Adult Congenital Heart Disease (oxford Specialist Handbooks In Cardiology)

To the adult cardiologist, the language of congenital heart disease can be confusing and the spectrum of disease bewildering. Many find the prospect of meeting an adult with complex congenital heart disease in the emergency department disturbing, and rightly so. This book aims to dispel confusion and equip cardiology trainees, general cardiologists, and acute medicine physicians with a sound understanding of the principles of the physiology and management of adult congenital heart disease, so that they can treat emergencies and recognize the need for referral to a specialist unit. With easy-to-understand diagrams and key clinical points, this handbook provides both rapid reference for use when the clinical need arises and also an insight into the basic principles of congenital heart disease, giving the reader a good grounding in the care of the adult with congenital heart disease. Contents 20 Part 1 Introduction to adult congenital heart disease 26 1 Morphology and classification 28 Introduction 29 Physiological classification 29 Sequential segmental analysis 31 Atrial arrangement 33 2 Non-invasive imaging 36 Chest X-ray (CXR) 37 Transthoracic echocardiography (TTE) 43 Transoesophageal echo (TOE) 45 Cardiovascular magnetic resonance (CMR) imaging 49 Computed tomography (CT) 55 3 Physiological testing 58 Physiological testing 59 Types of exercise tests 61 Bruce exercise testing 62 Metabolic exercise testing 63 4 Cardiac catheterization 66 Introduction 67 Indications for catheterization 69 Precatheterization care 71 Calculations 73 5 Influence of pulmonary blood flow on management and outcome 74 Introduction 75 Pulmonary vascular development in early life 75 Cyanotic heart disease and pulmonary blood flow 77 6 Cyanosis 82 Introduction 83 Non-cardiac manifestations of cyanosis 85 Checklist for inpatients and emergencies with cyanotic heart disease 87 Part 2 Specfiic lessions 88 7 Valve and outflow tract lesions 90 7a: Left ventricular outflow tract obstruction (LVOTO) 92 Introduction 93 Subvalvar aortic stenosis (AS) 95 Bicuspid aortic valve 97 Supravalvar AS 99 7b: Left ventricular inflow lesions 100 Congenital mitral valve abnormalities 101 Cor triatriatum 103 Shone syndrome 105 7c: Right ventricular outflow tract obstruction (RVOTO) 106 Introduction 107 Pulmonary valvar stenosis 107 Supravalvar pulmonary stenosis 109 Pulmonary artery stenosis 109 Double-chambered right ventricle 109 7d: Ebstein anomaly 110 Definition 111 Incidence 111 Associations 111 Natural history 111 Presenting features in the adult 111 Physical signs 113 8 Septal defects 116 8a: Atrial septal defects (ASDs) 118 Introduction 119 Ostium secundum ASD 121 Ostium primum ASD 125 Sinus venosus ASD 125 Coronary sinus defect 127 Patent foramen ovale 129 8b: Ventricular septal defects (VSDs) 130 Definition and incidence 131 Cardiac associations 131 Presentation 131 Physical signs 133 Investigation 133 Repair 133 8c: Atrioventricular septal defects (AVSDs) 134 Definition 135 Associations 135 Incidence and recurrence 135 Clinical presentation 137 Investigation 137 Surgical management 137 Late complications post repair of AVSD 137 9 The Eisenmenger syndrome 138 Introduction 139 Natural history 139 Complications and extra-cardiac manifestations 139 Physical signs 139 Investigations 141 Management 141 10 Aortic lesions 142 Coarctation of the aorta (CoA) 143 Patent ductus arteriosus (PDA) 147 Aortopulmonary window 147 Common arterial trunk/truncus arteriosus 149 Marfan syndrome 151 11 Venous anomalies 152 11a: Anomalies of systemic venous drainage 154 Introduction 155 Superior vena cava (SVC) anomalies 155 Inferior vena cava (IVC) anomalies 157 11b: Anomalies of pulmonary venous drainage 158 Total anomalous pulmonary venous drainage (TAPVD) 159 Partial anomalous pulmonary venous drainage (PAPVD) 161 Scimitar syndrome 163 12 Transposition complexes 166 12a: Complete transposition of the great arteries (TGA) 168 Introduction 169 Complete TGA 171 Interatrial repair—Mustard or Senning operation 173 Arterial switch operation 175 Rastelli operation 177 12b: Congenitally corrected TGA (ccTGA) 178 ccTGA—AV and VA discordance 179 13 Tetralogy of Fallot and pulmonary atresia with VSD 184 Tetralogy of Fallot 185 Natural (unoperated) or shunt-palliated history 187 Operated history—follow up after radical repair 189 Pulmonary atresia with VSD 193 14 Functionally univentricular hearts and Fontan circulation 196 The functionally univentricular heart 197 Staged approach to achieve definitive palliation 199 Fontan circulation 201 Hypoplastic left heart syndrome (HLHS) 211 15 Rare conditions presenting in adulthood 214 Coronary anomalies 215 Sinus of valsalva aneurysm 217 Part 3 General management issues of adult congenital heart disease 220 16 Emergencies 222 Introduction 223 Haemoptysis in a cyanotic patient 223 Other emergencies and pitfalls to avoid in cyanotic heart disease 225 Haemoptysis or haematemesis in patient with repaired coarctation 227 Tachyarrhythmias 229 17 Contraception and pregnancy 232 Introduction 233 General principles 233 Contraception 235 Preconception 239 Pregnancy and delivery 243 Post-partum 245 18 Endocarditis 246 Endocarditis prophylaxis 247 19 Lifestyle issues 252 Exercise 253 Diving 255 High-altitude travel 257 Flying 257 Insurance 259 Appendix 1 List of operations for congenital heart disease 260 List of operations 261 Appendix 2 List of syndromes 266 List of syndromes and their associations 267 Glossary of terms 270 A 270 B 270 C 270 D 270 E 270 F 270 G 270 H 270 I 271 M 271 P 271 R 271 S 271 T 271 V 272 W 272 Index 274 A 274 B 274 C 274 D 275 E 275 F 275 G 275 H 275 I 275 K 275 L 275 M 275 N 276 O 276 P 276 R 276 S 276 T 277 V 277 W 277 X 277 To the adult cardiologist, the language of congenital heart disease can be confusing and the spectrum of disease bewildering. Many find the prospect of meeting an adult with complex congenital heart disease in the emergency department disturbing, and rightly so. This book aims to dispel confusion and equip cardiology trainees, general cardiologists and acute medicine physicians with a sound understanding of the principles of the physiology and management of adult congenital heart disease, so that they can treat emergencies and recognise the need for referral to a specialist unit. With easy-to-understand diagrams and key clinical points, this handbook provides both rapid reference for use when the clinical need arises and also an insight into the basic principles of congenital heart disease, giving the reader a good grounding in the care of the adult with congenital heart disease. This specialist handbook provides a succinct, bullet-pointed description of Adult Congenital Heart Disease providing easy reading and aiding reference. It appeals to all those who come across ACHD, and who need to know how to manage emergencies, and when to refer for specialist help.