentation
Atypical Findings
Electrocardiography
Radiography
Echocardiography
Cardiac Magnetic Resonance and Computed Tomography
Catheterization and Angiography
NATURAL HISTORY
Natural History Studies
DIFFERENTIAL DIAGNOSIS
MEDICAL MANAGEMENT
INTERVENTIONAL MANAGEMENT
OUTCOMES
YOUNG ADULTS
Part B: Arterial Abnormalities
31 Patent Ductus Arteriosus and Aortopulmonary Window
PATENT DUCTUS ARTERIOSUS
Introduction
Epidemiology
Etiology
Morphology
Physiology
Clinical Features
Investigation
Natural History
Differential Diagnosis
Management
AORTOPULMONARY WINDOW
Introduction and Epidemiology
Etiology
Morphology
Physiology
Investigation
Management
Outcomes
32 Congenital Anomalies of the Coronary Vessels and the Aortic Root
CONGENITAL CORONARY ARTERY ANOMALIES
Embryology of Normal Coronary Development
Normal Coronary Anatomy
CONGENITAL ANOMALIES OF CORONARY ARTERIES IN THE ABSENCE OF STRUCTURAL HEART DISEASE
Anomalous Origin of a Coronary Artery from the Pulmonary Artery
Anomalous Aortic Origin of a Coronary Artery
CORONARY ARTERY FISTULAE
Pathophysiology/Clinical Features
Diagnostic Testing
Management
CONGENITAL CORONARY ARTERY ANOMALIES ASSOCIATED WITH OTHER CONGENITAL HEART DEFECTS
Transposition of the Great Arteries
Tetralogy of Fallot
Truncus Arteriosus
Pulmonary Atresia with Intact Ventricular Septum
Hypoplastic Left Heart Syndrome
Supravalvular Aortic Stenosis
CONGENITAL ANOMALIES OF THE AORTIC ROOT
Aortic-Left Ventricular Tunnel
Aneurysms of the Sinus of Valsalva
33 Aortic Arch and Vascular Anomalies
Normal Anatomy
Normal Embryology
ANOMALIES OF THE AORTIC ARCH
Mirror-Imaged Right Aortic Arch
Aberrant Origin of the Subclavian Artery
Aberrant Origin of the Subclavian Artery with Associated Diverticulum of Kommerell
Retroesophageal Brachiocephalic Artery
Isolated Branches of the Aortic Arch
Right Aortic Arch with Isolated Brachiocephalic Artery
Double Aortic Arch
Persistent Fifth Aortic Arch
Cervical Aortic Arch
Pulmonary Artery Sling
Anomalous Origin of the Left or Right Pulmonary Artery from Aorta
Brachiocephalic Artery Compression of the Trachea
Interrupted Aortic Arch
Putting It Together: Approaching the Patient with Suspected Aortic Arch Anomaly
MALFORMATIONS OF THE SYSTEMIC VASCULATURE
Vein of Galen Aneurysmal Malformation
Infantile Hemangiomas
PULMONARY ARTERIOVENOUS MALFORMATIONS
Anatomy
Epidemiology and Etiology
Clinical Manifestations
Diagnostic Findings
Management and Outcome
34 Abnormalities of the Aortic Root
MARFAN SYNDROME
Diagnosis
Therapy
BICUSPID AORTIC VALVE
Diagnosis
Therapy
TETRALOGY OF FALLOT
COARCTATION OF THE AORTA
TRANSPOSITION OF THE GREAT ARTERIES
OTHER LESIONS
CONCLUSIONS
Part C: Venous Abnormalities
35 Anomalies of the Pulmonary Veins
ANATOMY
Anomalous Connections and Drainage
Stenotic Connections
Abnormal Numbers of Pulmonary Veins
EMBRYOLOGY
Early Atresia of the Common Pulmonary Vein while Pulmonary-Systemic Venous Connections Are Still Present
Late Atresia of the Common Pulmonary Vein after Pulmonary-Systemic Connections Are Obliterated
Stenosis of the Common Pulmonary Vein
Anomalous Incorporation of the Common Pulmonary Vein into the Left Atrium
Early Atresia of the Common Pulmonary Vein while Pulmonary-Systemic Venous Connections Are Still Present
Normal Absorption of the Common Pulmonary Vein with Partially or Totally Abnormal Pulmonary Venous Drainage and Normal Connection of the Pulmonary Veins
PARTIALLY ANOMALOUS PULMONARY VENOUS CONNECTIONS
Anatomy
Physiology
Clinical Features
Associated Cardiac Defects and Syndromes
Diagnostic Features
Differential Diagnosis
Treatment
Prognosis
TOTALLY ANOMALOUS PULMONARY VENOUS CONNECTION OR DRAINAGE
Genetics and Epidemiology
Anatomy
Anatomic Sites of Obstruction to Pulmonary Venous Drainage
Associated Cardiac Anomalies
Microscopic Anatomy
Physiology
Clinical Manifestations
Differential Diagnosis
Treatment
Prognosis
ATRESIA OF THE COMMON PULMONARY VEIN AFTER THE PULMONARY SYSTEMIC CONNECTIONS ARE OBLITERATED
Atresia of the Common Pulmonary Vein
STENOSIS OF THE COMMON PULMONARY VEIN
Cor Triatriatum
ABNORMAL ABSORPTION OF THE COMMON PULMONARY VEIN INTO LEFT ATRIUM
Stenosis of the Individual Pulmonary Veins
36 Abnormal Systemic Venous Connections
EMBRYOLOGY
Normal Development of the Cardinal and Umbilicovitelline Venous Systems
Normal Development of the Right Superior Vena Cava and the Coronary Sinus
Normal Development of the Inferior Vena Cava
Normal Development of the Ductus Venosus
Azygos and Hemiazygos Veins
Normal Development of the Portal Vein
ANOMALIES OF THE SUPERIOR VENAE CAVAE
Bilateral Superior Venae Cavae with Normal Drainage to the Right Atrium
Bilateral Superior Venae Cavae with an Unroofed Coronary Sinus
Absent Right Superior Vena Cava in Visceroatrial Situs Solitus
Left Atrial or Biatrial Drainage of Right Superior Vena Cava
