BOOK
Diagnosis and Management of Adult Congenital Heart Disease E-Book
Michael A. Gatzoulis | Gary D. Webb | Piers E. F. Daubeney
(2010)
Additional Information
Book Details
Abstract
Diagnosis and Management of Adult Congenital Heart Disease, by Drs. Gatzoulis, Webb, and Daubeney, is a practical, one-stop resource designed to help you manage the unique challenges of treating long-term adult survivors of congenital heart disease. Authored by internationally known leaders in the field, this edition is the first that truly integrates anatomy and imaging technology into clinical practice, and includes new chapters on cardiac CT for ACHD assessment, critical and perioperative care, anesthesia for ACHD surgery, cardiac resynchronization therapy, and transition of care. Congenital defects are presented with high-quality illustrations and appropriate imaging modalities.
- Find all the information you need in one user-friendly resource that integrates anatomy, clinical signs, and therapeutic options.
- Confidently make decisions aided by specific recommendations about the benefits and risks of surgeries, catheter interventions, and drug therapy for difficult clinical problems.
- Recognize and diagnose morphologic disorders with the help of detailed, full-color diagrams.
Quickly find what you need thanks to easily accessible, consistently organized chapters and key annotated references.
- Keep pace with the latest advancements including five new chapters on cardiac CT for ACHD assessment, critical and perioperative care, anaesthesia for ACHD surgery, cardiac resynchronisation therapy, and transition of care
- Comply with the latest European Society of Cardiology (ESC) and American College of Cardiology (ACC) practice guidelines - integrated throughout the book - for cardiac pacing and cardiac resynchronisation therapy
- See imaging findings as they appear in practice and discern subtle nuances thanks to new, high-quality images and illustrations
Integrates anatomy, clinical signs and therapeutic options of congenital heart disease both in print and online!
Table of Contents
| Section Title | Page | Action | Price |
|---|---|---|---|
| Front Cover | Cover | ||
| Diagnosis and Management of Adult Congenital Heart Disease | iii | ||
| Copyright Page | iv | ||
| Dedication Page | v | ||
| Contributors | vii | ||
| Foreword | xiii | ||
| Preface | xv | ||
| Contents | xvii | ||
| Part I: General Principles | 1 | ||
| Chapter 1: Adults with Congenital Heart Disease:A Growing Population | 2 | ||
| Introduction | 2 | ||
| Organization of Care | 2 | ||
| Manpower, Training, and Research | 3 | ||
| Transfer of Care | 4 | ||
| Conclusion | 4 | ||
| References | 4 | ||
| Chapter 2: Cardiac Morphology and Nomenclature | 5 | ||
| Sequential Segmental Analysis: General Philosophy | 5 | ||
| Morphology of the Cardiac Chambers | 5 | ||
| Arrangement of Atrial Chambers | 8 | ||
| Determination of Ventricular Morphology and Topology | 9 | ||
| Analysis of the Atrioventricular Junction | 9 | ||
| Determination of Morphology of the Great Arteries | 11 | ||
| Analysis of the Ventriculoarterial Junction | 12 | ||
| Associated Malformations | 12 | ||
| Location of the Heart | 12 | ||
| Conclusion | 12 | ||
| References | 13 | ||
| Chapter 3: Adults with Congenital Heart Disease: A Genetic Perspective | 14 | ||
| What Is Congenital Heart Disease? | 14 | ||
| Genetic Evidence for Congenital Heart Disease | 14 | ||
| From Phenotype to Genotype | 14 | ||
| Clinical Utility of Genetic Testing | 15 | ||
| Current State of Genetic Technology | 17 | ||
| References | 18 | ||
| Chapter 4: Clinical Assessment | 19 | ||
| The History | 19 | ||
| The Physical Examination | 20 | ||
| Conclusion | 27 | ||
