BOOK
Diagnosis and Management of Adult Congenital Heart Disease E-Book
Michael A. Gatzoulis | Gary D. Webb | Piers E. F. Daubeney
(2017)
Additional Information
Book Details
Abstract
Designed to meet the needs of clinicians working with adults with congenital heart disease, Diagnosis and Management of Adult Congenital Heart Disease , by Drs. Michael A. Gatzoulis, Gary D. Webb, and Piers E. F. Daubeney, offers essential guidance on the anatomical issues, clinical presentation, diagnosis, and treatment options available to practitioners today. This latest edition features completely updated content, including new information for nurses and nurse practitioners who, now more than ever, are playing an important role in the care of adults with CHD. You'll also access four new chapters, illustrated congenital defects, coverage of long-term outcomes, and much more.
- Drs. Gatzoulis, Webb, and Daubeney lead a team of experts ideally positioned to provide state-of-the-art global coverage of this increasingly important topic.
- Each disease-oriented chapter is written to a highly structured template and provides key information on incidence, genetics, morphology, presentation, investigation and imaging, and treatment and intervention.
- Congenital defects are illustrated with full-color line drawings and by the appropriate imaging modality (for example, EKG, x-ray, echocardiogram, MRI, CT, ).
- Provides coverage of long-term outcomes, including the management of pregnant patients and patients undergoing non-cardiac surgery.
- Features the addition of four new chapters : A Historic Perspective; Quality of Life in Patients with Pulmonary Hypertension; Psychosocial Issues in ACHD; Supportive and Palliative Care for End-Stage ACHD.
Table of Contents
| Section Title | Page | Action | Price |
|---|---|---|---|
| Front Cover | Cover | ||
| Diagnosis and Management of Adult Congenital Heart Disease | i | ||
| Copyright | ii | ||
| Dedication | iii | ||
| CONTRIBUTORS | iv | ||
| FOREWORD | xi | ||
| PREFACE | xii | ||
| CONTENTS | xiii | ||
| I - General Principles | 1 | ||
| 1 - ADULTS WITH CONGENITAL HEART DISEASE: A GROWING POPULATION | 2 | ||
| Global Estimates of Incidence and Birth Prevalence of Congenital Heart Disease | 2 | ||
| Changes in Mortality, Survival, and Life Expectancy in the Congenital Heart Disease Population | 2 | ||
| Prevalence and Numbers of Adult Congenital Heart Disease | 4 | ||
| ORGANIZATION OF QUALITY-DRIVEN CARE | 4 | ||
| Targeting Disease Burden and Mortality | 4 | ||
| Impact of Specialized Adult Congenital Heart Disease Care | 5 | ||
| Manpower, Training, Education, and Research | 7 | ||
| Conclusions | 8 | ||
| REFERENCES | 8 | ||
| 2 - Grown-Up Congenital Heart or Adult Congenital Heart Disease: Historical Perspectives | 10 | ||
| 3 - Cardiac Morphology and Nomenclature | 13 | ||
| Sequential Segmental Analysis: General Philosophy | 13 | ||
| Morphology of the Cardiac Chambers | 13 | ||
| ATRIAL CHAMBERS | 13 | ||
| VENTRICLES | 14 | ||
| GREAT ARTERIES | 16 | ||
| Arrangement of Atrial Chambers | 17 | ||
| Determination of Ventricular Morphology and Topology | 17 | ||
| Analysis of the Atrioventricular Junction | 18 | ||
| Determination of Morphology of the Great Arteries | 20 | ||
| Analysis of the Ventriculoarterial Junction | 20 | ||
| Associated Malformations | 21 | ||
| Location of the Heart | 21 | ||
| Conclusion | 22 | ||
| REFERENCES | 22 | ||
| 4 - Adults With Congenital Heart Disease: AÂ Genetic Perspective | 24 | ||
| Genetic Basis of Congenital Heart Disease | 24 | ||
| Genetics in the Adult Congenital Heart Disease Population | 24 | ||
| Assessment by the Adult Congenital Heart Disease Clinician | 25 | ||
| ACCESS TO GENETICS EXPERTISE IN ADULT CONGENITAL HEART DISEASE CLINICS | 26 | ||
| Genetic Testing | 26 | ||
| TYPES OF TESTING | 26 | ||
| WHEN TO OFFER TESTING | 28 | ||
| PREPARING THE PATIENT FOR GENETIC TESTING | 28 | ||
| IMPLICATIONS OF GENETIC TEST RESULTS | 28 | ||
| IMPLICATIONS FOR FAMILY MEMBERS | 29 | ||
| Recurrence Risk of Congenital Heart Disease | 29 | ||
| REFERENCES | 30 | ||
| 5 - Clinical Assessment | 31 | ||
| Medical History | 31 | ||
| ANATOMIC DIAGNOSIS | 31 | ||
| SURGICAL/INTERVENTIONAL TREATMENT | 31 | ||
| COMMON SEQUELA POST INTERVENTION | 32 | ||
| SYMPTOMATOLOGY | 32 | ||
| Physical Examination | 32 | ||
| PHYSICAL APPEARANCE | 32 | ||
| ARTERIAL PULSE | 32 | ||
| VEINS: JUGULAR AND PERIPHERAL | 33 | ||
| CHEST INSPECTION | 33 | ||
| PRECORDIAL PERCUSSION AND PALPATION | 33 | ||
| AUSCULTATION | 33 | ||
| Electrocardiogram | 35 | ||
| Chest Radiograph | 37 | ||
| Conclusion | 40 | ||
| REFERENCES | 40 | ||
| 6 - Echocardiography | 41 | ||
| Sequential Segmental Analysis | 41 | ||
| Echocardiography in Specific Lesions | 41 | ||
| ATRIAL SEPTAL DEFECTS | 41 | ||
| Anatomy and Physiology | 41 | ||
| Associated Lesions | 41 | ||
| Transthoracic Echocardiography in Patients With Unrepaired Atrial Septal Defects | 41 | ||
| Contrast Echo | 43 | ||
| Transesophageal Echocardiography During the Surgical and Interventional Closure of Atrial Septal Defects | 43 | ||
| ATRIOVENTRICULAR SEPTAL DEFECT | 43 | ||
