The Natural and Unnatural History of Congenital Heart Disease

Evaluates the natural history of congenital heart lesions as a background to finding out if and how much treatment has improved outcomes Introduces and defines lesions, providing general information about its frequency, familial or syndromic associations, and associated congenital heart lesions Provides sections on pathological anatomy and physiology – important in determining outcomes Includes results of surgery, both in terms of survival and also in terms of event-free survival, that is, survival free of reoperation, cardiac failure, arrhythmias, and other late complications that are often seen Helps cardiologists and cardiac surgeons understand what is likely to happen to patients with or without treatment, and which forms of treatment currently in use provide the best outcomes to date

Foreword vii Preface ix Part 1 Introduction 1 Practical and Theoretical Considerations 3 2 Associated Noncardiac Problems 18 Part 2 Surgical Procedures 3 Aortopulmonary Shunts 25 4 Banding the Pulmonary Artery 33 5 Valves and Conduits 37 6 “Single Ventricle” Repairs 57 7 Transplantation 71 Part 3 Left-to-Right Shunts Section A Aortic Origin 8 Patent Ductus Arteriosus 79 9 Anomalous Origin of One Pulmonary Artery from the Aorta (Hemitruncus) 93 10 Aortopulmonary Window 97 11 Systemic Arteriovenous Fistula 103 12 Coronary Arterial Fistula 111 13 Congenital Aneurysms of the Sinus of Valsalva 121 Section B Ventricular or Atrial Origin 14 Atrial Septal Defect (Secundum) 133 15 Partial Anomalous Pulmonary Venous Connection with an Intact Atrial Septum 157 16 Scimitar Syndrome 161 17 Ostium Primum Defect 167 18 Complete Atrioventricular Septal Defect 175 19 Isolated Ventricular Septal Defect 183 20 Congenitally Corrected Transposition of the Great Arteries 206 Part 4 Obstructive Lesions Section C Right Ventricular Outflow Tract 21 Pulmonary Stenosis 223 22 Double-Chambered Right Ventricle 234 23 Stenosis of the Main and Branch Pulmonary Arteries 237   Section D Left Ventricular Outflow Tract and Aorta 24 Valvar Aortic Stenosis 245 25 Bicuspid Aortic Valve without Stenosis 264 26 Supravalvar Aortic Stenosis 277 27 Discrete Subvalvar Aortic Stenosis 282 28 Coarctation of the Aorta 291 29 Interrupted Aortic Arch 318 Section E Left Ventricular Inflow Tract 30 Cor Triatriatum Sinister 329 31 Congenital Atresia or Stenosis of Pulmonary Veins 335 32 Congenital Mitral Valve Obstruction 341 Section F Regurgitant Lesions 33 Aortic Valve Regurgitation 353 34 Aortoventricular Tunnel 356 35 Mitral Valve Regurgitation 359 36 Tricuspid Valve Regurgitation 364 37 Idiopathic Pulmonary Regurgitation 368 Part 5 Miscellaneous Aortic Root Lesions 38 Anomalous Origin of a Coronary Artery from the Pulmonary Artery 373 39 Abnormal Origins of the Coronary Arteries from the Aortic Root 384 40 Vascular Rings and Slings 401 Part 6 Right-to-Left Shunts 41 Tetralogy of Fallot 413 42 Tetralogy of Fallot with Pulmonary Atresia 437 43 Tetralogy of Fallot with Absent Pulmonary Valve 446 44 Complete Transposition of the Great Arteries 451 45 Tricuspid Atresia 486 46 Pulmonary Atresia with Intact Ventricular Septum 494 47 Ebstein Anomaly 507 48 Truncus Arteriosus 519 49 Hypoplastic Left Heart Syndrome 531 50 Single (Double-Inlet) Ventricle 546 51 Double-Outlet Right or Left Ventricle 556 52 Total Anomalous Pulmonary Venous Connection 566 53 Heterotaxy Syndromes 579 54 Pulmonary Arteriovenous Fistula 587 55 Eisenmenger Syndrome 595 Index 605