| References | 27 | ||
| Chapter 5: Echocardiography | 28 | ||
| Introduction | 28 | ||
| Using Segmental Analysis to Describe Abnormal Cardiovascular Connections | 28 | ||
| Echocardiography in Specific Diagnostic Groups | 28 | ||
| Special Topics in Adult Congenital Imaging | 41 | ||
| Conclusion | 43 | ||
| References | 43 | ||
| Chapter 6: Heart Failure, Exercise Intolerance,and Physical Training | 44 | ||
| Heart Failure in Adults with Congenital Heart Disease | 44 | ||
| Systemic Manifestations of the Heart Failure Syndrome in Adult Congenital Heart Disease | 48 | ||
| Treatment | 48 | ||
| References | 51 | ||
| Chapter 7: Cardiovascular Magnetic Resonance Imaging | 52 | ||
| Introduction | 52 | ||
| Safety | 52 | ||
| General Considerations | 52 | ||
| Image Display and Analysis | 53 | ||
| Techniques | 54 | ||
| Applications of Cardiac Magnetic Resonance Imaging in Specific Diseases | 55 | ||
| Conclusion | 57 | ||
| References | 58 | ||
| Chapter 8: Cardiac Computed Tomography | 59 | ||
| Introduction | 59 | ||
| Technical Considerations | 59 | ||
| Cardiac Computed Tomography in Clinical Practice | 60 | ||
| Conclusion | 67 | ||
| References | 67 | ||
| Chapter 9: Cardiac Catheterization in Adult Congenital Heart Disease | 68 | ||
| Introduction | 68 | ||
| Planning the Procedure | 68 | ||
| What Can Go Wrong | 69 | ||
| Equipment for Interventional Cardiac Catheterization | 69 | ||
| Flows and Shunts | 72 | ||
| Coronary Angiography | 73 | ||
| Chamber Angiography | 74 | ||
| Specific Lesions | 75 | ||
| Changing Indications for Cardiac Catheterization in ACHD | 81 | ||
| Conclusion | 84 | ||
| References | 84 | ||
| Chapter 10: Late Repair and Reoperations in Adults with Congenital Heart Disease | 86 | ||
| Introduction | 86 | ||
| Special Considerations | 86 | ||
| Management of Arrhythmias Associated with Right-Sided Lesions Requiring Surgical Intervention | 87 | ||
| Conclusion | 90 | ||
| References | 90 | ||
| Chapter 11: Venous Shunts and the Fontan Circulation in Adult Congenital Heart Disease | 91 | ||
| Introduction | 91 | ||
| Historical Aspects of Venous Shunts | 91 | ||
| Pathophysiologic Aspects of Venous Shunts | 91 | ||
| Fontan Circulation Complications | 93 | ||
| Therapeutic Options | 94 | ||
| Results of Fontan Conversion, Arrhythmia Surgery, and Repair of Associated Lesions | 96 | ||
| Conclusion | 100 | ||
| References | 102 | ||
| Chapter 12: Late Complications Following the Fontan Operation | 104 | ||
| Introduction | 104 | ||
| Clinical Evaluation | 104 | ||
| History and Long-Term Sequelae | 106 | ||
| Conclusion | 109 | ||
| References | 109 | ||
| Chapter 13: Heart and Lung Transplantation in Adult Congenital Heart Disease | 110 | ||
| Introduction | 110 | ||
| Indications | 110 | ||
| Types of Heart Disease Requiring Transplantation | 110 | ||
| Timing of Transplantation | 110 | ||
| Pre-transplantation Evaluation | 110 | ||
| Surgical Considerations | 111 | ||
| Outcomes | 112 | ||
| Conclusion | 114 | ||
| References | 114 | ||
| Chapter 14: Noncardiac Surgery in Adult Congenital Heart Disease | 115 | ||
| Introduction | 115 | ||
| Assessment of Risk | 115 | ||
| Perioperative Management | 116 | ||
| Postoperative Management | 119 | ||
| References | 120 | ||
| Chapter 15: Critical Care | 121 | ||
| Introduction | 121 | ||
| Cardiac Anatomy | 121 | ||
| Vascular Access | 121 | ||
| Infective Endocarditis Prophylaxis | 121 | ||
| Feeding | 122 | ||
| Coagulation Defects and Anticoagulation | 122 | ||
| Management of Oxygen Delivery | 122 | ||
| Conclusion | 124 | ||
| References | 124 | ||
| Chapter 16: Arrhythmias in Adults with Congenital Heart Disease | 126 | ||
| Introduction | 126 | ||