| Anatomy and Physiology | 43 | ||
| Associated Anomalies | 44 | ||
| Transthoracic Echocardiography in Unoperated Patients | 44 | ||
| Transesophageal Echocardiography | 45 | ||
| Three-Dimensional Transthoracic Echocardiography | 45 | ||
| Transthoracic Echocardiography in Operated Patients | 45 | ||
| VENTRICULAR SEPTAL DEFECTS | 45 | ||
| Anatomy and Physiology | 45 | ||
| Associated Lesions | 46 | ||
| Transthoracic Echocardiography in Unoperated Patients | 47 | ||
| CLOSURE OF VENTRICULAR SEPTAL DEFECTS | 48 | ||
| Transthoracic Echocardiography in Operated Patients | 48 | ||
| Three Dimensional Echocardiography | 48 | ||
| PATENT DUCTUS ARTERIOSUS | 49 | ||
| Anatomy and Physiology | 49 | ||
| Transthoracic Echocardiography in Unoperated Patients | 49 | ||
| Transthoracic Echocardiography in Operated Patients | 50 | ||
| Special Consideration | 50 | ||
| Three-Dimensional Echocardiography | 50 | ||
| COARCTATION OF THE AORTA | 50 | ||
| Anatomy and Physiology | 50 | ||
| Associated Lesions | 50 | ||
| Transthoracic Echocardiography in Unoperated Patients | 50 | ||
| Surgical Treatment | 51 | ||
| Interventional Treatment | 51 | ||
| Transthoracic Echocardiography After Repair | 52 | ||
| Three-Dimensional Echocardiography | 52 | ||
| PULMONARY STENOSIS | 52 | ||
| II - Septal Defects | 305 | ||
| 29 - Atrial Septal Defect (Interatrial Communication) | 306 | ||
| Definition and Morphology | 306 | ||
| Genetics and Epidemiology | 306 | ||
| Early Presentation and Management | 307 | ||
| TRANSCATHETER CLOSURE | 307 | ||
| Late Outcome | 308 | ||
| SURVIVAL AND FUNCTIONAL STATUS | 308 | ||
| LATE COMPLICATIONS | 308 | ||
| Outpatient Assessment | 309 | ||
| OPERATED PATIENTS | 309 | ||
| UNOPERATED PATIENTS | 309 | ||
| History and Clinical Examination | 309 | ||
| Pulse Oximetry | 310 | ||
| Electrocardiography | 310 | ||
| Chest Radiography | 310 | ||
| Echocardiography | 310 | ||
| Cardiac Catheterization | 310 | ||
| Magnetic Resonance Imaging | 311 | ||
| Computed Tomographic Angiography | 312 | ||
| Exercise Oxygen Saturation | 312 | ||
| Open-Lung Biopsy | 312 | ||
| Additional Tests | 312 | ||
| Late Management Options | 312 | ||
| LATE INTERVENTION | 312 | ||
| SURGICAL OUTCOMES | 313 | ||
| DEVICE CLOSURE | 313 | ||
| LATE REINTERVENTION | 313 | ||
| Arrhythmia and Sudden Cardiac Death | 313 | ||
| Pregnancy | 313 | ||
| Level of Follow-Up, Endocarditis Prophylaxis, and Exercise | 314 | ||
| REFERENCES | 314 | ||
| 30 - Ventricular Septal Defect | 316 | ||
| Definition and Morphology | 316 | ||
| Associated Abnormalities | 316 | ||
| Prevalence and Genetic Factors | 316 | ||
| Pathophysiology | 316 | ||
| Early Clinical Presentation | 317 | ||
| Management in Childhood | 318 | ||
| MEDICAL THERAPY AND THE SURGICAL CLOSURE OF VENTRICULAR SEPTAL DEFECTS | 318 | ||
| TRANSCATHETER CLOSURE | 318 | ||
| Late Outcome and Complications | 319 | ||
| UNOPERATED PATIENTS | 319 | ||
| OPERATED PATIENTS | 319 | ||
| Outpatient Assessment of the Adult Patient | 320 | ||
| PHYSICAL EXAMINATION | 320 | ||
| ELECTROCARDIOGRAPHY | 322 | ||
| CHEST X-RAY | 322 | ||
| ECHOCARDIOGRAPHY | 322 | ||
| CARDIAC MAGNETIC RESONANCE IMAGING | 322 | ||
| CARDIAC CATHETERIZATION | 322 | ||
| Management of the Adult Patient | 323 | ||
| PREGNANCY | 323 | ||
| PHYSICAL ACTIVITY | 323 | ||
| LEVEL OF SURVEILLANCE | 324 | ||
| INFECTIVE ENDOCARDITIS AND ENDOCARDITIS PROPHYLAXIS | 324 | ||
| REFERENCES | 325 | ||
| 31 - Atrioventricular Septal Defects | 326 | ||
| Definition and Morphology | 326 | ||
| DEFINITIONS | 326 | ||
| MORPHOLOGY | 326 | ||
| International Pediatric and Congenital Cardiac Code Classification | 326 | ||
| Rastelli Classification | 326 | ||
| Atrioventricular Valves, Left Ventricle Outflow Tract Anatomy, and Other Anatomical Characteristics | 327 | ||
| ASSOCIATED INTRACARDIAC AND EXTRACARDIAC ANOMALIES OF ATRIOVENTRICULAR SEPTAL DEFECTS | 328 | ||
| Atrioventricular Conduction Tissue | 329 | ||
| Embryology, Epidemiology, and Genetics/Maternal Exposure | 329 | ||
| EMBRYOLOGY | 329 | ||
| EPIDEMIOLOGY | 330 | ||
| GENETICS AND MATERNAL EXPOSURE | 331 | ||
| Early Presentation | 331 | ||
| PRENATAL DIAGNOSIS | 331 | ||
| POSTNATAL DIAGNOSIS | 332 | ||
| Physical Exam | 333 | ||
| Electrocardiography | 333 | ||
| Chest X-Ray | 333 | ||
| 3D Echo and Transesophageal Echo | 334 | ||
| Cardiac Computed Tomography | 334 | ||
| Cardiac Catheterization and Angiography | 335 | ||
| SURGICAL MANAGEMENT OF ATRIOVENTRICULAR SEPTAL DEFECTS | 335 | ||
| LATE OUTCOMES | 336 | ||
| Outpatient Assessment of the Adult with Atrioventricular Septal Defect | 336 | ||
| UNOPERATED PATIENTS | 336 | ||
| OPERATED PATIENTS | 337 | ||
| PREGNANCY MANAGEMENT | 337 | ||
| ENDOCARDITIS PROPHYLAXIS | 337 | ||
| REFERENCES | 337 | ||
| III - Diseases of the Mitral Valve | 339 | ||
| 32 - Cor Triatriatum and Congenital Mitral Stenosis | 340 | ||
| Introduction | 340 | ||
| Cor Triatriatum | 340 | ||
| DEFINITION AND EPIDEMIOLOGY | 340 | ||
| EMBRYOLOGY AND ANATOMY | 340 | ||
| Anatomic Variations and Classification | 340 | ||
| NATURAL HISTORY AND PRESENTATION | 340 | ||