Retroaortic Innominate Vein
ANOMALIES OF THE CORONARY SINUS
Coronary Sinus Defect and Unroofed Coronary Sinus
Coronary Sinus Orifice Atresia
Coronary Sinus Aneurysm or Diverticulum
ANOMALIES OF THE INFERIOR VENA CAVA
Interrupted Inferior Vena Cava
Bilateral Inferior Venae Cavae
Inferior Vena Cava Drainage to the Left Atrium
ANOMALIES OF THE DUCTUS VENOSUS
Anomalous Termination of the Umbilical Veins and Absent Ductus Venosus
Postnatal Persistence of the Ductus Venosus
PERSISTENT VALVES OF THE SINUS VENOSUS
Embryology of the Valves of the Sinus Venosus
Right Ventricular Outflow Tract Obstruction
Tricuspid Valve Obstruction
37 Vascular Tumors and Malformations
EMBRYOLOGY AND GENETICS
CLASSIFICATION
CLINICAL MANIFESTATIONS AND TREATMENT
Vascular Tumors
Vascular Malformations
PHARMACOTHERAPY OF VASCULAR MALFORMATIONS
Sildenafil
Sirolimus
ACKNOWLEDGMENT
Part D: The Right Ventricle
38 Tricuspid Valve Disorders: Atresia, Dysplasia, and Ebstein Anomaly
TRICUSPID VALVE ATRESIA
Pathology
Genetics
Hemodynamics
Clinical Findings
Clinical Management
EBSTEIN ANOMALY
Introduction
Anatomic Features
Genetics
Clinical Presentation
Diagnostic Testing
Electrophysiology Testing and Preoperative Ablation
Medical Management
Short-Term Outcome after Surgery for Ebstein Anomaly
Long-Term Outcome after Surgery for Ebstein Anomaly
NON-EBSTEIN TRICUSPID VALVE REGURGITATION: CONGENITAL AND ACQUIRED
UHL ANOMALY
ACKNOWLEDGMENTS
39 Pulmonary Stenosis
ISOLATED VALVAR PULMONARY STENOSIS
Embryology and Pathology
Physiology
Manifestations
Differential Diagnosis
PULMONARY STENOSIS ASSOCIATED WITH SYSTEMIC DISEASES
Treatment
Assessment of Severity, Course, and Prognosis
Indications for Pulmonary Valve Replacement for Induced Pulmonary Regurgitation Following Relief of Pulmonary Valve Stenosis
PULMONARY VALVE STENOSIS IN YOUNG ADULTS
SUBVALVULAR RIGHT VENTRICULAR OUTFLOW TRACT OBSTRUCTION
RIGHT VENTRICULAR OBSTRUCTION
Double-Chambered Right Ventricle
Pathology
Physiology
Manifestations
Cardiac Catheterization
Angiocardiography
Magnetic Resonance Imaging
Treatment
Course and Prognosis
DOUBLE-CHAMBERED RIGHT VENTRICLE IN ADULTS
PERIPHERAL PULMONARY ARTERY STENOSIS WITH INTACT VENTRICULAR SEPTUM
Embryology and Pathology
Physiology
Manifestations
Differential Diagnosis
Treatment
Course and Prognosis
PULMONARY ARTERY STENOSIS IN YOUNG ADULTS
40 Pulmonary and Atresia Intact Ventricular Septum
ROBERT MARK FREEDOM 1941-2005
EPIDEMIOLOGY
DEFINITION
MORPHOGENESIS
PATHOLOGY AND PHYSIOLOGY
Gross External Inspection
Nature of the Pulmonary Atresia
Great Veins, Atrial Septum, Coronary Sinus, and Venous Valves
Tricuspid Valve
Right Ventricle
Left Atrium, Left Ventricle, and Aortic Valve
Pulmonary Circulation
Myocardial Abnormalities
Coronary Arteries
Right Ventricular-Dependent Coronary Artery Circulation
CLINICAL FEATURES
Physical Examination
Radiographic Features
Electrocardiographic Features
Echocardiographic Features
Cardiac Catheterization
TREATMENT
Medical
Surgical
Management of the Patient Whose Coronary Circulation Is in Large Part Right Ventricular Dependent
Extreme Ebstein/Dysplastic Tricuspid Valve and True Pulmonary Atresia
Transcatheter Techniques in the Management of Pulmonary Atresia and Intact Ventricular Septum
TREATMENT OF THE ADULT
SUMMARY
41 Tetralogy of Fallot with Pulmonary Stenosis, Pulmonary Atresia, and Absent Pulmonary Valve
EPIDEMIOLOGY
GENETICS
Syndromic Tetralogy of Fallot
Nonsyndromic Tetralogy of Fallot
Genetic Testing and Counseling
ANATOMY (MORPHOLOGIC, ECHOCARDIOGRAPHIC, AND ANGIOGRAPHIC CORRELATIONS)
The Interventricular Communication
Aortic Position
Right Ventricular Outflow Tract Obstruction
Variations in Pulmonary Artery Anatomy
Aortopulmonary Collateral Arteries and the Patent Arterial Duct
Coronary Arteries
Aortic Arch
Other Associated Cardiac Abnormalities
CLINICAL FEATURES AND INVESTIGATION
Fetal Diagnosis
Postnatal Presentation and Natural History
Electrocardiographic Findings
Radiographic Findings
Echocardiography
Angiography and Cross-Sectional Imaging
MEDICAL TREATMENT
PERCUTANEOUS PALLIATION
THE EVOLUTION OF SURGICAL TREATMENT
Palliative Procedures
"Complete" Surgical Repair
Surgical Outcomes
Surgery for Tetralogy with Pulmonary Atresia
POSTOPERATIVE PATHOPHYSIOLOGY
Pulmonary Regurgitation and the Fate of Right Ventricle
Myocardial Fibrosis
Mechanoelectrical Interactions, Arrhythmia, and Sudden Cardiac Death
Right Ventricular Diastolic Dysfunction
Ventricular-Ventricular Interactions and the Fate of the Left Ventricle
ISSUES TO CONSIDER DURING LIFELONG FOLLOW-UP
The Role and Timing of Pulmonary Valve Replacement
Infective Endocarditis
The Fate of the Aortic Root
Follow-up Intervals, Imaging and Other Testing
Contraception and Pregnancy
42 Truncus Arteriosus
EMBRYOLOGY
PATHOLOGY
MANIFESTATIONS