| Bradycardia | 126 | ||
| Atrial Tachycardias | 127 | ||
| Accessory Atrioventricular Pathways | 128 | ||
| Ventricular Tachycardia | 129 | ||
| Conclusion | 131 | ||
| References | 131 | ||
| Chapter 17: Invasive Electrophysiology and Pacing | 132 | ||
| Introduction | 132 | ||
| Atrial Arrhythmias | 132 | ||
| Ventricular Arrhythmias | 135 | ||
| Devices in Adult Congenital Heart Disease | 135 | ||
| Conclusion | 137 | ||
| References | 137 | ||
| Chapter 18: Cardiac Resynchronization Therapy in Adult Congenital Heart Disease | 138 | ||
| Cardiac Resynchronization Therapy in Adults Without CHD | 138 | ||
| First Investigations of Cardiac Resynchronization Therapy in Congenital Heart Disease—Early Postoperative Pediatric Congenital Heart Disease | 138 | ||
| Defining Heart Failure in Adult Congenital Heart Disease | 138 | ||
| Heart Failure: Subpulmonary Right Ventricle | 139 | ||
| Heart Failure: Systemic Right Ventricle | 142 | ||
| Heart Failure: Systemic Left Ventricle | 144 | ||
| Heart Failure: Single-Ventricle Physiology | 144 | ||
| Conclusion | 145 | ||
| References | 145 | ||
| Chapter 19: Infective Endocarditis | 147 | ||
| Introduction | 147 | ||
| Incidence and Patients at Risk | 147 | ||
| Effect of Surgical Repair | 147 | ||
| Antecedent Events | 147 | ||
| Clinical Manifestations | 148 | ||
| Investigation | 149 | ||
| Diagnosis | 151 | ||
| Management | 152 | ||
| Prognosis | 152 | ||
| Prophylaxis | 152 | ||
| References | 153 | ||
| Chapter 20: Transition of the Young Adult with Complex Congenital Heart Disease from Pediatric to Adult Care | 154 | ||
| Important Aspects of the Transition Process | 154 | ||
| Key Elements of an Ideal Transition Program | 154 | ||
| Case 20-1 | 155 | ||
| Congenital Heart Disease Curriculum | 155 | ||
| Case 20-2 | 156 | ||
| Policy on the Timing of Transfer from Pediatric Care | 156 | ||
| Types of Services Needed in the Adult Congenital Heart Disease Environment | 156 | ||
| Steps to an Orderly Transfer Process | 157 | ||
| How to Design a Transition Process | 157 | ||
| References | 157 | ||
| Chapter 21: Pregnancy and Contraception | 158 | ||
| Introduction | 158 | ||
| Hemodynamic Changes During Pregnancy, Labor and Delivery, and Postpartum | 158 | ||
| Cardiac Examination During Pregnancy | 158 | ||
| Pregnancy Counseling for Patients with Cardiac Disease | 158 | ||
| Prognosis of Heart Disease in Pregnancy | 158 | ||
| Cardiac Contraindications to Pregnancy | 158 | ||
| Management of the Pregnant Patient with Cardiac Disease | 159 | ||
| Conclusion | 164 | ||
| References | 164 | ||
| Chapter 22: Obstetric Analgesia and Anesthesia | 165 | ||
| Introduction | 165 | ||
| Physiologic Effects of Pregnancy | 165 | ||
| Analgesia for Labor | 165 | ||
| Anesthesia for Cesarean Section and Other Procedures in Pregnant Patients with Congenital Heart Disease | 165 | ||
| Specific Considerations for Certain Conditions | 167 | ||
| What Anesthetists Want to Know from Cardiologists | 167 | ||
| Conclusion | 167 | ||
| References | 167 | ||
| Chapter 23: Anesthesia in Adult Congenital Heart Disease, Including Anesthesia for Noncardiac Surgery | 168 | ||
| Introduction | 168 | ||
| General Anesthesia for Cardiac Surgery | 168 | ||
| Anesthesia for Noncardiac Surgery | 170 | ||
| References | 170 | ||
| Chapter 24: Insurability of Adults with Congenital Heart Disease | 171 | ||
| Introduction | 171 | ||
| Expression of Mortality | 171 | ||
| Gender Difference in Mortality | 171 | ||
| Smoking/Nonsmoking Differences | 171 | ||
| Mortality and the Underwriting Process | 171 | ||
| Age and Mortality Ratios | 172 | ||
| How You Can Help Your Patient Obtain Insurance Coverage | 175 | ||
| Conclusion | 175 | ||
