Diagnosis and Management of Adult Congenital Heart Disease: Expert Consult - Online and Print (Expert Consult) 2nd Edition

Cover......Page 1
Front Matter......Page 2
ISBN    9780702034268......Page 3
Copyright......Page 4
Dedication......Page 5
Contributors......Page 8
References......Page 14
Preface......Page 15
Organization of Care......Page 17
Manpower, Training, and Research......Page 18
References......Page 19
Atrial chambers......Page 20
Ventricles......Page 21
Arrangement of Atrial Chambers......Page 23
Analysis of the Atrioventricular Junction......Page 24
Determination of Morphology \rof the Great Arteries......Page 26
Conclusion......Page 27
References......Page 28
From Phenotype to Genotype......Page 29
Implications of Genetic Test Results......Page 30
Reproductive Decision-Making......Page 31
Molecular Genetic Investigations......Page 32
References......Page 33
The History......Page 34
Physical Appearance......Page 35
Precordial Percussion and Palpation......Page 36
Auscultation......Page 37
The Electrocardiogram......Page 38
The Chest Radiograph......Page 41
References......Page 42
Determination of Type of Defect......Page 43
Associated Anomalies......Page 45
Direction of Flow......Page 46
Morphology......Page 47
Subaortic Stenosis......Page 48
Supravalvular Mitral Ring......Page 49
Right Ventricular Function......Page 50
Common Arterial Trunk (Truncus Arteriosus)......Page 51
Baffle Obstruction/Leak......Page 52
Congenitally Corrected Transposition of the Great Arteries......Page 53
Role of Transesophageal Echocardiography......Page 54
Assessing the Fontan Connections......Page 55
Role of Echocardiography in the Pregnant Woman with Congenital Heart Disease......Page 56
Assessment of Atrioventricular Valve Regurgitation......Page 57
References......Page 58
Ventricular Dysfunction......Page 59
Chronotropic Incompetence......Page 60
Cardiopulmonary Exercise Testing......Page 61
Counteracting neurohormonal activation......Page 63
Exercise Training in Adult Congenital Heart Disease......Page 64
References......Page 66
General Considerations......Page 67
Image Display and Analysis......Page 68
Myocardial infarction or fibrosis studied by late gadolinium imaging......Page 69
Marfan syndrome and other connective tissue disorders......Page 70
Fontan operations for functionally single ventricle......Page 71
Conclusion......Page 72
References......Page 73
Gating......Page 74
Functional assessment of the left and right ventricles......Page 75
Cardiac morphology and extracardiac assessment......Page 76
Coarctation of the Aorta......Page 77
Tetralogy of Fallot......Page 78
Major Aortopulmonary Collateral Arteries......Page 79
The Functionally Univentricular Heart......Page 80
Valvular assessment......Page 81
References......Page 82
Which catheters to use and the sequence of events......Page 83
Guide wires......Page 84
Embolization equipment......Page 85
Endovascular stents......Page 86
Clinical applications......Page 87
Coronary Angiography......Page 88
Biplane angiography......Page 89
Coarctation of the aorta......Page 90
Mustard baffle......Page 92
Bidirectional cavopulmonary connection......Page 93
Pulmonary valve stenosis \nand tetralogy of fallot......Page 94
Three-dimensional angiography......Page 95
Atrial Septal Defect Closure......Page 96
Transcatheter Valve Therapies......Page 97
Look at new and emerging interventions......Page 98
References......Page 99
Special Considerations......Page 101
Primary correction in adult life......Page 102
Conduct of Re-sternotomy......Page 103
Recurrent Coarctation......Page 104
References......Page 105
Unidirectional Glenn Shunt......Page 106
Bidirectional Glenn Shunt......Page 107
Systemic Venous Pathway Obstruction......Page 108
Therapeutic Options......Page 109
Results of Fontan Conversion, Arrhythmia Surgery, and Repair of Associated Lesions......Page 111
Conclusion......Page 115
Common causes of hypoxemia\r......Page 119
Arrhythmias......Page 121
Hepatic Dysfunction......Page 122
Exercise Tolerance and Quality of Life......Page 123
References......Page 124
Anatomic/Physiologic Considerations......Page 125
Choice of Procedure......Page 126
Cardiac Transplantation......Page 127