Prenatal Diagnosis
Clinical Features
Physical Examination
Electrocardiographic Features
Radiologic Features
Echocardiographic Features
Cardiac Catheterization and Angiocardiography
Magnetic Resonance Imaging and Cardiac Computed Tomography
DIFFERENTIAL DIAGNOSIS
NATURAL HISTORY
TREATMENT
SURGICAL CORRECTION/INTERVENTIONAL CARDIAC CATHETERIZATION
CARE OF THE ADULT PATIENT WITH REPAIRED TRUNCUS ARTERIOSUS
LONG-TERM ISSUES
Part E: Left Ventricular Inflow and Outflow Abnormalities
43 Anatomical and Functional Mitral Valve Abnormalities in the Pediatric Population
MITRAL VALVE DEVELOPMENT
INCIDENCE OF MITRAL VALVE ABNORMALITIES
MITRAL VALVE MORPHOLOGY AND FUNCTION
Mitral Annulus
Mitral Valve Leaflets and Supporting Apparatus
MITRAL VALVE PATHOLOGY: AN INTEGRATED MORPHOLOGIC AND HEMODYNAMIC APPROACH
Mitral Valve Dysplasia and Hypoplasia
Dysplasia of the Posterior or Mural Leaflet
Supravalvar Mitral Stenosis
Parachute Mitral Valve
Mitral Arcade
Double-Orifice Mitral Valve
Cleft Mitral Valve
The Mitral Valve in Congenitally Corrected Transposition of the Great Arteries
Straddling of the Mitral Valve
HEMODYNAMIC ASSESSMENT OF THE MITRAL VALVE
ASSESSMENT OF MITRAL VALVE REGURGITATION
Vena Contracta Size
Left Atrial and Left Ventricular Size
Pulmonary Venous Doppler Pattern
Continuous Doppler Regurgitant Jet Profile
Mitral E Velocity Dominance
QUANTITATIVE ASSESSMENT OF MITRAL REGURGITATION BY DOPPLER
Pulsed Doppler Assessment of Regurgitant Fraction, Volume, and EROA
Proximal Isovelocity Surface Area or Flow Convergence
MITRAL VALVE PROLAPSE
Echocardiographic Assessment of Mitral Valve Prolapse
CLINICAL PRESENTATION
ELECTROCARDIOGRAPHY
RADIOGRAPHY
MAGNETIC RESONANCE IMAGING
HEMODYNAMIC EVALUATION
MANAGEMENT AND PROGNOSIS OF CONGENITAL MITRAL VALVE STENOSIS
Medical Management
Balloon versus Surgical Management
Surgical Repair
Mitral Valve Replacement
MANAGEMENT AND PROGNOSIS OF CONGENITAL MITRAL REGURGITATION
Surgical Management
MANAGEMENT AND PROGNOSIS OF MITRAL VALVE PROLAPSE
ACKNOWLEDGMENT
44 Aortic Stenosis
INTRODUCTION
EPIDEMIOLOGY
EMBRYOLOGY AND PATHOLOGY
Valvar Aortic Stenosis
Subvalvar Aortic Stenosis
Supravalvar Aortic Stenosis
MOLECULAR GENETICS
Valvar Aortic Stenosis
Subvalvar Stenosis
Supravalvar Stenosis
PHYSIOLOGY
CLINICAL FEATURES AND DIAGNOSTIC METHODS
Symptoms
Physical Examination Findings
Electrocardiography
Radiography
Echocardiography
MRI
Exercise Testing
Cardiac Catheterization
NATURAL HISTORY
Valvar Aortic Stenosis
Subvalvar Aortic Stenosis
Supravalvar Aortic Stenosis
MEDICAL MANAGEMENT
THERAPEUTIC CARDIAC CATHETERIZATION
Fetal Interventions
SURGICAL MANAGEMENT
Valvar Aortic Stenosis
Subvalvar Aortic Stenosis
Supravalvar Aortic Stenosis
45 Coarctation of the Aorta
PREVALENCE, GENETICS AND ETIOLOGY
EMBRYOLOGY AND PATHOGENESIS
MORPHOLOGY
Associated Lesions
HEMODYNAMICS
CLINICAL FEATURES
Physical Examination
DIAGNOSTIC STUDIES
Electrocardiogram
Radiologic Features
Echocardiography
MRI and CT Angiography
Cardiac Catheterization and Angiography
NATURAL HISTORY
THERAPY AND MANAGEMENT
Surgical Repair
Balloon Angioplasty
Endovascular Stents
OUTCOMES
FUTURE DIRECTIONS
46 Hypoplastic Left Heart Syndrome
EPIDEMIOLOGY AND ETIOLOGY
THE DEVELOPING FETUS: ECHOCARDIOGRAPHY AND INTERVENTION
Fetal Echocardiography
Fetal Intervention
Maternal Hyperoxygenation
ANATOMY
Underdevelopment of the Left Ventricle Outflow-Aorta Complex
Variants of Hypoplastic Left Heart Syndrome
Additional Anatomic Considerations
The Borderline Left Ventricle
PRESENTATION, DIAGNOSIS, AND ECHOCARDIOGRAPHIC IMAGING
Presentation
Diagnosis
Echocardiography
PHYSIOLOGY, MONITORING, AND STABILIZATION
Parallel Circulation
Cardiovascular Reflexes and Physiology of Shock
Oxygen Flux in Single-Ventricle Parallel Circulation
Monitoring the Parallel Circulation
Preoperative Preparation
STAGED PALLIATION
Stage 1 Palliation
Interstage Management
Stage 2: Superior Cavopulmonary Connection
Stage 3: Completion Fontan
OUTCOMES FOR STAGED PALLIATION
Cardiac Catheterization
Late Fontan Concerns
Cardiac Transplantation
Neurodevelopmental Outcomes and Quality of Life
SUMMARY
ACKNOWLEDGMENTS
Part F: Abnormalities of the Origin of the Great Arteries
47 Transposition of the Great Arteries
INTRODUCTION
EPIDEMIOLOGY (INCIDENCE, PREVALENCE)
ETIOLOGY
Genetic
Noninherited and Environmental Risk Factors
Morphogenesis
MORPHOLOGY
Pathologic Anatomy, Variants and Conduction Anatomy
Associated Lesions
PHYSIOLOGY OF TRANSPOSITION OF THE GREAT ARTERIES
CLINICAL FEATURES AND INVESTIGATION
Presentation and Symptoms
Physical Findings
ECG (and Vector)
Radiography
Echocardiography
Magnetic Resonance Imaging and Computerized Tomography
Cardiac Catheterization/Angiography
NATURAL HISTORY AND MANAGEMENT
Natural History and Immediate Outcomes
Differential Diagnosis