| Presentation in Childhood | 340 | ||
| Adult Presentation | 341 | ||
| SYNDROMES AND ASSOCIATED DEFECTS | 341 | ||
| DIAGNOSIS | 341 | ||
| Examination | 341 | ||
| Electrocardiogram | 341 | ||
| Chest X-Ray | 341 | ||
| Echocardiogram | 341 | ||
| Computed Tomography and Magnetic Resonance Imaging | 343 | ||
| Exercise Testing | 343 | ||
| Cardiac Catheterization | 343 | ||
| MANAGEMENT | 343 | ||
| SPECIAL SITUATIONS | 343 | ||
| Pregnancy | 343 | ||
| Endocarditis Prophylaxis and Exercise Recommendations | 344 | ||
| FOLLOW UP AND OUTCOME | 344 | ||
| Congenital Mitral Stenosis | 344 | ||
| DEFINITION AND EPIDEMIOLOGY | 344 | ||
| ANATOMY AND VARIATIONS | 344 | ||
| PRESENTATION | 345 | ||
| Presentation in Childhood | 345 | ||
| Adult Presentation | 345 | ||
| SYNDROMES AND ASSOCIATED DEFECTS | 345 | ||
| DIAGNOSIS | 345 | ||
| Electrocardiogram | 345 | ||
| Chest X-Ray | 345 | ||
| Echocardiogram | 345 | ||
| Computed Tomography and Magnetic Resonance Imaging | 346 | ||
| Cardiac Catheterization | 346 | ||
| MANAGEMENT | 346 | ||
| Presentation in Childhood | 346 | ||
| Adult Presentation | 346 | ||
| Outcome and Prognosis | 346 | ||
| SPECIAL SITUATIONS | 346 | ||
| Pregnancy | 346 | ||
| Endocarditis Prophylaxis, Follow-up, and Exercise | 346 | ||
| REFERENCES | 346 | ||
| 33 - Mitral Valve Prolapse, Mitral Regurgitation | 348 | ||
| Anatomy of the Mitral Valve | 348 | ||
| Definition, Etiology, and Pathology of Mitral Valve Prolapse | 348 | ||
| Prevalence of Mitral Valve Prolapse | 349 | ||
| Early Presentation of Mitral Valve Prolapse | 349 | ||
| Management of Mitral Valve Prolapse | 350 | ||
| Late Outcome of Mitral Valve Prolapse | 350 | ||
| Late Management Options | 351 | ||
| Pregnancy | 351 | ||
| Exercise and Mitral Valve Prolapse | 352 | ||
| Conclusions | 352 | ||
| REFERENCES | 352 | ||
| 34 - Partial Anomalous Pulmonary Venous Connections and the Scimitar Syndrome | 354 | ||
| Definition and Morphology | 354 | ||
| Scimitar Syndrome | 355 | ||
| Genetics and Epidemiology | 355 | ||
| Early Presentation and Management | 355 | ||
| Late Outcome | 357 | ||
| OPERATED PATIENTS | 357 | ||
| IV - Diseases of the Left Ventricular Outflow Tract | 363 | ||
| 35 - Valvular Aortic Stenosis | 364 | ||
| Definition, Morphology, and Epidemiology | 364 | ||
| Associated Lesions | 364 | ||
| Genetics and Molecular Biology | 364 | ||
| Pathophysiology and Clinical Course (Without Intervention) | 366 | ||
| Physical Examination | 366 | ||
| Investigations | 367 | ||
| BRAIN NATRIURETIC PEPTIDE | 367 | ||
| ELECTROCARDIOGRAPHY | 367 | ||
| CHEST RADIOGRAPHY | 367 | ||
| ECHOCARDIOGRAPHY | 367 | ||
| COMPUTED TOMOGRAPHY AND MAGNETIC RESONANCE IMAGING | 368 | ||
| CARDIAC CATHETERIZATION | 369 | ||
| STRESS TESTING | 369 | ||
| Management | 370 | ||
| MEDICAL MANAGEMENT | 371 | ||
| BALLOON AORTIC VALVULOPLASTY | 371 | ||
| SURGERY | 372 | ||
| Timing | 372 | ||
| Surgical Options | 372 | ||
| Transcatheter Aortic Valve Implantation | 373 | ||
| PREGNANCY | 374 | ||
| ENDOCARDITIS | 375 | ||
| EXERCISE | 376 | ||
| Aortic Root Dilation | 376 | ||
| Aortic Stenosis | 376 | ||
| Outcomes and Follow-Up | 376 | ||
| REFERENCES | 377 | ||
| 36 - Subvalvular and Supravalvular Aortic Stenosis | 379 | ||
| Subvalvular Aortic Stenosis | 379 | ||
| DEFINITION AND MORPHOLOGY | 379 | ||
| ASSOCIATED LESIONS | 379 | ||
| GENETICS AND ETIOLOGY | 379 | ||
| EARLY PRESENTATION AND MANAGEMENT | 379 | ||
| OUTPATIENT ASSESSMENT | 380 | ||
| MANAGEMENT | 380 | ||
| LATE OUTCOMES AND COMPLICATIONS | 381 | ||
| LEVEL OF FOLLOW-UP | 382 | ||
| Supravalvular Aortic Stenosis | 382 | ||
| DEFINITION AND MORPHOLOGY | 382 | ||
| ASSOCIATED LESIONS | 382 | ||
| GENETICS AND ETIOLOGY | 382 | ||
| OUTPATIENT ASSESSMENT | 383 | ||
| LATE MANAGEMENT | 383 | ||
| LATE OUTCOMES | 384 | ||
| LEVEL OF FOLLOW-UP | 385 | ||
| Pregnancy in the Presence of Left Ventricular Outflow Tract Obstruction | 385 | ||
| REFERENCES | 385 | ||
| 37 - Aortic Regurgitation | 387 | ||
| Etiology | 387 | ||
| Primary Aortic Valve Abnormality | 387 | ||
| Aortic Root Pathology | 387 | ||
| Aortic Regurgitation Associated With Other Congenital Lesions | 387 | ||
| Acquired Aortic Regurgitation | 387 | ||
| Clinical and Natural Course in Children with Aortic Regurgitation | 387 | ||
| Acute Aortic Regurgitation | 388 | ||
| Chronic Aortic Regurgitation | 388 | ||
| PATHOPHYSIOLOGY | 388 | ||
| CLINICAL PRESENTATION | 388 | ||
| History | 388 | ||
| Physical Examination | 388 | ||
| Chest Radiography | 389 | ||
| Echocardiography | 389 | ||
| Cardiac Catheterization | 390 | ||
| Magnetic Resonance Imaging | 390 | ||
| Exercise Testing | 390 | ||
| Stages of Chronic Aortic Regurgitation | 390 | ||
| Disease Progression Considerations | 390 | ||
| Outpatient Management | 390 | ||
| Vasodilator Therapy | 390 | ||
| Timing of Surgery | 390 | ||
| Surgical Options | 392 | ||
| Transcatheter Aortic Valve Replacement for Aortic Regurgitation | 392 | ||
| Outcomes After Surgery | 393 | ||
| Pregnancy | 393 | ||
| Endocarditis Prophylaxis | 393 | ||
| Exercise | 393 | ||
| REFERENCES | 394 | ||