| References | 176 | ||
| Chapter 25: Atrial Septal Defect | 180 | ||
| Definition and Morphology | 180 | ||
| Genetics and Epidemiology | 180 | ||
| Early Presentation and Management | 180 | ||
| Late Outcome | 181 | ||
| Outpatient Assessment | 182 | ||
| Late Management Options | 184 | ||
| Arrhythmia and Sudden Cardiac Death | 186 | ||
| Pregnancy | 186 | ||
| Level of Follow-Up, Endocarditis Prophylaxis, and Exercise | 186 | ||
| References | 187 | ||
| Part II: Septal Defects | 179 | ||
| Chapter 26: Ventricular Septal Defect | 188 | ||
| Definition and Morphology | 188 | ||
| Associated Abnormalities | 188 | ||
| Prevalence and Genetic Factors | 188 | ||
| Pathophysiology | 188 | ||
| Early Clinical Presentation | 189 | ||
| Management in Childhood | 190 | ||
| Late Outcome and Complications | 191 | ||
| Outpatient Assessment | 192 | ||
| Management | 194 | ||
| Pregnancy | 195 | ||
| Level of Follow-Up, Endocarditis Prophylaxis, and Exercise | 195 | ||
| References | 195 | ||
| Chapter 27: Atrioventricular Septal Defect: Complete and Partial (Ostium Primum Atrial Septal Defect) | 196 | ||
| Definition and Morphology | 196 | ||
| Genetics and Epidemiology | 197 | ||
| Early Presentation | 197 | ||
| Early Management of Atrioventricular Septal Defect | 200 | ||
| Late Outcome | 200 | ||
| Outpatient Assessment of the Adult with Atrioventricular Septal Defect | 200 | ||
| Late Management Options | 201 | ||
| Arrhythmia and Sudden Cardiac Death | 201 | ||
| Pregnancy | 202 | ||
| Level of Follow-up, Endocarditis Prophylaxis, and Exercise | 202 | ||
| References | 203 | ||
| Part III: Diseases of the Mitral Valve | 205 | ||
| Chapter 28: Cor Triatriatum and Mitral Stenosis | 206 | ||
| Introduction | 206 | ||
| Cor Triatriatum | 206 | ||
| Definition and Morphology | 206 | ||
| Associated Defects | 206 | ||
| Embryology | 206 | ||
| Anatomy | 206 | ||
| Early Presentation | 206 | ||
| Late Presentation | 206 | ||
| Diagnosis | 207 | ||
| Management | 208 | ||
| Late Outcome | 208 | ||
| Pregnancy | 209 | ||
| Level of Follow-Up, Endocarditis Prophylaxis, and Exercise | 209 | ||
| Mitral stenosis | 209 | ||
| Definition and Epidemiology | 209 | ||
| Anatomy | 209 | ||
| Associated Defects | 209 | ||
| Early Presentation | 209 | ||
| Adult Presentation | 209 | ||
| Investigations | 210 | ||
| Management | 210 | ||
| Pregnancy | 210 | ||
| Level of Follow-Up, Endocarditis Prophylaxis, and Exercise | 210 | ||
| References | 210 | ||
| Chapter 29: Mitral Valve Prolapse, Mitral Regurgitation | 212 | ||
| Introduction | 212 | ||
| Anatomy of the Mitral Valve | 212 | ||
| Definition, Etiology, and Pathology | 212 | ||
| Prevalence | 212 | ||
| Early Presentation | 212 | ||
| Early Management | 214 | ||
| Late Outcome | 214 | ||
| Late Management Options | 215 | ||
| Pregnancy | 215 | ||
| Exercise and Mitral Valve Prolapse | 215 | ||
| Conclusion | 215 | ||
| References | 215 | ||
| Chapter 30: Partial Anomalous Pulmonary Venous Connections and the Scimitar Syndrome | 216 | ||
| Introduction | 216 | ||
| Definition and morphology | 216 | ||
| Scimitar Syndrome | 216 | ||
| Genetics and Epidemiology | 216 | ||
| Early Presentation and Management | 218 | ||
| Late Outcome | 218 | ||
| Outpatient Assessment | 219 | ||
| Late Management Options | 221 | ||
| Pregnancy | 221 | ||
| Endocarditis Prophylaxis, Exercise, and Noncardiac Surgery | 221 | ||
| Acknowledgments | 221 | ||
| References | 221 | ||
| Part IV: Diseases of the Left Ventricular Outflow Tract | 223 | ||
| Chapter 31: Valvular Aortic Stenosis | 224 | ||
| Introduction | 224 | ||
| Definition, Morphology, and Epidemiology | 224 | ||
| Associated Lesions | 224 | ||
| Genetics and Molecular Biology | 224 | ||
