Neonatal Heart Disease

ISBN-13: 9781447118169 / Angielski / Miękka / 2012 / 881 str.

Robert M. Freedom; Leland N. Benson; Jeffrey F. Smallhorn

Opis

As pediatric cardiology becomes more and more neonatal cardiology and even fetal cardiology, Neonatal Heart Disease by Robert M. Freedom, MO, Leland N. Benson, MD, and Jeffrey F. Smallhorn, MB is extraordinarily timely. Neonatal Heart Disease consists of 50 chapters by 25 distinguished contributors and is a worthy successor to The Neonate With Congenital Heart Disease by Richard D. Rowe, MD and his colleagues (1968 and 1981). The first dition of this book in 1968 established Richard D. Rowe, MD as the father of neonatal cardiology. As most pediatric cardiologists now know, Dick Rowe died on January 18, 1988 after a brief illness. It will therefore come as no surprise that the present volume is dedicated to this great and gentle man. Nor will it come as a surprise that I have been asked to devote this Foreword to Richard D. Rowe, MD, pioneering neonatal cardiologist and incomparable personal friend. What can one say about Dick Rowe? Well, there are at least two very different tales. There is Dick Rowe the public man -the factual account of Dick Rowe's achievements as a physician, educator, and research man - the Dick Rowe that virtually "everyone" knows. And then there is Dick Rowe the private man -the extraordinary human being who only his personal friends were privileged to know. I shall try to tell something of both stories. First, the public man - the factual account - is really quite amazing."

Spis treści

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.