| 38 - Sinus of Valsalva Aneurysms | 395 | ||
| Introduction | 395 | ||
| Morphology | 395 | ||
| Associated Defects | 395 | ||
| Epidemiology | 395 | ||
| Investigations | 396 | ||
| ELECTROCARDIOGRAPHY AND CHEST RADIOGRAPHY | 396 | ||
| IMAGING | 396 | ||
| Presentation | 396 | ||
| UNRUPTURED SINUS OF VALSALVA | 396 | ||
| RUPTURED SINUS OF VALSALVA | 397 | ||
| Management | 397 | ||
| RUPTURED SINUS OF VALSALVA ANEURYSM | 397 | ||
| Reoperation | 398 | ||
| NONRUPTURED ANEURYSMS | 398 | ||
| Outpatient Assessment | 398 | ||
| Arrhythmia and Sudden Death | 398 | ||
| Pregnancy | 398 | ||
| Level of Follow-Up and Endocarditis Prophylaxis | 398 | ||
| REFERENCES | 399 | ||
| V - Diseases of the Aorta | 401 | ||
| 39 - Patent Ductus Arteriosus and Aortopulmonary Window | 402 | ||
| Patent Ductus Arteriosus | 402 | ||
| Embryology | 402 | ||
| Incidence and Classification | 402 | ||
| Late Outcome | 402 | ||
| Outpatient Assessment | 403 | ||
| PATIENTS WITH REPAIRED PATENT DUCTUS ARTERIOSUS | 403 | ||
| PATIENT WITH UNREPAIRED OR RESIDUAL PATENT DUCTUS ARTERIOSUS | 403 | ||
| CLINICAL EXAMINATION | 404 | ||
| ELECTROCARDIOGRAPHY | 404 | ||
| CHEST RADIOGRAPHY | 404 | ||
| ECHOCARDIOGRAPHY | 404 | ||
| CARDIAC CATHETERIZATION | 404 | ||
| COMPUTED TOPOGRAPHY AND CARDIAC MAGNETIC RESONANCE | 404 | ||
| Late Management Options | 404 | ||
| INDICATIONS FOR CLOSURE | 404 | ||
| CATHETER INTERVENTION (DEVICE CLOSURE) | 405 | ||
| SURGICAL CLOSURE | 405 | ||
| Pregnancy | 405 | ||
| Level of Follow-Up, Endocarditis Prophylaxis, and Exercise | 406 | ||
| Aortopulmonary Window | 406 | ||
| Definition and Morphology | 406 | ||
| Associated Lesions | 406 | ||
| Early Presentation | 407 | ||
| Late Presentation | 407 | ||
| Outpatient Assessment and Management | 408 | ||
| REFERENCES | 408 | ||
| 40 - Aortic Coarctation and Interrupted Aortic Arch | 409 | ||
| Coarctation of the Aorta | 409 | ||
| DEFINITION AND MORPHOLOGY | 409 | ||
| GENETICS AND EPIDEMIOLOGY | 409 | ||
| EARLY PRESENTATION | 409 | ||
| MANAGEMENT | 410 | ||
| Surgical Treatment | 410 | ||
| Interventional Treatment | 410 | ||
| LATE OUTCOME | 410 | ||
| Survival and Functional Status | 410 | ||
| Late Complications | 411 | ||
| Arterial Hypertension | 411 | ||
| Re-coarctation or Residual Coarctation | 412 | ||
| Aneurysms of the Ascending Aorta or in the Region of the Aortic Isthmus | 412 | ||
| Infective Endocarditis or Endarteritis | 413 | ||
| Bicuspid Aortic Valve | 413 | ||
| Aneurysms of the Circle of Willis | 413 | ||
| Coronary Artery Disease | 414 | ||
| Elevation of the Left Shoulder | 414 | ||
| OUTPATIENT ASSESSMENT | 414 | ||
| Physical Findings | 414 | ||
| Electrocardiography | 414 | ||
| Chest Radiography | 414 | ||
| Echocardiography | 415 | ||
| Magnetic Resonance Imaging and Computed Tomography | 415 | ||
| Cardiac Catheterization | 416 | ||
| LATE MANAGEMENT OPTIONS | 416 | ||
| Medical Intervention | 416 | ||
| Surgical or Catheter Interventional Treatment | 416 | ||
| PREGNANCY | 417 | ||
| LEVEL OF FOLLOW-UP, ENDOCARDITIS PROPHYLAXIS, AND EXERCISE | 417 | ||
| Interrupted Aortic Arch | 417 | ||
| DEFINITION AND MORPHOLOGY | 417 | ||
| GENETICS AND EPIDEMIOLOGY | 417 | ||
| EARLY PRESENTATION | 417 | ||
| MANAGEMENT | 418 | ||
| Medical Treatment | 418 | ||
| Surgical Treatment | 418 | ||
| Transcatheter Treatment | 418 | ||
| LATE OUTCOME | 418 | ||
| Survival and Functional Status | 418 | ||
| Late Complications | 418 | ||
| OUTPATIENT ASSESSMENT | 418 | ||
| Physical Findings | 418 | ||
| Electrocardiography | 418 | ||
| Chest Radiography | 418 | ||
| Echocardiography | 418 | ||
| Magnetic Resonance Imaging and Computed Tomography | 419 | ||
| Cardiac Catheterization | 419 | ||
| LATE MANAGEMENT OPTIONS | 419 | ||
| LEVEL OF FOLLOW-UP, ENDOCARDITIS PROPHYLAXIS, AND EXERCISE | 419 | ||
| REFERENCES | 419 | ||
| 41 - Truncus Arteriosus | 421 | ||
| Definition and Morphology | 421 | ||
| TRUNCAL VALVE | 421 | ||
| VENTRICULAR SEPTAL DEFECT | 421 | ||
| PULMONARY ARTERIES | 421 | ||
| CORONARY ARTERIES | 421 | ||
| DUCTUS ARTERIOSUS | 422 | ||
| VENTRICLES | 423 | ||
| ASSOCIATED ANOMALIES | 423 | ||
| Epidemiology and Genetics | 423 | ||
| Early Presentation and Natural History | 423 | ||
| Surgical Repair | 423 | ||
| Late Outcome and Management | 423 | ||
| OUTPATIENT ASSESSMENT | 425 | ||
| LATE REINTERVENTIONS | 425 | ||
| ARRHYTHMIAS | 426 | ||
| MEDICATION | 426 | ||
| PREGNANCY AND CONTRACEPTION | 426 | ||
| EXERCISE AND SPORTS PARTICIPATION | 427 | ||
| ENDOCARDITIS PROPHYLAXIS | 427 | ||
| REFERENCES | 427 | ||
| 42 - Vascular Rings, Pulmonary Slings, and Other Vascular Abnormalities | 429 | ||
| Normal Embryogenesis | 429 | ||
| Classification and Morphology of Individual Lesions | 429 | ||
| NATIVE ANATOMIC ANOMALIES | 429 | ||
| Right Aortic Arch with Aberrant Left Subclavian Artery | 430 | ||
| Right Aortic Arch with Mirror Image Branching | 430 | ||
| Left Aortic Arch With Aberrant Right Subclavian Artery | 430 | ||
| Left Aortic Arch with Retroesophageal Right Descending Aorta | 431 | ||
| Aberrant Left Brachiocephalic (Innominate) Artery | 431 | ||
