Neonatal Heart Disease

1 General Considerations.- 1 Etiology of Congenital Heart Disease.- Pathogenesis of Congenital Cardiac Malformations.- Ectomesenchymal Tissue Migration Abnormalities.- Defects Associated with Abnormalities of Intracardiac Blood Flow.- Genetic and Environmental Factors.- Genetic and Environmental Interaction — the Polygenic and Multifactorial Threshold Concept.- 2 Cardiovascular Malformations: Prevalence at Livebirth.- Public Health Approaches to Disease.- Estimation of CVM Prevalence at Livebirth.- The Baltimore-Washington Infant Study.- Summary.- 3 Trends in Neonatal Congenital Heart Disease: The Toronto Experience.- Congenital Cardiovascular Malformations.- Non-structural Heart Disease.- 4 Developmental Biology of Mammalian Myocardium.- Substrate Utilization.- Biochemistry.- Myocardial Cellular Differentiation.- Role of Oxygen Reactive Species and Antioxidant Systems.- Tolerance of Adverse Metabolic Conditions.- Conclusions.- 5 Developmental Biology of the Pulmonary Vascular Bed.- Normal Pulmonary Vascular Development.- Consequences of Hypoxic Vasoconstriction in the Fetus and Newborn.- Postnatal Pulmonary Vascular Abnormalities.- Cell Culture Systems.- Summary Hypothesis Related to the Pathogenesis of Pulmonary Hypertension.- 6 Developmental Biology of Specialized Conduction Tissue.- Embryology.- Anatomy.- Action Potential and Ionic Currents.- Developmental Electrophysiology.- Developmental Changes in Ionic Channels.- Antiarrhythmic Agents and the Developing Heart.- Conclusions.- 7 Physical Examination of the Cardiovascular System of the Neonate.- Condition at Delivery.- Signs Signalling Involvement of the Cardiovascular System.- Auscultation.- Normal Cardiac Examination.- Abnormal Cardiac Examination.- 8 The Electrocardiogram of the Neonate.- The Premature Infant.- Enlargement and Hypertrophy Patterns.- General Comments about Specific Cardiac Conditions.- 9 Imaging of the Neonate with Congenital Heart Disease.- The Chest Radiograph.- Angiography.- Special Techniques.- 10 The Segmental Approach to Congenital Heart Disease.- The Cardiac Segments.- Types of Atrioventricular Connection.- Ventriculoarterial Connection.- Infundibular Anatomy.- 11 Echocardiography and the Neonate with Real or Suspected Heart Disease.- Advances in Equipment.- Role of Echocardiography in the Newborn.- Pitfalls in Echocardiography Imaging — to Catheterize or Not?.- Postoperative Echocardiography.- 12 The Transitional Circulation.- The Fetal Circulation.- Anomalies of the Heart and Circulation.- The Normal Transitional Circulation.- Clinical Findings.- Abnormalities of the Transitional Circulation..- Clinical Findings.- 13 The Clinical Diagnostic Approach in Congenital Heart Disease.- Classification of Congenital Heart Disease.- Congestive Heart Failure.- Differential Diagnosis of Heart Failure.- Cyanotic Lesions.- Clinical Classification.- 2 Specific Conditions.- 14 Transposition of the Great Arteries.- Definition.- Morphogenesis and Morphology.- Commonly Associated Malformations.- The Coronary Arteries.- Clinical Features.- Medical Management.- Surgical Considerations.- 15 Tetralogy of Fallot.- Morphology.- Clinical Findings.- Medical Therapy.- Surgical Therapy.- 16 Pulmonary Atresia and Ventricular Septal Defect.- Morphogenesis.- Clinical Presentation.- Medical Therapy.- Surgical Therapy.- 17 Tetralogy of Fallot with “Absent” Pulmonary Valve.- Etiology and Prevalence.- Morphology and Morphogenesis.- Clinical Features.- Differential Diagnosis.- Indications for Surgery in the Neonate and Outcome.- 18 Tricuspid Atresia.- Prevalence.- Morphology.- Clinical Findings.- Medical Therapy.- Palliative Surgery.- 19 Pulmonary Atresia and Intact Ventricular Septum.- Incidence.- Morphogenesis.- Morphology.- Clinical Presentation.- Medical Management.- Surgical Management.