Medical Management and Timing of Balloon Atrial Septostomy and Surgery
OPERATIVE AND INTERVENTIONAL CATHETERIZATION APPROACHES
Balloon Atrial Septostomy
Other Interventional Procedures
Operative Procedures
LONG-TERM OUTCOMES AND YOUNG ADULT ISSUES
48 Congenitally Corrected Transposition of the Great Arteries
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
Isolated ccTGA
ccTGA with VSD and/or PS
RIGHT VENTRICULAR DYSFUNCTION AND TRICUSPID REGURGITATION
ELECTROCARDIOGRAPHIC FEATURES
RADIOGRAPHIC FEATURES
ECHOCARDIOGRAPHY
MAGNETIC RESONANCE IMAGING
CARDIAC CATHETERIZATION
ANGIOCARDIOGRAPHY
NATURAL HISTORY AND MANAGEMENT
UNOPERATED NATURAL HISTORY
MEDICAL MANAGEMENT
PACING
PALLIATIONS, PHYSIOLOGIC SURGICAL REPAIRS, AND OUTCOMES
RIGHT VENTRICULAR DYSFUNCTION AND TRICUSPID REGURGITATION
ANATOMIC SURGICAL REPAIR AND OUTCOMES
QUALITY OF LIFE
PREGNANCY
CONCLUSION
ACKNOWLEDGMENTS
49 Double-Outlet Right Ventricle
DEFINITION
INCIDENCE
MORPHOLOGY
CLINICAL FEATURES: COMMON VARIANTS
Tetralogy Type
Transposition Type
VSD Type
Clinical Features of Less Common Variants
DORV with Noncommitted VSD
Other Less 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
Part G: Complex Cardiac Abnormalities
50 Univentricular Atrioventricular Connection
NOMENCLATURE
PATHOLOGIC ANATOMY
Ventricular Morphology
Atrioventricular Connections
Ventriculoarterial Connections
Interventricular Communications
COMMON FORMS OF UNIVENTRICULAR ATRIOVENTRICULAR CONNECTION
Double-Inlet Left Ventricle
Double-Inlet Right Ventricle
Double-Inlet Ventricle of Mixed Morphology
Double-Inlet Ventricle with Indeterminate or Undifferentiated Morphology
Absent Atrioventricular Connection
Double-Inlet Ventricle via a Common AV Valve
Clinical Features
Echocardiographic Features
Radiographic Features
Cardiac Catheterization and Angiography
Treatment
51 Cardiac Malpositions and Abnormalities of Atrial and Visceral Situs
DEFINITIONS
SEQUENTIAL SEGMENTAL APPROACH TO CARDIAC DIAGNOSIS
VISCERAL AND ATRIAL SITUS
Abdominal Organs
Cardiac Atria
The Lungs
ATRIOVENTRICULAR CONNECTIONS AND VALVE MORPHOLOGY
VENTRICULAR POSITIONS AND MORPHOLOGY
GREAT ARTERY RELATIONS
CARDIAC IMAGING: USE OF THE SEGMENTAL APPROACH
Visceral Situs
Venous Connections
Atrial Situs
Atrioventricular Connection
Cardiac Base-Apex Axis
Ventricles, Ventriculoarterial Connection, and Great Arteries
SPECTRUM OF MALFORMATIONS INVOLVING CARDIAC MALPOSITION
Dextrocardia with Atrial Situs Solitus
Dextrocardia with Situs Inversus
LEVOCARDIA (ISOLATED LEVOCARDIA)
MESOCARDIA
SITUS AMBIGUOUS
ASPLENIA
POLYSPLENIA
DOUBLE-OUTLET GREAT ARTERIAL CONNECTIONS
TREATMENT STRATEGIES
General Considerations in Cardiac Malposition
Infections and the Asplenia Syndrome
CONGENITAL PERICARDIAL DEFECTS
Manifestations
Diagnosis
Treatment
ECTOPIA CORDIS
Treatment
ACKNOWLEDGMENTS
Section VII: Diseases of the Endocardium, Myocardium, and Pericardium
52 Hypertrophic Cardiomyopathy
DEFINITION AND NOMENCLATURE
PREVALENCE
MORPHOLOGY
Left Ventricular Hypertrophy
Mitral Valve Apparatus
Coronary Artery Bridging
HISTOPATHOLOGY
PATHOPHYSIOLOGY
Genetic Pathogenesis of HCM
Left Ventricular Outflow Tract Obstruction
Myocardial Ischemia
Diastolic Dysfunction
GENETICS
CLINICAL SCREENING STRATEGIES IN FAMILIES
CLINICAL FEATURES
Physical Examination
Electrocardiogram and Ambulatory Monitoring
Echocardiography
Cardiac Magnetic Resonance Imaging
HCM AND ATHLETE'S HEART
EXERCISE AND SPORTS PARTICIPATION
PREPARTICIPATION SCREENING FOR HCM IN ATHLETIC POPULATIONS
CLINICAL COURSE
Infants
Children
RISK STRATIFICATION AND SUDDEN CARDIAC DEATH
MANAGEMENT
Medical Management
Prevention of Sudden Death
Nonpharmacologic Treatment of Left Ventricular Outflow Tract Obstruction
CONCLUSION
53 Dilated Cardiomyopathy
HISTORY
ETIOLOGIES
PATHOPHYSIOLOGY
Clinical Evaluation
Treatment
Management of Dilated Cardiomyopathy with Acute Heart Failure
Chronic Management of Dilated Cardiomyopathy
Cardiac Resynchronization Therapy and Implantable Cardioverter Defibrillators
Ventricular Assist Devices
Long-Term Outcomes
New and Novel Therapies
ACKNOWLEDGMENT
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
55 Myocarditis
INTRODUCTION
EPIDEMIOLOGY
ETIOLOGY
Causative Agents
Mechanisms of Disease-A Host Response
Autoimmunity
CLINICAL FEATURES AND INVESTIGATION
Clinical Phenotypes
Clinical Presentation and Physical Examination
Laboratory Evaluation
Electrocardiography
Radiographic Evaluation
Echocardiographic Findings
Cardiac Magnetic Resonance Imaging
ENDOMYOCARDIAL BIOPSY
TREATMENT
Activity Restrictions
Medical Management
Immunomodulators, Immunosuppression, and Antiviral Therapy
Mechanical Circulatory Support
Survival and Heart Transplantation
CONCLUSION
56 Restrictive Cardiomyopathy
EPIDEMIOLOGY
GENETICS
Sarcomeric Protein Gene Defects
Nonsarcomeric Protein Gene Defects