| Cervical Arch | 431 | ||
| Absent Pulmonary Valve Syndrome | 433 | ||
| Direct Aortic Compression | 433 | ||
| Innominate Artery Compression | 433 | ||
| ACQUIRED UPPER AIRWAY COMPRESSION | 433 | ||
| Postoperative Airway Compression | 433 | ||
| Compression by the Left Atrium | 433 | ||
| Aortic Aneurysm | 433 | ||
| Genetics and Epidemiology | 433 | ||
| Clinical Manifestations | 434 | ||
| HISTORY | 434 | ||
| EXAMINATION | 434 | ||
| Investigations | 435 | ||
| CHEST RADIOGRAPHY, BRONCHOSCOPY, BRONCHOGRAPHY, AND LUNG FUNCTION TESTING | 435 | ||
| BARIUM SWALLOW | 435 | ||
| ECHOCARDIOGRAPHY | 435 | ||
| CARDIAC CATHETERIZATION (RADIOGRAPHIC ANGIOGRAPHY) | 435 | ||
| MAGNETIC RESONANCE IMAGING AND COMPUTED TOMOGRAPHY | 436 | ||
| Management | 436 | ||
| RELIEF OF NATIVE ANATOMIC ANOMALIES | 436 | ||
| RELIEF OF ACQUIRED UPPER AIRWAY OBSTRUCTION | 437 | ||
| Late Outcome | 438 | ||
| Arrhythmia and Sudden Cardiac Death | 438 | ||
| Pregnancy | 438 | ||
| Level of Follow-Up, Endocarditis Prophylaxis, and Exercise | 438 | ||
| REFERENCES | 438 | ||
| VI - Diseases of theTricuspid Valve | 441 | ||
| 43 - Ebstein Anomaly | 442 | ||
| Definition and Morphology | 442 | ||
| ANATOMIC FEATURES | 442 | ||
| Genetics and Epidemiology | 442 | ||
| Early Presentation | 442 | ||
| Outpatient Assessment | 442 | ||
| IMAGING | 443 | ||
| ELECTROPHYSIOLOGY | 443 | ||
| EXERCISE TESTING | 443 | ||
| ADDITIONAL EVALUATIONS | 444 | ||
| Early Management | 444 | ||
| Late Outcome | 444 | ||
| NATURAL HISTORY | 444 | ||
| Late Management | 444 | ||
| ELECTROPHYSIOLOGY | 444 | ||
| OPERATIVE INTERVENTION | 444 | ||
| TRICUSPID VALVE REPAIR | 444 | ||
| TRICUSPID VALVE REPLACEMENT | 447 | ||
| VENTRICLE REPAIR | 447 | ||
| INTRAOPERATIVE ARRHYTHMIA PROCEDURES | 447 | ||
| EARLY OPERATIVE OUTCOMES AND CARE | 447 | ||
| LATE OPERATIVE OUTCOMES | 448 | ||
| CARDIAC TRANSPLANTATION | 448 | ||
| Pregnancy | 448 | ||
| Level of Follow-up, Endocarditis Prophylaxis, Exercise | 448 | ||
| REFERENCES | 448 | ||
| 44 - Tricuspid Regurgitation and Stenosis | 450 | ||
| Definitions and Morphology | 450 | ||
| TRICUSPID STENOSIS | 450 | ||
| TRICUSPID REGURGITATION | 450 | ||
| Genetics and Epidemiology | 451 | ||
| Early Presentation and Management | 451 | ||
| Late Outcome | 452 | ||
| SURVIVAL AND FUNCTIONAL STATUS | 452 | ||
| Outpatient Assessment | 452 | ||
| UNOPERATED PATIENTS | 452 | ||
| Role of Echocardiography | 453 | ||
| OPERATED PATIENTS | 454 | ||
| Late Management Options | 455 | ||
| LATE INTERVENTION | 455 | ||
| LATE REINTERVENTION | 456 | ||
| Arrhythmia and Sudden Cardiac Death | 456 | ||
| Pregnancy, Exercise, and Endocarditis Prophylaxis | 457 | ||
| REFERENCES | 457 | ||
| VII - Diseases of the Right Ventricular Outflow Tract | 459 | ||
| 45 - Pulmonary Stenosis | 460 | ||
| Definition and Morphology | 460 | ||
| Early Presentation and Management | 460 | ||
| Late Outcome | 460 | ||
| SURVIVAL AND FUNCTIONAL STATUS | 460 | ||
| Late Complications | 460 | ||
| Outpatient Assessment | 461 | ||
| UNREPAIRED ADULT | 461 | ||
| Physical Examination | 461 | ||
| Electrocardiography | 461 | ||
| Chest Radiography | 461 | ||
| Echocardiography | 461 | ||
| Stress Test | 462 | ||
| Magnetic Resonance Imaging or Computed Tomography | 462 | ||
| Cardiac Catheterization | 462 | ||
| OPERATED/REPAIRED ADULT | 463 | ||
| Late Management Options | 463 | ||
| INDICATIONS FOR INTERVENTION AND REINTERVENTION | 463 | ||
| Arrhythmia and Sudden Cardiac Death | 463 | ||
| Pregnancy | 463 | ||
| Level of Follow-Up, Endocarditis Prophylaxis, and Exercise | 464 | ||
| REFERENCES | 464 | ||
| 46 - Double-Chambered Right Ventricle | 465 | ||
| Definition and Morphology | 465 | ||
| Genetics and Epidemiology | 465 | ||
| Presentation and Diagnosis | 466 | ||
| Management | 467 | ||
| Late Outcomes | 469 | ||
| SURVIVAL AND FUNCTIONAL STATUS | 469 | ||
| Outpatient Assessment of the Adult With Double-Chambered Right Ventricle | 469 | ||
| REPAIRED PATIENTS | 469 | ||
| UNREPAIRED PATIENTS | 469 | ||
| ARRHYTHMIA AND SUDDEN CARDIAC DEATH | 469 | ||
| PREGNANCY | 470 | ||
| LEVEL OF FOLLOW-UP | 470 | ||
| ENDOCARDITIS PROPHYLAXIS | 470 | ||
| EXERCISE | 470 | ||
| REFERENCES | 470 | ||
| VIII - Cyanotic Conditions | 473 | ||
| 47 - Tetralogy of Fallot | 474 | ||
| Definition and Morphology | 474 | ||
| VENTRICULAR SEPTAL DEFECT | 474 | ||
| PULMONARY STENOSIS | 474 | ||
| AORTIC OVERRIDING | 474 | ||
| ASSOCIATED LESIONS | 474 | ||
| Genetics and Epidemiology | 475 | ||
| Early Presentation and Management | 475 | ||
| Late Outcomes | 476 | ||
| SURVIVAL AND FUNCTIONAL STATUS | 476 | ||
| Repaired Patients | 476 | ||
| Palliated Patients | 476 | ||
| Unoperated Patients | 477 | ||
| Outpatient Assessment | 477 | ||
| REPAIRED PATIENTS | 477 | ||
| PALLIATED-ONLY OR UNOPERATED PATIENTS | 479 | ||
| Late Management Options | 480 | ||
| REPAIRED PATIENTS | 480 | ||
| Indications for Reintervention | 480 | ||
| Surgical/Catheter Interventional Options | 481 | ||
| PALLIATED-ONLY OR UNOPERATED PATIENTS | 484 | ||
| Arrhythmia and Sudden Cardiac Death | 484 | ||
| CONDUCTION ABNORMALITIES | 484 | ||