| Pathophysiology and Clinical Course (Without Intervention) | 225 | ||
| Physical Examination | 226 | ||
| Investigations | 226 | ||
| Management | 228 | ||
| Outcomes and Follow-Up | 234 | ||
| References | 235 | ||
| Chapter 32: Subvalvular and Supravalvular Aortic Stenosis | 236 | ||
| Subvalvular aortic stenosis | 236 | ||
| Late Outcomes and Complications | 238 | ||
| Level of Follow-Up | 238 | ||
| Supravalvular aortic stenosis | 238 | ||
| Pregnancy in subvalvular and supravalvular aortic stenosis | 241 | ||
| References | 241 | ||
| Chapter 33: Aortic Regurgitation | 243 | ||
| Introduction | 243 | ||
| Morphology | 243 | ||
| Pathophysiology | 243 | ||
| Natural History | 244 | ||
| Genetics and Epidemiology | 244 | ||
| Early Presentation and Management | 244 | ||
| Late Outcome | 246 | ||
| Pregnancy | 248 | ||
| Level of Follow-Up, Endocarditis Prophylaxis, and Exercise | 248 | ||
| References | 248 | ||
| Chapter 34: Sinus of Valsalva Aneurysms | 250 | ||
| Introduction | 250 | ||
| Morphology | 250 | ||
| Associated Defects | 250 | ||
| Epidemiology | 250 | ||
| Investigations | 250 | ||
| Presentation | 251 | ||
| Management | 252 | ||
| Outpatient Assessment | 252 | ||
| Arrhythmia and Sudden Death | 252 | ||
| Pregnancy | 252 | ||
| Level of Follow-Up and Endocarditis Prophylaxis | 253 | ||
| References | 253 | ||
| Part V: Diseases of the Aorta | 255 | ||
| Chapter 35: Patent Ductus Arteriosus and Aortopulmonary Window | 256 | ||
| Patent Ductus Arteriosus | 256 | ||
| Late Management Options | 257 | ||
| Catheter Intervention and Surgical Outcomes | 258 | ||
| Pregnancy | 258 | ||
| Level of Follow-Up, Endocarditis Prophylaxis, and Exercise | 258 | ||
| Aortopulmonary window | 258 | ||
| References | 260 | ||
| Chapter 36: Aortic Coarctation and Interrupted Aortic Arch | 261 | ||
| Coarctation of the Aorta | 261 | ||
| Late Outcome | 263 | ||
| Outpatient Assessment | 265 | ||
| Late Management Options | 267 | ||
| Pregnancy | 268 | ||
| Level of Follow-Up, Endocarditis Prophylaxis, and Exercise | 268 | ||
| Interrupted Aortic Arch | 268 | ||
| Late Outcome | 269 | ||
| Outpatient Assessment | 269 | ||
| Late Management Options | 269 | ||
| Level of Follow-Up, Endocarditis Prophylaxis, and Exercise | 269 | ||
| References | 270 | ||
| Chapter 37: Truncus Arteriosus | 271 | ||
| Definition and Morphology | 271 | ||
| Early Presentation\nand Management | 272 | ||
| Surgical History | 273 | ||
| Late Outcome | 274 | ||
| Outpatient Assessment | 274 | ||
| Pregnancy | 275 | ||
| Endocarditis Prophylaxis and Exercise | 276 | ||
| References | 276 | ||
| Chapter 38: Vascular Rings, Pulmonary Slings, and Other Vascular Abnormalities | 277 | ||
| Introduction | 277 | ||
| Normal Embryogenesis | 277 | ||
| Classification and Morphology of Individual Lesions | 277 | ||
| Genetics and Epidemiology | 281 | ||
| Clinical Manifestations | 281 | ||
| Investigations | 282 | ||
| Management | 283 | ||
| Late Outcome | 284 | ||
| Arrhythmia and Sudden Cardiac Death | 284 | ||
| Pregnancy | 284 | ||
| Level of Follow-Up, Endocarditis Prophylaxis, and Exercise | 284 | ||
| References | 285 | ||
| Part VI: Diseases of the Tricuspid Valve | 287 | ||
| Chapter 39: Ebstein Anomaly | 288 | ||
| Definition and Morphology | 288 | ||
| Genetics and Epidemiology | 288 | ||
| Early Presentation and Management | 288 | ||
| Late Outcome | 288 | ||
| Late Complications | 288 | ||
| Outpatient Assessment | 288 | ||
| Late Management | 289 | ||
| Atrial Arrhythmias and Sudden Cardiac Death | 293 | ||
| Pregnancy | 293 | ||
| Level of Follow-Up, Endocarditis Prophylaxis, and Exercise | 293 | ||
| References | 293 | ||
| Chapter 40: Tricuspid Stenosis and Regurgitation | 295 | ||