- 20 Anomalies of Pulmonary Venous Connections Including Cor Triatriatum and Stenosis of Individual Pulmonary Veins.- Incidence.- Morphology and Morphogenesis.- Embryology.- Physiology.- Clinical Features.- Medical Therapy.- Surgery and Outcome.- Cor Triatriatum.- Individual Stenosis of Pulmonary Veins.- 21 Hypoplastic Left Heart Syndrome.- Prevalence and Etiology.- Morphology and Morphogenesis.- Typical Anatomical Features.- Clinical Features.- Differential Diagnosis.- Rationale for Staged Surgical Intervention.- 22 Aortic Stenosis.- Incidence and Etiology.- Morphology and Morphogenesis.- Clinical Features.- Differential Diagnosis.- Medical Treatment.- Surgical Treatment.- Supravalvar Aortic Stenosis.- Subvalvular Aortic Stenosis.- 23 Coarctation of the Aorta.- Prevalence and Etiology.- Morphology and Morphogenesis.- Pathophysiology.- Clinical Features.- Differential Diagnosis.- Medical Treatment.- Surgery and Outcome.- 24 Interruption of the Aortic Arch.- Incidence and Etiology..- Morphological Considerations.- Clinical Features.- Medical Therapy.- Surgical Therapy.- 25 Anomalies of the Coronary Arteries.- Anomalies of the Number and Position of Coronary Arterial Ostia from the Aorta.- Anomalous Origin of the Coronary Arteries from the Main Pulmonary Artery.- Coronary Arterial Fistulae.- Coronary Arterial Obstructive Lesions.- Congenital Coronary Artery Aneurysms.- The Role of Echocardiography.- 26 Anomalies of Aortopulmonary Septation: Persistent Truncus Arteriosus, Aortopulmonary Septal Defect, and Hemitruncus Arteriosus.- Persistent Truncus Arteriosus.- Incidence.- Morphology and Morphogenesis.- Clinical Features.- Medical Therapy.- Surgical Therapy.- Aortopulmonary Septal Defect.- Prevalence.- Morphology and Morphogenesis.- Clinical Features.- Surgical Intervention.- Hemitruncus Arteriosus.- Morphology and Morphogenesis.- Clinical Features.- Therapy.- 27 Double-Outlet Right Ventricle.- Definition.- Types of Double-Outlet Right Ventricle.- Clinical Findings.- Medical Treatment.- Surgical Treatment Modalities.- 28 Ebstein’s Malformation of the Tricuspid Valve.- Anatomy of the Tricuspid Valve.- Clinical Features.- Medical Therapy.- Surgical Therapy.- 29 Anomalies of Systemic Venous Connections, Persistence of the Right Venous Valve and Silent Cardiovascular Causes of Cyanosis.- Anomalies of Systemic Venous Connections.- Anomalies of the Right Superior Vena Cava.- Anomalies of the Left Superior Vena Cava.- Anomalies of the Coronary Sinus.- Anomalies of the Inferior Vena Cava.- Persistent Right Venous Valve.- Silent Cardiovascular Causes of Cyanosis and Hypoxemia.- 30 Hearts with a Univentricular Atrioventricular Connection.- Definition.- Types of Univentricular Atrioventricular Connection.- The Ventricular Septal Defect.- Physical Examination and Clinical Findings.- Medical Therapy.- Palliative Surgery.- 31 Congenitally Corrected Transposition of the Great Arteries.- Prevalence and Etiology.- Morphology.- Clinical Features.- Medical Management.- Surgical Management.- 32 Syndromes of Right or Left Atrial Isomerism.- The Concept of Atrial and Pulmonary Symmetry.- Types of Heart Malformations Identified in Patients with Right or Left Atrial.- Isomerism.- Prevalence.- Clinical Suspicion of Asplenia or Polysplenia Syndromes.- Medical Management.- Surgical Management.- 33 Double-Outlet Left Ventricle; Isolated Atrioventricular Discordance; Anatomically Corrected Malposition of the Great Arteries; and Syndrome of Juxtaposition of the Atrial Appendages.- Double-Outlet Left Ventricle.- Isolated Atrioventricular Discordance (Isolated Ventricular Inversion).- Anatomically Corrected Malposition of the Great Arteries.- Syndrome of Juxtaposition of the Atrial Appendages.- 34 Ventricular Septal Defect.- Prevalence.- Etiology.- Pathology.- Clinical Findings.- Medical Management.- Surgical Management.