| SUPRAVENTRICULAR ARRHYTHMIA | 484 | ||
| VENTRICULAR ARRHYTHMIA | 484 | ||
| Nonsustained Ventricular Arrhythmia | 484 | ||
| Sustained Monomorphic Ventricular Tachycardia | 484 | ||
| SUDDEN CARDIAC DEATH AND RISK STRATIFICATION | 485 | ||
| Pregnancy | 486 | ||
| Level of Follow-Up | 487 | ||
| Endocarditis Prophylaxis | 487 | ||
| Exercise | 487 | ||
| Future Therapies | 487 | ||
| REFERENCES | 487 | ||
| 48 - Pulmonary Atresia With Ventricular Septal Defect | 489 | ||
| General Overview | 489 | ||
| Anatomy | 489 | ||
| PULMONARY VASCULATURE | 489 | ||
| INTRACARDIAC ANATOMY | 489 | ||
| Genetics and Epidemiology | 491 | ||
| Early Presentation | 491 | ||
| Management | 492 | ||
| Long-Term Outcome | 493 | ||
| Outpatient Assessment | 493 | ||
| Imaging | 494 | ||
| Long-Term Management | 495 | ||
| INTERVENTION | 495 | ||
| Pregnancy | 496 | ||
| Other Recommendations | 497 | ||
| REFERENCES | 497 | ||
| 49 - Absent Pulmonary Valve Syndrome | 499 | ||
| Definition and Morphology | 499 | ||
| Epidemiology and Genetics | 499 | ||
| Tetralogy of Fallot with Absent Pulmonary Valve | 499 | ||
| CLINICAL PRESENTATION AND DIAGNOSIS | 499 | ||
| MANAGEMENT AND OUTCOME | 500 | ||
| LONG-TERM FOLLOW-UP | 500 | ||
| Absent Pulmonary Valve in Isolation | 501 | ||
| CLINICAL PRESENTATION AND DIAGNOSIS | 501 | ||
| MANAGEMENT AND OUTCOME | 501 | ||
| Summary | 501 | ||
| REFERENCES | 502 | ||
| 50 - Pulmonary Atresia With Intact Ventricular Septum | 503 | ||
| Definition and Morphology | 503 | ||
| Genetics and Epidemiology | 503 | ||
| Fetal Presentation | 503 | ||
| Early Presentation and Management | 505 | ||
| BIVENTRICULAR STRATEGY | 505 | ||
| 1.5-VENTRICLE REPAIR | 505 | ||
| UNIVENTRICULAR STRATEGY | 506 | ||
| SEVERELY DILATED RIGHT VENTRICLES | 506 | ||
| Late Outcome | 506 | ||
| SURVIVAL AND FUNCTIONAL STATUS | 506 | ||
| Biventricular Repair | 507 | ||
| 1.5-Ventricle Repair | 507 | ||
| Univentricular Repair | 507 | ||
| Outpatient Assessment | 508 | ||
| Late Management Options | 509 | ||
| SEPARATED PULMONARY AND SYSTEMIC CIRCULATIONS ACHIEVED | 509 | ||
| Biventricular Repair | 509 | ||
| 1.5-Ventricle Repair | 510 | ||
| Univentricular Repair | 510 | ||
| INCOMPLETE SEPARATION OF PULMONARY AND SYSTEMIC CIRCULATIONS | 510 | ||
| Good-Sized Right Ventricle | 510 | ||
| Medium-Sized Right Ventricle | 510 | ||
| Small-Sized Right Ventricle | 511 | ||
| Arrhythmia and Sudden Cardiac Death | 511 | ||
| Pregnancy | 511 | ||
| BIVENTRICULAR REPAIR WITH RESIDUAL PULMONARY STENOSIS OR REGURGITATION | 511 | ||
| UNIVENTRICULAR CIRCULATION | 511 | ||
| MIXED CIRCULATIONS WITH CYANOSIS | 511 | ||
| Follow-Up, Endocarditis Prophylaxis, and Exercise | 511 | ||
| REFERENCES | 512 | ||
| 51 - Transposition of the Great Arteries | 513 | ||
| Definition and Morphology | 513 | ||
| GREAT ARTERY ORIGINS | 513 | ||
| CORONARY ARTERIES | 513 | ||
| ASSOCIATED DEFECTS | 513 | ||
| Ventricular Septal Defect | 513 | ||
| Left Ventricular Outflow Tract Obstruction | 513 | ||
| Genetics and Epidemiology | 514 | ||
| Early Presentation and Management | 514 | ||
| BALLOON ATRIAL SEPTOSTOMY | 514 | ||
| ATRIAL BAFFLE REPAIR | 515 | ||
| Mustard and Senning Operations | 515 | ||
| Arterial Switch Operation | 515 | ||
| Rastelli Operation | 516 | ||
| Late Outcome | 516 | ||
| NATURAL HISTORY | 516 | ||
| ATRIAL BAFFLE REPAIRS | 516 | ||
| Survival and Functional Status | 516 | ||
| Arrhythmias and Sudden Cardiac Death | 517 | ||
| Bradyarrhythmias | 517 | ||
| Tachyarrhythmias | 518 | ||
| Sudden Death | 518 | ||
| Ventricular Function | 519 | ||
| Tricuspid Valve Function | 519 | ||
| Baffle Obstruction and Baffle Leaks | 520 | ||
| Pulmonary Hypertension | 520 | ||
| ARTERIAL SWITCH OPERATION | 520 | ||
| Survival and Functional Status | 520 | ||
| Arrhythmias and Sudden Cardiac Death | 520 | ||
| Ventricular Function | 520 | ||
| Pulmonary Artery Stenosis | 520 | ||
| Neoaortic Valve Regurgitation | 520 | ||
| Coronary Arterial and Vascular Complications | 521 | ||
| Neurologic Outcomes | 521 | ||
| RASTELLI OPERATION | 521 | ||
| Survival and Functional Status | 521 | ||
| Arrhythmias and Sudden Cardiac Death | 521 | ||
| Ventricular Function | 521 | ||
| Conduit Stenosis and Outflow Tract Obstruction | 521 | ||
| Outpatient Assessment | 521 | ||
| ATRIAL BAFFLE AND RASTELLI PATIENTS | 521 | ||
| ARTERIAL SWITCH PATIENTS | 522 | ||
| Late Management Options | 523 | ||
| MEDICAL THERAPY | 523 | ||
| SURGICAL AND CATHETER INTERVENTIONS | 523 | ||
| Arterial Switch Conversion | 523 | ||
| Pulmonary Artery Banding | 524 | ||
| Radiofrequency Ablation of Arrhythmias | 524 | ||
| Implantable Cardiac Defibrillators | 524 | ||
| Resynchronization Therapy | 524 | ||
| Coronary Artery Interventions | 524 | ||
| IX - Univentricular Hearts | 563 | ||
| 55 - Double-Inlet Ventricle | 564 | ||
| Definition and Morphology | 564 | ||
| CONDUCTION SYSTEM | 564 | ||
| ASSOCIATED LESIONS | 564 | ||
| Genetics and Epidemiology | 565 | ||
| Early Presentation and Management | 565 | ||
| Management | 566 | ||
| Late Outcome | 566 | ||
| Outpatient Assessment | 567 | ||