| Definitions and Morphology | 295 | ||
| Genetics and Epidemiology | 295 | ||
| Early Presentation and Management | 295 | ||
| Late Outcome | 296 | ||
| Outpatient Assessment | 297 | ||
| Late Management Options | 299 | ||
| Arrhythmia and Sudden Cardiac Death | 300 | ||
| Pregnancy, Exercise, and Endocarditis Prophylaxis | 300 | ||
| References | 301 | ||
| Part VII: Diseases of the Right Ventricular Outflow Tract | 303 | ||
| Chapter 41: Pulmonary Stenosis | 304 | ||
| Definition and Morphology | 304 | ||
| Early Presentation and Management | 304 | ||
| Late Outcome | 304 | ||
| Late Complications | 304 | ||
| Outpatient Assessment | 304 | ||
| Late Management Options | 306 | ||
| Arrhythmia and Sudden Cardiac Death | 307 | ||
| Pregnancy | 307 | ||
| Level of Follow-Up, Endocarditis Prophylaxis, and Exercise | 307 | ||
| References | 307 | ||
| Chapter 42: Double-Chambered Right Ventricle | 308 | ||
| Definition and Morphology | 308 | ||
| Genetics and Epidemiology | 308 | ||
| Early Presentation and Management | 308 | ||
| Late Outcomes | 310 | ||
| Outpatient Assessment | 310 | ||
| Late Management Options | 311 | ||
| Arrhythmia and Sudden Cardiac Death | 312 | ||
| Pregnancy | 312 | ||
| Level of Follow-Up | 313 | ||
| Endocarditis Prophylaxis | 313 | ||
| Exercise | 313 | ||
| References | 313 | ||
| Part VIII: Cyanotic Conditions | 315 | ||
| Chapter 43: Tetralogy of Fallot | 316 | ||
| Definition and Morphology | 316 | ||
| Genetics and Epidemiology | 316 | ||
| Early Presentation and Management | 317 | ||
| Late Outcomes | 318 | ||
| Outpatient Assessment | 318 | ||
| Late Management Options | 323 | ||
| Arrhythmia and Sudden Cardiac Death | 324 | ||
| Pregnancy | 326 | ||
| Level of Follow-Up | 326 | ||
| Endocarditis Prophylaxis | 327 | ||
| Exercise | 327 | ||
| Future Therapies | 327 | ||
| References | 327 | ||
| Chapter 44: Pulmonary Atresia with Ventricular Septal Defect | 328 | ||
| Introduction | 328 | ||
| Anatomy | 328 | ||
| Genetics and Epidemiology | 328 | ||
| Early Presentation | 328 | ||
| Management | 330 | ||
| Long-Term Outcome | 331 | ||
| Outpatient Assessment | 332 | ||
| Long-Term Management | 333 | ||
| Pregnancy | 334 | ||
| Exercise and Endocarditis Prophylaxis | 335 | ||
| References | 335 | ||
| Chapter 45: Absent Pulmonary Valve Syndrome | 336 | ||
| Definition and Morpholog | 336 | ||
| Epidemiology and Genetics | 336 | ||
| Tetralogy of Fallot and Absent Pulmonary Valve | 336 | ||
| Absent Pulmonary Valve In Isolation | 337 | ||
| References | 338 | ||
| Chapter 46: Pulmonary Atresia with Intact Ventricular Septum | 339 | ||
| Definition and Morphology | 339 | ||
| Genetics and Epidemiology | 339 | ||
| Fetal Presentation | 339 | ||
| Early Presentation and Management | 339 | ||
| Late Outcome | 342 | ||
| Outpatient Assessment | 343 | ||
| Late Management Options | 344 | ||
| Arrhythmia and Sudden Cardiac Death | 345 | ||
| Pregnancy | 346 | ||
| Follow-Up, Endocarditis Prophylaxis, and Exercise | 346 | ||
| References | 346 | ||
| Chapter 47: Transposition of the Great Arteries | 347 | ||
| Definition and Morphology | 347 | ||
| Associated Defects | 347 | ||
| Genetics and Epidemiology | 347 | ||
| Early Presentation and Management | 348 | ||
| Late Outcome | 350 | ||
| Outpatient Assessment | 354 | ||
| Late Management Options | 355 | ||
| Pregnancy | 356 | ||
| Level of Follow-Up, Endocarditis Prophylaxis, and Exercise | 357 | ||
| References | 357 | ||
| Chapter 48: Eisenmenger Syndrome | 358 | ||
| Definition and Morphology | 358 | ||
| Etiology | 358 | ||
| Clinical Classification (Dana Point) | 359 | ||
| Genetics and Epidemiology | 359 | ||
| Early Presentation and Management | 360 | ||