ETIOLOGIES
Endomyocardial Disease
Hypereosinophilic Syndrome/Löffler Endocarditis
Infiltrative and Storage Diseases
Drugs and Therapeutic Agents
Idiopathic
Pathology
PATHOPHYSIOLOGY
CLINICAL PRESENTATION
PHYSICAL EXAMINATION
DIAGNOSTIC STUDIES
Electrocardiograms
Holter
Chest Radiographs
Echocardiography
Cardiac Catheterization and Endomyocardial Biopsy
DIFFERENTIAL DIAGNOSIS
OUTCOME
MANAGEMENT
57 The Heart in Muscular Dystrophies
MUSCULAR DYSTROPHIES WITH CLINICAL CARDIAC DISEASE
ASSESSMENT TOOLS
Electrocardiography
Holter Monitoring
Echocardiography
Other Imaging Methods
MUSCULAR DYSTROPHIES WITH CARDIAC INVOLVEMENT
Dystrophinopathies
Dystrophinopathies: Duchenne Muscular Dystrophy
Dystrophinopathies: Becker Muscular Dystrophy
Dystrophinopathies: X-Linked Cardiomyopathy
MYOTONIC MUSCULAR DYSTROPHY
EMERY-DREIFUSS MUSCULAR DYSTROPHY
FACIOSCAPULOHUMERAL MUSCULAR DYSTROPHY
LIMB-GIRDLE MUSCULAR DYSTROPHIES
FRIEDREICH ATAXIA
58 Kawasaki Disease (Mucocutaneous Lymph Node Syndrome)
EPIDEMIOLOGIC FEATURES
ETIOLOGY AND PATHOGENESIS
PATHOLOGY
MANIFESTATIONS
Clinical Features: Systemic
KAWASAKI SHOCK SYNDROME
RECURRENCE AND RECRUDESCENCE
DIAGNOSIS AND DIFFERENTIAL DIAGNOSIS
ALGORITHM FOR CLINICAL EVALUATION AND TREATMENT OF CHILDREN WITH SUSPECTED KAWASAKI DISEASE
Clinical Features: Cardiovascular
Electrocardiographic Features
Radiologic Features
Echocardiographic Features
Stress Testing
Cardiac Catheterization and Angiography
Magnetic Resonance Imaging, Magnetic Resonance Angiography, and Computed Tomography
Long-Term Cardiovascular Effects
TREATMENT: ACUTE PHASE
Intravenous Immunoglobulin
Aspirin and Other Antithrombotic Therapies
Adjunctive Therapies for Primary Treatment
Additional Therapies for Patients with IVIG Resistance (Rescue Therapy)
Antithrombotic Therapy in the Acute Phase
TREATMENT: CHRONIC PHASE
Coronary Risk Stratification
Anticoagulation for Giant Aneurysms
Management of Myocardial Infarction and Nonobstructive Coronary Thrombosis
Surgical and Transcatheter Revascularization
Exercise Recommendations
SUMMARY
59 Rheumatic Fever and Rheumatic Heart Disease
EPIDEMIOLOGY
Scope of the Problem
Environment
Host
Streptococcal Infection
PATHOGENESIS
Organism
Susceptible Host
Immunopathogenesis
Natural History
PATHOLOGY
PERICARDITIS
Myocarditis
Endocarditis
Vasculitis
CLINICAL MANIFESTATIONS
Diagnosis and Evaluation
Arthritis and Arthralgia
Chorea
Carditis
Erythema Marginatum
Subcutaneous Nodules
Minor Criteria and Other Clinical Manifestations
Evidence of a Preceding Group A Streptococcal Infection
Cardiac Catheterization
Other Cardiac Imaging
CHRONIC RHEUMATIC HEART DISEASE
Mitral Regurgitation
Aortic Regurgitation
Mitral Stenosis
Aortic Stenosis
Right Heart Involvement
TREATMENT
Medical Management of Acute RF
Medical Management of Acute Rheumatic Carditis and Heart Failure
Medical Management of Chronic RHD
Heart Failure in Chronic RHD
Pregnancy and Rheumatic Heart Disease
SURGICAL AND CATHETER-BASED TREATMENT
Acute Carditis
Chronic RHD
PREVENTION AND PROPHYLAXIS
Primary Prophylaxis
Secondary Prophylaxis
Screening for Rheumatic Heart Disease
Primordial Prevention
Endocarditis Prophylaxis
SUMMARY
60 Inflammatory and Immune-Mediated Noninfectious Cardiovascular Diseases
INTRODUCTION
JUVENILE IDIOPATHIC ARTHRITIS
JIA Epidemiology
JIA Etiology/Pathophysiology/Diagnosis
General Treatment Strategies for JIA
Cardiac Involvement in JIA
SYSTEMIC LUPUS ERYTHEMATOSUS
SLE Epidemiology
SLE Etiology
SLE Clinical Features and Investigation
SLE Cardiac Involvement
TAKAYASU ARTERITIS
TA Diagnosis
TA Epidemiology
TA Etiology/Pathology
TA Clinical Features
TA Cardiac Involvement
Presentation
Physical Findings
Laboratory Testing
TA Differential Diagnosis
TA Management
Monitoring Disease Activity Over Time
TA Outcomes
JUVENILE IDIOPATHIC INFLAMMATORY MYOPATHIES
JIIM Diagnosis/Pathophysiology
JIIM Treatment
JIIM Cardiac Involvement
BEHçET DISEASE
Etiology/Pathology
BD Cardiac Involvement
SCLERODERMA
Scleroderma Epidemiology
Scleroderma Etiology/Pathology
Scleroderma Clinical Features and Diagnosis
Differential Diagnosis
Cardiac Involvement in Scleroderma
SARCOIDOSIS
Epidemiology
Sarcoid Clinical Features
Sarcoid Cardiac Involvement
Treatment
POLYARTERITIS NODOSA
PAN Epidemiology
PAN Etiology/Pathophysiology
PAN Diagnosis/Clinical Presentation
PAN Cardiac Involvement
PAN Treatment/Outcome
61 Pericardial Diseases
ANATOMY AND PHYSIOLOGY
ACUTE PERICARDITIS
Symptoms
Physical Examination
Cardiac Tamponade
Chest Radiography
Electrocardiography
Echocardiography
Cardiac Catheterization
Other Imaging Modalities
Management of Tamponade
ETIOLOGY
Viral Pericarditis
Bacterial Pericarditis
Tuberculous Pericarditis
HIV and Other Infections
Other Causes of Pericarditis and Pericardial Effusion
Congenital Abnormalities of the Pericardium