- 35 Patent Ductus Arteriosus.- Incidence.- Morphology and Morphogenesis.- Clinical Features.- Investigations.- Medical and Surgical Therapy and Outcome.- 36 Atrioventricular Septal Defect.- Prevalence.- Morphology.- Anatomic Risk Factors.- Clinical Findings.- Medical Therapy.- Surgery.- 37 Atrial Septal Defect.- Incidence.- Etiology.- Morphology and Morphogenesis.- Clinical Features.- Investigations.- 38 Pulmonary Valve Stenosis, Pulmonary Arterial Stenosis, and Isolated Right Ventricular Hypoplasia.- Pulmonary Valve Stenosis: Incidence and Etiology.- Morphology and Morphogenesis.- Clinical Features.- Investigations.- Pulmonary Arterial Stenosis.- Pathology.- Clinical Features.- Isolated Right Ventricular Hypoplasia.- 39 Supero-Inferior Ventricles, Criss-Cross Atrioventricular Connections and the Straddling Atrioventricular Valve.- Supero-Inferior Ventricles.- Criss-Cross Heart.- Straddling Atrioventricular Valves.- 40 Congenital Valvular Regurgitation.- Aortic Regurgitation.- Congenital Pulmonary Regurgitation.- Congenital Tricuspid Regurgitation.- Congenital Mitral Regurgitation.- 41 Myocardial Disorders.- Incidence.- Pathophysiologic Classification of Cardiomyopathies.- Clinical Features: General Considerations.- Metabolic Cardiomyopathies.- Glucose Metabolism.- Glycogen Synthesis.- Protein Metabolism.- Mitochondrial Disorders.- Diagnostic Evaluation of an Infant with Suspected Metabolic Cardiomyopathy.- Specific Cardiomyopathies.- 42 Cardiac Neoplasms.- Incidence.- Types and Sites of Cardiac Tumors.- Diagnosis in the Neonate.- 43 Rings, Slings, and Other Things: Vascular Structures Contributing to a Neonatal “Noose”.- Embryology of the Aortic Arch System.- The Most Common Vascular Rings or Slings Presenting in the Neonatal Period.- 44 Cardiac Malpositions Including Ectopia Cordis and Congenital Pericardial Defect.- Dextrocardia.- Isolated Levocardia.- Ectopia Cordis.- Congenital Pericardial Defect.- 45 Arteriovenous Fistulae: a Consideration of Extracardiac Causes of Congestive Heart Failure.- Incidence.- Etiology.- Morphology.- Physiology.- Clinical Features.- Investigations.- Surgery and Outcome.- Embolization Therapy.- 46 Conjoined Thoracopagus Twins.- The Basic Malformation.- Diagnosis of Cardiac Conjunction and Site of Cardiac Fusion.- 47 Disorders of Heart Rate and Rhythm.- Incidence and Etiology.- Physiology and Pathophysiology.- Mechanisms of Arrhythmia.- Classification.- Atrioventricular Block.- Supraventricular Dysrhythmias.- Ventricular Dysrhythmias.- Fetal Arrhythmias.- Infant Apnea Syndrome: Cardiac Implications.- 48 Fetal Echocardiography.- Indications for Fetal Cardiac Evaluation.- Methodology.- Epidemiological Studies.- Cardiac Anomalies Detected in Utero: Natural History.- 49 Interventional Cardiac Catheterization in Neonates.- Balloon Atrial Septostomy.- Blade Atrial Septostomy.- Valvular Pulmonary Stenosis.- Valvular Aortic Stenosis.- Coarctation of the Aorta.- Pulmonary Vein Stenosis.- Procedural Complications.- 50 Glossary of Congenital Cardiac Operations.- Cardiopulmonary Bypass and Myocardial Protection in Neonatal Surgery.- Systemic-Pulmonary Shunts.- Pulmonary Arterial Banding.- Blalock-Hanlon Atrial Septectomy.- Cavopulmonary (Glenn) Anastomosis.- Bidirectional Cavopulmonary Anastomosis.- Patent Ductus Arteriosus.- Coarctation of the Aorta.- Interrupted Aortic Arch.- Vascular Rings.- Atrial Septal Defect.- Ventricular Septal Defects.- Atrioventricular Septal Defect.- Critical Pulmonary Stenosis.- Pulmonary Atresia-Intact Ventricular Septum.- Tetralogy of Fallot.- Congenital Aortic Stenosis.- Double-Outlet Right Ventricle.- Truncus Arteriosus.- Transposition of the Great Arteries.- Tricuspid Atresia.- Total Anomalous Pulmonary Venous Drainage.- Aortopulmonary Window.- Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery.- Hypoplastic Left Heart Syndrome.