| Late Outcome | 360 | ||
| Outpatient Assessment | 362 | ||
| Late Management Options | 365 | ||
| Arrhythmia and Sudden Cardiac Death | 368 | ||
| Pregnancy | 369 | ||
| Level of Follow-Up and Endocarditis Prophylaxis | 369 | ||
| Exercise, Air Travel, and Exposure to High Altitude | 369 | ||
| References | 369 | ||
| Chapter 49: Congenitally Corrected Transposition of the Great Arteries | 371 | ||
| Definition and Morphology | 371 | ||
| Genetics and Epidemiology | 371 | ||
| Early Presentation and Management | 371 | ||
| Late Outcome | 372 | ||
| Outpatient Assessment | 374 | ||
| Arrhythmia and Sudden Death | 376 | ||
| Pregnancy | 376 | ||
| Level of Follow-Up, Endocarditis Prophylaxis, and Exercise | 377 | ||
| References | 377 | ||
| Chapter 50: Double-Outlet Right Ventricle | 378 | ||
| Introduction | 378 | ||
| Definition | 378 | ||
| History | 378 | ||
| Embryology | 378 | ||
| Epidemiology | 378 | ||
| Classification | 378 | ||
| Pathophysiology and Presentation | 379 | ||
| Other Considerations | 380 | ||
| Indications for Repair and Preoperative Evaluation | 380 | ||
| Surgical Repair | 381 | ||
| Special Situations | 382 | ||
| Surgical Outcomes | 382 | ||
| Outpatient Assessment of the Adult with Double-Outlet Right Ventricle | 382 | ||
| Arrhythmias and Sudden Cardiac Death | 383 | ||
| Pregnancy | 383 | ||
| Follow-Up and Endocardit\nProphylaxis | 383 | ||
| Conclusion | 383 | ||
| References | 384 | ||
| Part IX: Univentricular Hearts | 385 | ||
| Chapter 51: Double-Inlet Ventricle | 386 | ||
| Definition and Morphology | 386 | ||
| Genetics and Epidemiology | 386 | ||
| Early Presentation and Management | 386 | ||
| Management | 386 | ||
| Late Outcome | 388 | ||
| Outpatient Assessment | 388 | ||
| Late Management Options | 388 | ||
| Arrhythmia and Sudden Cardiac Death | 389 | ||
| Pregnancy | 389 | ||
| Level of Follow-up, Endocarditis Prophylaxis, and Exercise | 389 | ||
| References | 389 | ||
| Chapter 52: Atrioventricular Valve Atresia | 390 | ||
| Introduction | 390 | ||
| Tricuspid Atresia | 390 | ||
| Mitral Atresia and Hypoplastic Left Heart Syndrome | 391 | ||
| Late Outcome | 393 | ||
| Outpatient Assessment | 394 | ||
| Post-Fontan or Post–Total Cavopulmonary Connection Patients with Tricuspid Atresia or Hypoplastic Left Heart Syndrome | 394 | ||
| Late Management Options | 395 | ||
| Arrhythmia | 395 | ||
| Pregnancy and Contraception | 395 | ||
| Level of Follow-Up, Endocarditis Prophylaxis, and Exercise | 396 | ||
| References | 396 | ||
| Chapter 53: Heterotaxy and Isomerismof the Atrial Appendages | 397 | ||
| Definitions | 397 | ||
| Morphology | 397 | ||
| Epidemiology and Genetics | 398 | ||
| Associated Cardiac Lesions in Heterotaxy Syndrome | 399 | ||
| Early Presentation and Initial Management | 399 | ||
| Late Presentation and Outcomes | 399 | ||
| Complications and Late Management Options | 400 | ||
| Outpatient Assessment | 400 | ||
| Conduction System Abnormalities and Arrhythmias | 401 | ||
| Pregnancy | 401 | ||
| Level of Follow-Up and Endocarditis Prophylaxis | 401 | ||
| References | 402 | ||
| Part X: Coronary ArteryAbnormalities | 403 | ||
| Chapter 54: Congenital Anomalies of the Coronary Arteries | 404 | ||
| Primary Congenital Coronary Anomalies | 404 | ||
| Secondary Congenital Coronary Anomalies | 409 | ||
| References | 410 | ||
| Chapter 55: Kawasaki Disease | 411 | ||
| Definition and Morphology | 411 | ||
| Genetics, Epidemiology, and Etiology | 411 | ||
| Early Presentation and Management | 411 | ||
| Adult-Onset Kawasaki Disease | 411 | ||
| Late Outcome and Long-Term Management | 412 | ||
| Late Management Options | 414 | ||
| Pregnancy | 414 | ||