CONSTRICTIVE PERICARDITIS
Differentiating Constrictive Pericarditis from Restrictive Cardiomyopathy
Special Circumstances
62 Infective Endocarditis and Prevention
DEFINITION
EPIDEMIOLOGY
MICROORGANISMS
PATHOGENESIS
CLINICAL FEATURES
LABORATORY DIAGNOSIS
ECHOCARDIOGRAPHY
DIAGNOSTIC CRITERIA
ANTIMICROBIAL THERAPY
General Principles
Streptococcal Endocarditis
Staphylococcal Endocarditis
Gram-Negative Bacterial Endocarditis
Fungal Endocarditis
Culture-Negative Endocarditis
Prosthetic Valve Endocarditis
PREVENTION
Prophylaxis
ACKNOWLEDGMENT
63 Heart Transplantation
BEFORE CARDIAC TRANSPLANT
Indications
Transplant Candidacy and the Pretransplant Evaluation
Pretransplant Management
Considerations for Multiorgan Transplantation
Transplant Listing
Risk Factors for Death While Waiting for Transplant
PERITRANSPLANT
Donor Evaluation
Donation
Perioperative Planning and Treatment
POSTTRANSPLANT IMMUNOSUPPRESSION
General Considerations
Oversuppression
Immunosuppressive Medications
COMPLICATIONS
Rejection
Transplant Coronary Artery Disease and Coronary Angiography
Infections
Posttransplant Lymphoproliferative Disease (PTLD)
Chronic Kidney Disease
Diabetes
Hypertension
Hyperlipidemia
Other Immunologic Complications
MONITORING AFTER TRANSPLANT
Cardiac Status
Routine Posttransplant Surveillance
CARDIAC CATHETERIZATION AND ENDOMYOCARDIAL BIOPSY
Stress Testing
Immune Status
Cylex® Level
AlloMap®
Cell-free DNA
Donor Specific Antibodies
Multiorgan Surveillance
Infectious Surveillance and Prophylaxis
HEALTH CARE MAINTENANCE
Rehabilitation/Physical Activity
Pediatric Challenges
Immunizations
Quality of Life/Neurodevelopment
RETRANSPLANT
FUTURE DIRECTIONS
64 Pathophysiology of Pulmonary Hypertension
INTRODUCTION
HEART DEFECTS WITH INCREASED PULMONARY BLOOD FLOW
High-Flow Congenital Heart Defects and Pulmonary Hypertension
Structural Remodeling and Growth
Lung Biopsy
Wedge Angiography
Reactive Pulmonary Circulation
Pathophysiology Based upon Further Pathologic Assessments
Experimental Studies Simulating High Flow and Pressure
HEART DEFECTS WITH INCREASED PULMONARY VENOUS PRESSURE
Vascular Changes
Experimental Studies
HEART DEFECTS WITH DECREASED PULMONARY BLOOD FLOW
Structural Features
Experimental Studies
HYPOXIA-INDUCED PULMONARY VASCULAR DISEASE
Hypoxia and Pulmonary Vasoconstriction
Experimental Studies of Acute Hypoxia
CHRONIC HYPOXIA
Clinical Features of Chronic Hypoxia
Experimental Studies
LUNG DISEASE
Obstructive Lung Disease
UPPER AIRWAY OBSTRUCTION
DISORDERS OF THE CHEST WALL
PERSISTENT PULMONARY HYPERTENSION OF THE NEWBORN
Development of the Normal Perinatal Pulmonary Vasculature
Underdevelopment of the Lung
Maladaptation of the Pulmonary Vascular Bed
Maldevelopment of the Pulmonary Vascular Bed
THROMBOEMBOLIC DISEASES
SICKLE CELL DISEASE AND OTHER HEMAGLOBINOPATHIES
PORTAL HYPERTENSION
GRANULOMATOUS AND COLLAGEN VASCULAR DISEASES AND IMMUNE/INFECTIOUS DISEASES
DRUGS AND TOXINS
Clinical Studies
Experimental Studies
IDIOPATHIC AND HERITABLE PULMONARY ARTERIAL HYPERTENSION
SUMMARY
FUTURE DIRECTIONS
Section VIII: Pulmonary Vascular Disease
65 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
Idiopathic Pulmonary Arterial Hypertension
Hepatopulmonary Syndrome and Portopulmonary Hypertension
Human Immunodeficiency Virus (HIV)
Hemoglobinopathies
Pulmonary Capillary Hemangiomatosis and Pulmonary Veno-Occlusive Disease
Left Heart Disease
Respiratory Disease
Down Syndrome
Thromboembolic Disease
PHARMACOLOGIC THERAPY OF PAH
Conventional Therapy
Anticoagulation
Vasoreactivity Testing
Calcium Channel Blockers
Prostacyclins
Endothelin
Phosphodiesterase-5 Inhibitors
Soluble Guanylate Cyclase Stimulators
COMBINATION THERAPY
ATRIAL SEPTOSTOMY
POTTS SHUNT
TRANSPLANTATION
NOVEL THERAPIES
CONCLUSION
66 The Adolescent and Adult with Congenital Heart Disease
Delivery of ACHD Care
Barriers to ACHD Care and Transition
Training in Adult Congenital Heart Disease
Outcomes
SHUNT LESIONS
Atrial Septal Defects
Ventricular Septal Defects
Atrioventricular Septal Defects
Patent Ductus Arteriosus
LEFT-SIDED LESIONS
Bicuspid Aortic Valve
Management of BAV Disease
Management of BAV Aortopathy
Subaortic Stenosis
Coarctation of the Aorta
RIGHT-SIDED LESIONS
Pulmonary Valve Stenosis
Tetralogy of Fallot
Ebstein Anomaly of the Tricuspid Valve
D-Transposition of the Great Arteries
Arterial Switch for D-TGA
Congenitally Corrected Transposition of the Great Arteries
FUNCTIONAL SINGLE-VENTRICLE PHYSIOLOGY AND THE FONTAN
PULMONARY HYPERTENSION AND EISENMENGER SYNDROME
Pulmonary Hypertension in Left-to-Right Shunts
Pulmonary Hypertension in D-TGA
Eisenmenger Syndrome
CHRONIC CYANOSIS IN ACHD
Section IX: Unique Issues Regarding the Young Adult with Congenital Heart Disease
67 Transition to Adult Care
WHAT IS TRANSITION AND TRANSFER?
WHY TRANSITION?