| OPERATED PATIENTS | 567 | ||
| UNOPERATED OR PALLIATED PATIENTS (WITH NON-FONTAN PROCEDURES) | 567 | ||
| Late Management Options | 567 | ||
| LATE INTERVENTION | 567 | ||
| LATE REINTERVENTION | 567 | ||
| Arrhythmia and Sudden Cardiac Death | 567 | ||
| Pregnancy | 568 | ||
| Level of Follow-Up, Endocarditis Prophylaxis, and Exercise | 568 | ||
| REFERENCES | 568 | ||
| 56 - Atrioventricular Valve Atresia | 570 | ||
| Tricuspid Atresia | 570 | ||
| GENETICS AND EPIDEMIOLOGY | 570 | ||
| EARLY PRESENTATION AND MANAGEMENT | 570 | ||
| Unoperated Survival | 570 | ||
| Mitral Atresia and Hypoplastic Left Heart Syndrome | 571 | ||
| GENETICS AND EPIDEMIOLOGY | 571 | ||
| MORPHOLOGY | 571 | ||
| EARLY PRESENTATION | 571 | ||
| STAGED SURGICAL MANAGEMENT | 572 | ||
| Late Outcome | 573 | ||
| PROTEIN-LOSING ENTEROPATHY | 573 | ||
| ATRIAL ARRHYTHMIA | 574 | ||
| Outpatient Assessment | 575 | ||
| UNOPERATED OR POST-SHUNT PATIENTS WITH TRICUSPID ATRESIA | 575 | ||
| Post-Fontan or Post–Total Cavopulmonary Connection Patients With Tricuspid Atresia or Hypoplastic Left Heart Syndrome | 575 | ||
| Late Management Options | 576 | ||
| LATE INTERVENTION IN UNOPERATED OR SHUNT-PALLIATED PATIENTS WITH TRICUSPID ATRESIA | 576 | ||
| LATE REINTERVENTION IN PATIENTS AFTER A FONTAN PROCEDURE | 576 | ||
| Pregnancy and Contraception | 577 | ||
| CONTRACEPTION | 577 | ||
| PREGNANCY IN UNOPERATED OR SHUNT-PALLIATED PATIENTS | 577 | ||
| PREGNANCY IN POST–FONTAN PATIENTS | 577 | ||
| Level of Follow-Up, Endocarditis Prophylaxis, and Exercise | 577 | ||
| REFERENCES | 577 | ||
| 57 - Heterotaxy and Isomerism of the Atrial Appendages | 579 | ||
| Definitions | 579 | ||
| Morphology | 579 | ||
| ATRIUM | 579 | ||
| BRONCHOPULMONARY ANATOMY | 579 | ||
| SPLEEN | 580 | ||
| ATRIOVENTRICULAR JUNCTIONS | 580 | ||
| VENTRICULOARTERIAL JUNCTIONS | 581 | ||
| VENOATRIAL CONNECTIONS | 581 | ||
| Epidemiology and Genetics | 581 | ||
| Associated Cardiac Lesions in Heterotaxy Syndrome | 581 | ||
| NONCARDIAC MALFORMATIONS | 581 | ||
| Early Presentation and Initial Management | 582 | ||
| INITIAL MANAGEMENT | 582 | ||
| Late Presentation and Outcomes | 582 | ||
| Complications and Late Management Options | 582 | ||
| Outpatient Assessment | 582 | ||
| ELECTROCARDIOGRAPHY | 582 | ||
| CHEST RADIOGRAPHY | 583 | ||
| ECHOCARDIOGRAPHY | 583 | ||
| MAGNETIC RESONANCE IMAGING AND COMPUTED TOMOGRAPHY | 583 | ||
| CARDIAC CATHETERIZATION | 584 | ||
| Conduction System Abnormalities and Arrhythmias | 584 | ||
| Pregnancy | 584 | ||
| Level of Follow-Up and Endocarditis Prophylaxis | 584 | ||
| REFERENCES | 584 | ||
| X - Coronary Artery Abnormalities | 587 | ||
| 58 - Congenital Anomalies of the Coronary Arteries | 588 | ||
| Primary Congenital Coronary Anomalies | 588 | ||
| CLASSIFICATION | 588 | ||
| PREVALENCE | 588 | ||
| ECTOPIC CORONARY ARTERIES | 589 | ||
| Definition | 589 | ||
| Circumflex Artery Arising From the Right Sinus of Valsalva or Proximal Right Coronary Artery | 589 | ||
| Origin of Coronary Arteries in the Opposite Sinus | 589 | ||
| Right Coronary Artery Arising From the Left Sinus of Valsalva | 590 | ||
| Clinical Manifestations of Coronary Arteries Arising From the Opposite Sinus | 590 | ||
| Anatomic Diagnosis of a Coronary Artery Originating in the Opposite Sinus | 591 | ||
| Management | 591 | ||
| Anomalous Origin of the Left Coronary Artery in the Pulmonary Artery | 594 | ||
| Diagnosis | 594 | ||
| Management | 594 | ||
| CORONARY ARTERIOVENOUS SHUNTS | 595 | ||
| Anatomy and Physiology | 595 | ||
| Diagnosis | 595 | ||
| Management | 595 | ||
| Secondary Congenital Coronary Anomalies | 595 | ||
| TETRALOGY OF FALLOT | 596 | ||
| TRANSPOSITION OF THE GREAT ARTERIES | 596 | ||
| BICUSPID AORTIC VALVE | 596 | ||
| TRUNCUS ARTERIOSUS | 596 | ||
| PULMONARY ATRESIA WITH INTACT VENTRICULAR SEPTUM | 596 | ||
| REFERENCES | 596 | ||
| 59 - Kawasaki Disease | 598 | ||
| Definition/Morphology | 598 | ||
| Genetics, Epidemiology and Etiology | 598 | ||
| Early Presentation and Management | 598 | ||
| Adult Onset Kawasaki Disease | 599 | ||
| Late Outcome, Long-Term Management | 599 | ||
| Patients With No Evidence of Coronary Artery Abnormalities | 599 | ||
| PATIENTS WITH TRANSIENT OR SMALL (<5 MM DIAMETER) OR MEDIUM (5 TO 8 MM) CORONARY ARTERY ANEURYSM | 600 | ||
| Patients With Large and Giant (>8 mm) Coronary Artery Aneurysm or Post Coronary Artery Occlusion | 600 | ||
| Myocardial Infarction | 600 | ||
| Coronary Artery Sequelae | 601 | ||
| Outpatient Assessment of the Adult With Kawasaki Disease | 601 | ||
| Late Management Opinions | 602 | ||
| Pregnancy | 602 | ||
| Level of Follow-Up | 602 | ||
| REFERENCES | 603 | ||
| XI - Other Lesions | 605 | ||
| 60 - Myocarditis and Dilated Cardiomyopathy | 606 | ||
| Definition | 606 | ||
| Epidemiology and Etiology | 606 | ||
| Genetics of Dilated Cardiomyopathy | 607 | ||
| Pathogenesis | 608 | ||
| Clinical Presentation and Assessment | 608 | ||
| Evidence of Myocardial Inflammation and Infection | 608 | ||
| Management | 609 | ||
| Outpatient Assessment, Level of Follow-Up, and Exercise | 609 | ||