Lung and Heart-Lung Transplantation......Page 128
References......Page 129
Comorbid Conditions......Page 130
Antiplatelet Agents......Page 131
Obstructive Valvular Disease......Page 132
Intraoperative Monitoring......Page 133
Postoperative Management......Page 134
References......Page 135
Infective Endocarditis Prophylaxis......Page 136
Blood Transfusion......Page 137
Other Factors Influencing Pulmonary Vascular Resistance in Critically III Patients......Page 138
References......Page 139
Pacemaker Issues......Page 141
Pacemaker Therapy......Page 142
Accessory Atrioventricular Pathways......Page 143
Risk Assessment......Page 144
Treatment Options......Page 145
References......Page 146
Typical Inter-atrial Reentry Tachycardia in Patients with Atrial Septal Defects......Page 147
Typical Atrial Arrhythmias in Patients with Tetralogy of Fallot......Page 148
Typical Atrial Arrhythmias in Patients with Surgically Corrected Transposition of the Great Arteries (Mustard or Senning)......Page 149
Devices in Adult Congenital Heart Disease......Page 150
Resynchronization Therapy......Page 151
References......Page 152
Defining Heart Failure in Adult Congenital Heart Disease......Page 153
Heart Failure: Subpulmonary Right Ventricle......Page 154
Congenitally Corrected Transposition of the Great Arteries......Page 157
d-Transposition of the Great Arteries......Page 158
Heart Failure: Single-Ventricle \rPhysiology......Page 159
References......Page 160
Open-Heart Surgery and Noncardiac Surgery......Page 162
Clinical Manifestations......Page 163
Echocardiography......Page 164
Diagnosis......Page 166
Prophylaxis......Page 167
References......Page 168
Key Elements of an Ideal Transition Program......Page 169
Congenital Heart Disease Curriculum......Page 170
Types of Services Needed in the Adult Congenital Heart Disease Environment......Page 171
References......Page 172
Cardiac Contraindications to Pregnancy......Page 173
Management of the Pregnant Patient with Cardiac Disease......Page 174
Percutaneous Procedures During \nPregnancy......Page 175
Fetal Risks......Page 176
Ventricular Tachycardia......Page 177
Contraception......Page 178
References......Page 179
Anesthesia for Cesarean Section and Other Procedures in Pregnant Patients with Congenital Heart Disease......Page 180
References......Page 182
General Anesthesia for Cardiac Surgery......Page 183
Arrhythmias......Page 184
References......Page 185
Mortality and the Underwriting Process......Page 186
Mortality Ratios for Adults with Repaired Congenital Heart Lesions......Page 187
Transposition of Great Arteries and Atrial Procedures (Mustard and Senning)......Page 188
Limitations to Existing Long-Term Mortality Data......Page 189
Conclusion......Page 190
References......Page 191
Transcatheter Closure......Page 195
Survival and Functional Status......Page 196
Operated Patients......Page 197
Exercise Oxygen Saturation......Page 198
Late Intervention......Page 199
Late Re-intervention......Page 200
Level of Follow-Up, Endocarditis Prophylaxis, and Exercise......Page 201
References......Page 202
Pathophysiology......Page 203
Early Clinical Presentation......Page 204
Catheter Closure......Page 205
Operated Patients......Page 206
Physical Examination......Page 207
Echocardiography......Page 208
Management......Page 209
References......Page 210
Atrioventricular Conduction Tissue......Page 211
Early Presentation......Page 212
Echocardiographic Goals and Features......Page 214
Unoperated Patients......Page 215
Arrhythmia and Sudden Cardiac Death......Page 216
Level of Follow-up, Endocarditis Prophylaxis, and Exercise......Page 217
References......Page 218
Late Presentation......Page 221
Diagnosis......Page 222
Late Outcome......Page 223
Adult Presentation......Page 224
References......Page 225
Early Presentation\r......Page 227
\rLate Outcome......Page 229
References......Page 230
Genetics and Epidemiology......Page 231
Operated Patients......Page 233
Chest Radiography......Page 234
Cardiac Catheterization......Page 235
References......Page 236
Genetics and Molecular Biology\r......Page 239
Pathophysiology and Clinical Course (Without Intervention)\r......Page 240
Chest Radiography......Page 241
Echocardiography......Page 242