Recommendations to Transition
Volume of Pediatric and Adult Cardiology Providers
Adult-Centered Healthcare Benefits
TIMING OF TRANSITION
COMPONENTS OF TRANSITION
Formal Transition
Continuous Care
Patient Autonomy
Patient Education
Organization of Care
Psychological Issues
Advance Directives and End-of-Life Planning
Noncardiac Medical Care
Reproductive Health
Employment, Insurance, Disability, and Government Aid
TRANSITION TIMELINE
PERSONNEL REQUIRED FOR TRANSITION
"Giving" Providers
"Receiving" Providers
Support Personnel
BARRIERS TO TRANSITION
Problem: Lost to Follow-up
Problem: Communication Issues
Problem: Healthcare Providers
Problem: Loss of Insurance
Problem: Geographic Complications
IMPLEMENTING A TRANSITION PROGRAM
SUMMARY
68 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
Cardiac Shunts
Right Ventricular Outflow Tract Obstruction
Tetralogy of Fallot
Left Ventricular Outflow Tract Obstruction
Coarctation of the Aorta
Aortopathy
Ebstein Anomaly
Transposition of the Great Arteries
Fontan Circulation
Cyanotic Heart Disease
Prosthetic Heart Valves
TRANSMISSION OF CARDIAC DISEASE TO OFFSPRING
MANAGEMENT ISSUES DURING PREGNANCY
Preconception Issues
Antepartum Issues
Management of Heart Failure
Management of Arrhythmias
Management of Anticoagulation
High-Risk Conditions
Cardiac Surgery during Pregnancy
MANAGEMENT ISSUES DURING LABOR AND DELIVERY
CONTRACEPTION
69 Chest Pain in Children and Adolescents
IDIOPATHIC CHEST WALL PAIN
MUSCULOSKELETAL/CHEST WALL PAIN
Costochondritis
Trauma and Muscle Strain
Precordial Catch Syndrome
Slipping Rib Syndrome
Tietze Syndrome
Hypersensitive Xiphoid Syndrome
OTHER NONCARDIAC CAUSES OF CHEST PAIN
Pericarditis
Pneumothorax/Pneumomediastinum
Pulmonary
Gastrointestinal
Psychogenic
Miscellaneous
CARDIAC CAUSES OF CHEST PAIN
MEDICAL EVALUATION
TREATMENT
OUTCOME
ACKNOWLEDGMENT
Section X: Other Special Problems and Issues
70 Coronary Risk Factors in Children
ATHEROSCLEROSIS
Imaging
Risk Factors for Atherosclerosis
Summary
LIPIDS AND LIPOPROTEINS
Normal Levels of Lipids and Lipoproteins
Epidemiology
Factors Causing Dyslipidemia
Clinical Recommendations
Individual Approach
Treatment
Summary
HYPERTENSION
Epidemiology
Target Organ Effects
Normal Blood Pressure
Definition of Hypertension
Measurement of Blood Pressure
Etiology of Hypertension
Evaluation of Target Organ Abnormalities
Treatment of Hypertension
Diet
Physical Activity
Treatment of Severe Hypertension
Summary
71 Cardiac Tumors
HISTORY OF CARDIAC TUMORS
INCIDENCE
DIAGNOSTIC PROCEDURES
Electrocardiogram and Chest Roentgenogram
Echocardiography
CT Scan
Magnetic Resonance Imaging
Angiography
3D Print Modeling
FDG PET/CT
RHABDOMYOMAS
FIBROMAS
MYXOMAS
INTRAPERICARDIAL TERATOMAS
OTHER PRIMARY BENIGN MYOCARDIAL TUMORS
PRIMARY MALIGNANT MYOCARDIAL TUMORS
SECONDARY CARDIAC TUMORS
72 Chronic Heart Failure in Children
IMPACT OF DISEASE
CLASSIFICATION
ETIOLOGY
NEONATES AND INFANTS
CHILDHOOD AND ADOLESCENCE
Structurally Abnormal Hearts
Preoperative Structural Heart Disease
Postoperative Structural Heart Disease
Structurally Normal Hearts with Acquired Heart Disease
Primary Cardiomyopathies
BIOMARKERS IN CHRONIC HEART FAILURE
MANAGEMENT OF CHRONIC HEART FAILURE
Diuretics
Angiotensin-Converting Enzyme Inhibitors and Angiotensin-Receptor Blockers
Beta-Blockers
Mineralocorticoid Receptor Antagonists
Angiotensin Receptor-Neprilysin Inhibitors
Digoxin
Inotropic Therapy for Chronic Heart Failure
Novel Therapies in Chronic Heart Failure
Antiarrhythmic Medications in Heart Failure
Dietary Supplements and Alternative Therapies for Heart Failure
Device Therapy for Heart Failure
OUTCOMES IN PEDIATRIC HEART FAILURE
SUMMARY OF GUIDELINES AND CONSENSUS STATEMENTS
73 Neurodevelopmental Outcomes after Heart Surgery in Children
GENETIC ABNORMALITIES
BRAIN ANATOMY, PATHOLOGY, AND NEUROIMAGING
PERIOPERATIVE RISK FACTORS
Perioperative Monitoring Approaches
Intraoperative Support Techniques
Patient Preoperative Factors and Host Susceptibility
Postoperative Factors
Relative Impact of Patient/Preoperative, Operative, and Postoperative Factors on Neurodevelopmental Outcome after Cardiac Surgery
OTHER CAUSES OF NEUROLOGIC MORBIDITY
LONG-TERM NEURODEVELOPMENTAL OUTCOME
Outcomes in Diagnostic Groups
Psychosocial Function and Academic Ability and Achievement
Mental Health
CONCLUSION
74 Hematologic Aspects of Pediatric and Adolescent Heart Disease: Bleeding, Clotting, and Blood Component Abnormalities
BASIC PRINCIPLES OF HEMATOLOGY
Red Blood Cells
White Blood Cells
Hemostasis
HEMATOLOGIC DISORDERS
Special Consideration of Hematologic Disorders in Congenital and Acquired Heart Disease
Disorders of Red Blood Cells
Disorders of White Blood Cells
Disorders of Hemostasis Associated with Bleeding
CPB, ECMO, AND VENTRICULAR ASSIST DEVICES
Postoperative Bleeding after CPB
Management of Postoperative Bleeding
ECMO and Ventricular Assist Devices
THROMBOSIS
Thrombosis in Children and Adolescents with Congenital and Acquired Heart Disease
The Propensity for Thrombosis in Children and Adolescents with Congenital and Acquired Heart Disease
Consequence of Significant Thrombi in Children and Adolescents with Cyanotic Heart Disease
Common Thrombotic Complications in Congenital Heart Disease: Prevention, Diagnosis, and Treatment
Direct Oral Anticoagulants
Prosthetic Valves
Arrhythmias
Acquired Heart Disease
Bridging Therapy
Thrombolytic Therapy in Children and Adolescents with Heart Disease
Future Directions
75 The Fontan Circulation
EPIDEMIOLOGY OF SINGLE-VENTRICLE HEART DISEASE
HISTORY OF THE FONTAN PALLIATION
PREOPERATIVE EVALUATION
OPERATIVE TECHNIQUES
SURVIVAL OUTCOMES FOR THE FONTAN OPERATION
LIVING WITH A FONTAN CIRCULATION: CHALLENGES AND COMPLICATIONS
Ventricular Dysfunction
Atrioventricular Valve Regurgitation
Arrhythmia
Abnormalities of the Lymphatic Circulation
Liver Disease
Renal Disease
Neurologic, Psychological, and Behavioral Outcomes
STRATEGIES FOR MANAGEMENT AND TREATMENT
Surveillance Strategies
Pharmacologic Drug Therapies
Exercise
The Failing Fontan Circulation
Durable Mechanical Circulatory Support
Heart Transplantation
FUTURE DIRECTIONS
76 Evaluation of Quality of Life in Children and Adolescents with Congenital and Acquired Heart Disease
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?