| Level of Follow-Up | 415 | ||
| References | 415 | ||
| Part XI: Other Lesions | 417 | ||
| Chapter 56: Myocarditis and DilatedCardiomyopathy | 418 | ||
| Introduction | 418 | ||
| Definition | 418 | ||
| Epidemiology and Etiology | 418 | ||
| Pathogenesis | 418 | ||
| Clinical Presentation and Management | 419 | ||
| Outpatient Assessment, Level of Follow-Up, and Exercise | 420 | ||
| Arrhythmia and Sudden Cardiac Death | 420 | ||
| Evidence of Myocardial Inflammation and Infection | 420 | ||
| Emerging Role of Cardiovascular Magnetic Resonance | 421 | ||
| Prognosis | 422 | ||
| Pregnancy | 422 | ||
| Conclusion | 424 | ||
| References | 424 | ||
| Chapter 57: Hypertrophic Cardiomyopathy | 425 | ||
| Definition | 425 | ||
| Morphology and Pathophysiology | 425 | ||
| Epidemiology | 425 | ||
| Genetics | 425 | ||
| Presentation and Outpatient Assessment | 426 | ||
| Late Outcomes | 427 | ||
| Management | 428 | ||
| Pregnancy | 429 | ||
| Endocarditis Prophylaxis and Exercise | 429 | ||
| References | 429 | ||
| Chapter 58: Constrictive Pericarditis and Restrictive Cardiomyopathy | 430 | ||
| Introduction | 430 | ||
| Definition and Morphology | 430 | ||
| Epidemiology and Genetics | 430 | ||
| Early Presentation and Management | 430 | ||
| Late Outcome | 436 | ||
| Outpatient Assessment | 436 | ||
| Late Management Options | 436 | ||
| Arrhythmia and Sudden Cardiac Death | 436 | ||
| Pregnancy | 436 | ||
| Level of Follow-Up, Endocarditis Prophylaxis, and Exercise | 436 | ||
| References | 437 | ||
| Chapter 59: Arrhythmogenic Right Ventricular Cardiomyopathy | 438 | ||
| Introduction | 438 | ||
| Clinical Features and Natural History | 438 | ||
| Pathology and Pathogenesis | 438 | ||
| Clinical and Differential Diagnosis | 438 | ||
| Genetics | 440 | ||
| Risk Stratification and Therapy | 442 | ||
| Prevention of Sudden Death | 443 | ||
| References | 443 | ||
| Chapter 60: Noncompacted Myocardium | 444 | ||
| Introduction and Definitions | 444 | ||
| Pathology and Pathogenesis | 444 | ||
| Diagnostic Criteria | 444 | ||
| Clinical Presentation (Cardiac and Noncardiac Findings) | 445 | ||
| Genetics | 446 | ||
| Management | 447 | ||
| References | 447 | ||
| Chapter 61: Rheumatic Fever | 449 | ||
| Introduction | 449 | ||
| Pathogenesis | 449 | ||
| Epidemiology | 449 | ||
| Clinical Presentation | 449 | ||
| Pathology | 450 | ||
| Natural History | 450 | ||
| Treatment | 451 | ||
| Conclusion | 452 | ||
| References | 452 | ||
| Chapter 62: Cardiac Tumors | 453 | ||
| Introduction | 453 | ||
| Definition and Morphology | 453 | ||
| Genetics and Epidemiology | 454 | ||
| Early Presentation and Management | 455 | ||
| Late Outcome | 457 | ||
| Arrhythmia and Sudden Cardiac Death | 458 | ||
| References | 458 | ||
| Chapter 63: Marfan Syndrome: A Cardiovascular Perspective | 459 | ||
| Introduction | 459 | ||
| Genetics | 459 | ||
| Clinical Presentation and Natural History | 459 | ||
| Treatment | 459 | ||
| Outpatient Assessment | 462 | ||
| Pregnancy | 463 | ||
| Level of Follow-Up | 463 | ||
| Endocarditis Prophylaxis | 463 | ||
| Exercise | 463 | ||
| References | 463 | ||
| Chapter 64: Idiopathic Pulmonary Arterial Hypertension | 464 | ||
| Introduction | 464 | ||
| Epidemiology | 464 | ||
| Definition and Clinical Classification | 464 | ||
| Pulmonary Vascular Pathophysiology and Genetic Factors | 464 | ||
| Cardiac Pathophysiology | 465 | ||
| Clinical Presentation and Assessment | 466 | ||
| Management Options | 467 | ||
| Pregnancy | 469 | ||
| Level of Follow-Up, Endocarditis Prophylaxis, and Exercise | 469 | ||
| References | 469 | ||
| Selected Terms Used in Adult\nCongenital Heart Disease | 471 | ||
| Acknowledgments | 485 | ||
| Index | 487 |