| Arrhythmia and Sudden Cardiac Death | 610 | ||
| Emerging Role of Cardiovascular Magnetic Resonance | 610 | ||
| Prognosis | 610 | ||
| Pregnancy | 611 | ||
| Conclusion | 613 | ||
| 61 - Hypertrophic Cardiomyopathy | 615 | ||
| Definition | 615 | ||
| Morphology and Pathophysiology | 615 | ||
| DIASTOLIC FUNCTION | 615 | ||
| SYSTOLIC DYSFUNCTION | 615 | ||
| LEFT VENTRICULAR OUTFLOW TRACT OBSTRUCTION | 615 | ||
| ARRHYTHMIA | 615 | ||
| Epidemiology | 616 | ||
| Genetics | 616 | ||
| Presentation and Outpatient Assessment | 616 | ||
| PHYSICAL EXAMINATION | 616 | ||
| ELECTROCARDIOGRAPHY | 616 | ||
| AMBULATORY ELECTROCARDIOGRAPHY | 617 | ||
| ECHOCARDIOGRAPHY | 617 | ||
| CARDIOPULMONARY EXERCISE TESTING | 618 | ||
| CARDIOVASCULAR MAGNETIC RESONANCE IMAGING | 618 | ||
| Late Outcomes | 618 | ||
| Management | 618 | ||
| FAMILY SCREENING | 618 | ||
| MANAGEMENT OF SYMPTOMS IN PATIENTS WITH LEFT VENTRICULAR OUTFLOW TRACT OBSTRUCTION | 619 | ||
| MANAGEMENT OF SYMPTOMS WITHOUT LEFT VENTRICULAR OUTFLOW TRACT OBSTRUCTION | 619 | ||
| Atrial Fibrillation | 619 | ||
| SUDDEN CARDIAC DEATH | 620 | ||
| Prevention of Sudden Cardiac Death | 620 | ||
| Pregnancy | 620 | ||
| Endocarditis Prophylaxis and Exercise | 620 | ||
| REFERENCES | 621 | ||
| 62 - Constrictive Pericarditis and Restrictive Cardiomyopathy | 622 | ||
| Definition and Morphology | 622 | ||
| Epidemiology and Genetics | 622 | ||
| Early Presentation and Management | 622 | ||
| DIAGNOSIS | 623 | ||
| Pathophysiology | 623 | ||
| Past Medical History | 623 | ||
| Physical Examination | 623 | ||
| Biomarkers | 624 | ||
| Electrocardiography | 624 | ||
| Chest Radiography | 624 | ||
| Two-Dimensional and Doppler Echocardiography | 624 | ||
| Myocardial Properties | 625 | ||
| Effect of Respiration on Ventricular Filling | 625 | ||
| Computed Tomography and Magnetic Resonance Imaging | 626 | ||
| Cardiac Catheterization | 627 | ||
| Endomyocardial Biopsy | 628 | ||
| Exploratory Thoracotomy | 629 | ||
| TREATMENT | 629 | ||
| Late Outcome | 629 | ||
| Outpatient Assessment | 629 | ||
| Late Management Options | 629 | ||
| Arrhythmia and Sudden Cardiac Death | 629 | ||
| Pregnancy | 630 | ||
| Level of Follow-Up, Endocarditis Prophylaxis, and Exercise | 630 | ||
| REFERENCES | 630 | ||
| 63 - Arrhythmogenic Cardiomyopathy | 631 | ||
| Pathologic Findings | 631 | ||
| Pathogenesis of Arrhythmogenic Cardiomyopathy | 631 | ||
| ABNORMAL CELL-CELL ADHESION | 631 | ||
| ABNORMAL INTERCELLULAR JUNCTION PROTEINS AND INTRACELLULAR SIGNALING | 631 | ||
| GAP JUNCTION AND ION CHANNEL REMODELING | 632 | ||
| FROM EXPERIMENTAL MODELS TO TARGET THERAPY | 632 | ||
| Clinical Findings and Natural History | 632 | ||
| ARRHYTHMOGENIC CARDIOMYOPATHY DIAGNOSIS | 632 | ||
| ARRHYTHMOGENIC CARDIOMYOPATHY DIAGNOSIS IN THE PEDIATRIC AGE | 632 | ||
| LEFT-DOMINANT ARRHYTHMOGENIC CARDIOMYOPATHY DIAGNOSIS | 634 | ||
| DIFFERENTIAL DIAGNOSIS | 634 | ||
| ARRHYTHMOGENIC CARDIOMYOPATHY GENES/MUTATIONS AND DIAGNOSTIC IMPLICATIONS | 635 | ||
| Outpatient Assessment, Management, and Treatment | 636 | ||
| CARDIAC TRANSPLANTATION | 637 | ||
| Risk Stratification | 637 | ||
| PREGNANCY | 637 | ||
| ENDOCARDITIS PROPHYLAXIS | 637 | ||
| ACKNOWLEDGMENTS | 638 | ||
| REFERENCES | 638 | ||
| 64 - Noncompacted Myocardium | 640 | ||
| Definitions | 640 | ||
| Epidemiology | 640 | ||
| Pathology and Pathogenesis | 640 | ||
| Current Diagnostic Criteria | 641 | ||
| Clinical Presentation (Cardiac and Noncardiac Findings) | 643 | ||
| Association With Neuromuscular Disorders, Metabolic and Genetic Syndromes, and Congenital Heart Defects | 644 | ||
| Genetics | 644 | ||
| Management and Conclusion | 646 | ||
| REFERENCES | 646 | ||
| 65 - Rheumatic Fever | 649 | ||
| Pathogenesis | 649 | ||
| EPIDEMIOLOGY | 649 | ||
| CLINICAL PICTURE | 649 | ||
| Special Clinical Manifestations | 650 | ||
| CARDITIS | 650 | ||
| MITRAL REGURGITATION—JET CHARACTERISTICS | 650 | ||
| MITRAL STENOSIS—JET CHARACTERISTICS | 650 | ||
| ARTHRITIS | 651 | ||
| CHOREA | 651 | ||
| ERYTHEMA MARGINATUM | 651 | ||
| SUBCUTANEOUS NODULES | 651 | ||
| Pathology | 651 | ||
| Natural History | 651 | ||
| Therapy of Inflammatory Manifestations | 652 | ||
| Conclusion | 653 | ||
| REFERENCES | 653 | ||
| 66 - Cardiac Tumors | 655 | ||
| Diagnosis of Cardiac Tumors | 655 | ||
| GENERAL CLINICAL FEATURES | 655 | ||
| DIAGNOSTIC APPROACH | 655 | ||
| Treatment and Prognosis | 656 | ||
| Primary Benign Cardiac Tumors | 656 | ||
| MYXOMA | 656 | ||
| Clinical Manifestations | 657 | ||
| Diagnosis | 657 | ||
| Management and Treatment | 657 | ||
| PAPILLARY FIBROELASTOMA | 658 | ||
| CLINICAL MANIFESTATIONS | 658 | ||
| SELECTED TERMS USED IN ADULT CONGENITAL HEART DISEASE | 678 | ||
| Acknowledgments | 701 | ||
| INDEX | 703 | ||
| A | 703 | ||
| B | 705 | ||
| C | 705 | ||
| D | 708 | ||
| E | 709 | ||
| F | 710 | ||
| G | 711 | ||
| H | 711 | ||
| I | 712 | ||
| J | 713 | ||
| K | 713 | ||
| L | 713 | ||
| M | 714 | ||
| N | 714 | ||
| O | 715 | ||
| P | 715 | ||
| Q | 717 | ||
| R | 717 | ||
| S | 718 | ||
| T | 719 | ||
| U | 720 | ||
| V | 720 | ||
| W | 721 | ||
| Z | 721 |