HRQOL Studies in the General CHD Population
HRQOL Studies in Specific HD Subgroups
Predictors of HRQOL in the Pediatric HD Population
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
77 Psychological Impact of Congenital Heart Disease: Key Timestamps and Clinical Strategies
PSYCHOSOCIAL IMPACT OF CHD: AN OVERVIEW
Parents and Caregivers
Children and Adolescents with CHD
Adults with CHD
TIMESTAMPS: PSYCHOSOCIAL CHALLENGES AND OPPORTUNITIES FOR INTERVENTION
Timestamp 1: Initial Diagnosis
Timestamp 2: Initial CHD Surgery
Timestamp 3: Beginning School
Timestamp 4: Sports Participation
Timestamp 5: Major Medical Events in Older Childhood and Adolescence
Timestamp 6: Transfer from Pediatric to Adult Care
Timestamp 7: Declining Health in Adulthood
CONCLUSIONS
78 Safety and Quality in the Heart Center
CASE STUDY
ROOT CAUSE ANALYSIS: WHAT HAPPENED, WHY, AND NEXT STEPS
When Individuals Fail
THE IMPROVEMENT TEAM-AIM, KEY DRIVERS, AND INTERVENTIONS
Specific Aim and Key Driver Diagram
Teams
Bundles and Checklists
DATA SUPPORTS CHANGE--INFORMATION DRIVES CHANGE-DON'T LET DATA PARALYZE THE TEAM
HIGH RELIABILITY-DO WHAT YOU SAY AND DO IT CONSISTENTLY
Systems Thinking
Human Factors That Contribute to Error
Creating High Reliability Organizations
SAFETY II-A DIFFERENT PATH FORWARD IN ERROR PREVENTION
How Might Safety II Behavior Have Avoided the Error/Harm in AJ's Case?
PATIENT- AND FAMILY-CENTERED CARE
CULTURE-THE SECRET SAUCE
THE "SECOND VICTIM" PHENOMENON
CONCLUSION
79 Global Challenge of Heart Disease
GLOBAL BURDEN OF CARDIOVASCULAR DISEASE
THE CONCEPT OF THE EPIDEMIOLOGIC TRANSITION
THE IMPENDING GLOBAL EPIDEMIC OF ISCHEMIC CARDIOVASCULAR DISEASE
Ischemic Cardiovascular Disease, Not Just a Disease of the Affluent and the Elderly
Risk Factors for Cardiovascular Disease in Low- and Middle-Income Countries
Managing the Impending Global Epidemic: A Focus on Children
RHEUMATIC HEART DISEASE
Epidemiology of Rheumatic Heart Disease
The Role of Echocardiography in Diagnosis and Screening
Prevention and Treatment
CONGENITAL HEART DISEASE
The Impact of the Health Transition
Addressing the Global Challenge of CHD
ARRHYTHMIA
CHAGAS DISEASE
SUMMARY
ACKNOWLEDGMENT
80 Design, Execution, and Appraisal of Clinical Trials
WHAT IS EVIDENCE-BASED MEDICINE AND WHY ARE CLINICAL TRIALS SO RELEVANT TO THAT PRACTICE?
Definition and Practice of Evidence-Based Medicine
Both Absence and Proliferation of Evidence
Evidence and Clinical Trials
HOW DO CLINICAL TRIALS ACHIEVE A HIGH GRADE OF EVIDENCE?
Causality
Randomization
Prospective and Concurrent Data Collection
Appraisal and Regulation
WHY ARE THERE NOT MORE CLINICAL TRIALS?
Resource Requirements and Conflicts of Interest
Equipoise and Execution
Feasibility
Failure of Assumptions
Publication Bias
Specialty-Specific Challenges
DESIGN ISSUES FOR CLINICAL TRIALS
Phases of Clinical Trials
Questions, Hypotheses, and Aims
Interventions and Clinical Trial Designs
Study Participants
Study Measurements
Randomization
Cointerventions and Blinding
Sample Size and Power
Execution Issues for Clinical Trials
Operationalization
Recruitment
Retention
Crossover and Compliance
Quality Control
Data and Safety Monitoring
ANALYSIS ISSUES FOR CLINICAL TRIALS
Data Management, Cleaning, and Descriptive Statistics
Test of Randomization
Outcomes' Comparisons
Reporting and Appraisal Issues for Clinical Trials
ACKNOWLEDGMENT
RECOMMENDED RESOURCES
81 Pharmacology
COMMON DEFINITIONS OF TERMS IN CLINICAL PHARMACOLOGY
THE EXPOSURE-RESPONSE RELATIONSHIP
DEVELOPMENTAL PHARMACOKINETICS
Drug Absorption
Drug Distribution
Drug Metabolism
Renal Drug Elimination
DEVELOPMENTAL PHARMACODYNAMICS
DEVELOPMENTAL PHARMACOGENOMICS
CARDIOVASCULAR DRUGS
CARDIAC GLYCOSIDES
Digoxin
ADRENERGIC AGONISTS
Dopamine
Fenoldopam
Dobutamine
Epinephrine
Norepinephrine
Isoproterenol
Phenylephrine
PHOSPHODIESTERASE INHIBITORS
Milrinone
CALCIUM SENSITIZING AGENTS
DIURETICS
Loop Diuretics
Furosemide
Ethacrynic Acid
Bumetanide
Thiazide Diuretics
Metolazone
Potassium Sparing Diuretics
VASODILATORS
Nitric Oxide Modifying Drugs
Nitroglycerin
Nitroprusside
Pulmonary Vasodilators
a-Adrenergic Receptor Antagonists
Calcium Channel Antagonists
Angiotensin-Converting Enzyme Inhibitors
Angiotensin Receptor Blockers
ALDOSTERONE RECEPTOR ANTAGONISTS
HORMONES
Nesiritide
Thyroxine and Triiodothyronine
Vasopressin
ß-ADRENERGIC RECEPTOR BLOCKERS
Propranolol
Esmolol
Metoprolol and Atenolol
Carvedilol
Prostaglandin E1
ANTIARRHYTHMIC DRUGS
Class I Agents
Class II (Propranolol, Atenolol, Esmolol)
Class III (Amiodarone, Sotalol)
Class IV (Calcium Channel Blockers)
Digoxin
Ivabradine
Adenosine
Dexmedetomidine
CONCLUSIONS
Index

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