Management\r......Page 243
Medical Management......Page 244
Surgical Options......Page 245
Transcatheter Aortic Valve Implantation......Page 246
Endocarditis......Page 248
Outcomes and Follow-Up\r......Page 249
References......Page 250
Outpatient Assessment\r......Page 251
Management\r......Page 252
Associated Lesions\r......Page 253
Late Management\r......Page 254
References......Page 256
Pathophysiology\r......Page 258
Early Presentation and Management\r......Page 259
Outpatient Management......Page 260
Outcome After Surgery......Page 261
Arrhythmia and Sudden Death......Page 262
References......Page 263
Imaging......Page 265
Ruptured Sinus of Valsalva......Page 266
Pregnancy\r......Page 267
References......Page 268
Patients with Repaired Patent \nDuctus Arteriosus......Page 271
Closure......Page 272
Definition and Morphology\r......Page 273
Early Presentation\r......Page 274
References......Page 275
Surgical Treatment......Page 276
Interventional Treatment......Page 277
Arterial Hypertension......Page 278
Aneurysms of the Ascending Aorta or \nin the Region of the Aortic Isthmus......Page 279
Physical Findings......Page 280
Echocardiography......Page 281
Surgical or Catheter Interventional Treatment......Page 282
Genetics and Epidemiology\r......Page 283
Level of Follow-Up, Endocarditis Prophylaxis, and Exercise\r......Page 284
References......Page 285
Truncus Arteriosus......Page 286
Early Presentation\r and Management......Page 287
Surgical History\r......Page 288
Outpatient Assessment\r......Page 289
Pregnancy\r......Page 290
References......Page 291
Right Aortic Arch with Aberrant Left Subclavian Artery......Page 292
Cervical Arch......Page 293
Absent Pulmonary Valve Syndrome......Page 294
Postoperative Airway Compression......Page 295
Examination......Page 296
Computed Tomography and Magnetic Resonance Imaging......Page 297
Relief of Native Anatomic Anomalies......Page 298
Level of Follow-Up, Endocarditis Prophylaxis, and Exercise\r......Page 299
References......Page 300
Outpatient Assessment\r......Page 303
Late Management\r......Page 304
Tricuspid Valve Replacement......Page 305
1.5-Ventricle Repair......Page 306
Plication of the Atrialized Right Ventricle......Page 307
References......Page 308
Early Presentation and Management\r......Page 310
Survival and Functional Status......Page 311
Unoperated Patients......Page 312
Role of Echocardiography......Page 313
Late Intervention......Page 314
Pregnancy, Exercise, and Endocarditis Prophylaxis......Page 315
References......Page 316
Physical Examination......Page 319
Echocardiography......Page 320
Indications for Intervention......Page 321
References......Page 322
Early Presentation and Management\r......Page 323
Patients with Repaired Double-Chambered Right Ventricle......Page 325
Indications......Page 326
Pregnancy\r......Page 327
References......Page 328
Genetics and Epidemiology\r......Page 331
Early Presentation and Management\r......Page 332
Patients with Repaired Tetralogy of Fallot......Page 333
Palliated-Only or Unoperated Patients......Page 337
Surgical/Catheter Interventional Options......Page 338
Sustained Monomorphic Ventricular Tachycardia......Page 339
Sudden Cardiac Death and Risk Stratification......Page 340
Level of Follow-Up......Page 341
References......Page 342
Early Presentation\r......Page 343
Management\r......Page 345
Long-Term Outcome\r......Page 346
Outpatient Assessment\r......Page 347
Intervention......Page 348
Pregnancy\r......Page 349
References......Page 350
Management and Outcome\r......Page 351
Clinical Presentation and Diagnosis\r......Page 352
References......Page 353
Early Presentation and Management\r......Page 354
Univentricular Strategy......Page 356
Univentricular Repair......Page 357
Outpatient Assessment\r......Page 358
Univentricular Repair......Page 359
Arrhythmia and Sudden Cardiac Death\r......Page 360
References......Page 361
Genetics and Epidemiology\r......Page 362
Mustard and Senning Operations......Page 363
Arterial Switch Operation......Page 364
Survival and Functional Status......Page 365
Ventricular Function......Page 367
Ventricular Function......Page 368
Atrial baffle Repair and Rastelli Operation Patients......Page 369
Arterial Switch Conversion......Page 370
Mustard and Senning Operation Patients......Page 371
References......Page 372
Pulmonary Vascular Pathology......Page 373
Genetics and Epidemiology\r......Page 374
Survival and Functional Class......Page 375
Secondary Erythrocytosis......Page 376
Outpatient Assessment\r......Page 377
Phlebotomy......Page 380
Ischemic Complications......Page 381
Oxygen Therapy......Page 382
Ventricular Arrhythmias......Page 383
References......Page 384
Congestive Heart Failure......Page 386
Classic Repairs\r......Page 387
Unoperated Patients......Page 388
Echocardiography......Page 389
Cardiac Magnetic Resonance......Page 390
Pregnancy\r......Page 391
References......Page 392
Classification\r......Page 393
Subpulmonary Ventricular Septal Defect......Page 394
Indications for Repair and Preoperative Evaluation\r......Page 395
Doubly Committed Ventricular Septal Defect......Page 396
Operated Patients......Page 397
Conclusion\r......Page 398
References......Page 399
Management......Page 401
Late Intervention......Page 403
References......Page 404
Operations......Page 405
Staged Surgical Management......Page 406
Late Outcome......Page 408
Post-Fontan or Post–Total Cavopulmonary Connection Patients with Tricuspid Atresia or Hypoplastic Left Heart Syndrome......Page 409
Contraception......Page 410
References......Page 411
Atrioventricular Junctions......Page 412
Epidemiology and Genetics......Page 413
Late Presentation and Outcomes......Page 414
Echocardiography......Page 415
Level of Follow-Up and Endocarditis Prophylaxis......Page 416
References......Page 417
Left Coronary Artery Arising from Right Sinus of Valsalva......Page 419
Right Coronary Artery Arising from the Left Sinus of Valsalva......Page 420
Anomalous Origin of a Coronary Artery from the Pulmonary Artery......Page 421
Diagnosis......Page 423
Tetralogy of Fallot......Page 424
References......Page 425
Adult-Onset Kawasaki Disease......Page 426
Patients with Large and Giant Coronary Artery Aneurysms or After Coronary Artery Occlusion......Page 427
Outpatient Assessment of the Adult with Kawasaki Disease......Page 428
Pregnancy......Page 429
References......Page 430
Pathogenesis......Page 433
Clinical Presentation and Management......Page 434
Evidence of Myocardial Inflammation and Infection......Page 435
Emerging Role of Cardiovascular Magnetic Resonance......Page 436
Pregnancy......Page 437
References......Page 439
Genetics......Page 440
Echocardiography......Page 441
Late Outcomes......Page 442
Sudden Cardiac Death......Page 443
References......Page 444
Pathophysiology......Page 445
Past Medical History......Page 446
Two-Dimensional and Doppler Echocardiography......Page 447
Computed Tomography and Magnetic Resonance Imaging......Page 448
Cardiac Catheterization......Page 449
Endomyocardial Biopsy......Page 450
Level of Follow-Up, Endocarditis Prophylaxis, and Exercise......Page 451
References......Page 452
Clinical and Differential Diagnosis......Page 453
Genetics......Page 455
Risk Stratification and Therapy......Page 457
References......Page 458
Diagnostic Criteria......Page 459
Clinical Presentation (Cardiac and Noncardiac Findings)......Page 460
Genetics......Page 461
References......Page 462
Diagnostic Criteria......Page 464
Natural History......Page 465
Inflammatory Manifestations......Page 466
References......Page 467
Malignant Tumors......Page 468
Genetics and Epidemiology......Page 469
Early Presentation and Management......Page 470
Assessment of Patient with Suspected Cardiac Tumor......Page 472
References......Page 473
Medical Treatment......Page 474
Surgical Treatment......Page 475
Late Outcome......Page 477
References......Page 478
Pulmonary Vascular Pathophysiology and Genetic Factors......Page 479
Cardiac Pathophysiology......Page 480
Chest Radiography......Page 481
Medical Treatment......Page 482
References......Page 484
Selected Terms Used in Adult\rCongenital Heart Disease......Page 486
Acknowledgments\r......Page 500
A......Page 502
C......Page 505
D......Page 508
E......Page 509
F......Page 511
H......Page 512
L......Page 513
M......Page 514
N......Page 515
P......Page 516
S......Page 519
T......Page 521
Z......Page 523