Pediatric Heart Failure (Fundamental and Clinical Cardiology)

Cover......Page 1
Series Introduction......Page 10
Preface......Page 12
Contents......Page 14
Contributors......Page 22
DEFINING HEART FAILURE......Page 28
HEART FAILURE SYNDROMES......Page 31
Cardiac Failure in the Fetus......Page 32
Acute Postnatal Cardiac Failure......Page 33
Subacute and Chronic Cardiac Failure......Page 35
MECHANISMS OF CARDIAC FAILURE Mechanical Factors......Page 36
Neurohormonal Responses......Page 38
Natriuretic Peptides......Page 45
TREATMENT Digitalis in Adults......Page 46
Digitalis in Infants and Children......Page 47
FUTURE DIRECTIONS......Page 49
INTRODUCTION......Page 58
Cardiovascular Function in the Pressure–Volume Plane......Page 59
Determinants of Ventricular Performance......Page 65
FETAL CARDIOVASCULAR PERFORMANCE Fetal Circulation......Page 67
Fetal Systolic Mechanics......Page 70
Fetal Diastolic Mechanics......Page 75
POSTNATAL CARDIOVASCULAR PERFORMANCE Circulatory Changes After Birth......Page 76
Postnatal Changes in Cardiac Mechanics......Page 78
Developmental Changes in Cardiac Mechanics After Birth......Page 81
Developmental Differences in the Response to Metabolic Derangements......Page 84
3 Cellular and Molecular Aspects of Myocardial Dysfunction......Page 92
CELLULAR AND MOLECULAR BASIS OF CARDIAC CONTRACTILITY......Page 93
Contractile Elements......Page 94
Troponin and Tropomyosin......Page 95
Anchoring Proteins......Page 97
Calcium Cycling......Page 98
Nonmyocytes......Page 100
ACUTE MYOCARDIAL DYSFUNCTION......Page 101
Inflammatory Reaction......Page 102
Protease Activation......Page 103
Treatment......Page 104
MYOCARDIAL COMPENSATION AND CHRONIC HEART FAILURE......Page 107
SUMMARY......Page 118
INTRODUCTION......Page 132
CLINICAL ASPECTS OF NEUROHORMONAL ACTIVATION IN PEDIATRIC HEART FAILURE......Page 134
Children with Cardiac Defects Before Cardiac Surgery......Page 137
Children and Young Adults with Cardiac Defects After Cardiac Surgery......Page 139
METHODOLOGICAL ASPECTS OF NEUROHORMONAL ACTIVATION IN PEDIATRIC HEART FAILURE Renin–Angiotensin–Aldosterone System......Page 141
Sympathetic Nervous System......Page 142
PATHOPHYSIOLOGY OF NEUROHORMONAL ACTIVATION IN PEDIATRIC HEART FAILURE Renin–Angiotensin–Aldosterone System......Page 143
Sympathetic Nervous System......Page 146
Endothelin......Page 148
Natriuretic Peptides......Page 149
IMMUNOLOGIC ASPECTS OF PEDIATRIC HEART FAILURE Pathophysiology of Immune Activation in Heart Failure......Page 150
Cytokines in Pediatric Heart Failure......Page 152
Cytokines as a Target of Medical Treatment......Page 155
INTRODUCTION......Page 164
DILATED CARDIOMYOPATHY, A HERITABLE FORM OF HEART FAILURE......Page 165
IDENTIFYING GENES FOR DILATED CARDIOMYOPATHY......Page 168
X-LINKED DCM AND DUCHENNE MUSCULAR DYSTROPHY—A COMMON ETIOLOGY......Page 171
AUTOSOMAL DOMINANT DCM—A GENETICALLY HETEROGENEOUS DISORDER......Page 176
INHERITED DEFECTS IN ACTIN—DCM GENE DISCOVERY USING A CANDIDATE GENE APPROACH......Page 178
MUTATIONS IN GENES ENCODING CYTOSKELETAL PROTEINS—A UNIFYING ETIOLOGY FOR DCM......Page 180
DCM WITH CONDUCTION DISEASE—NOVEL DISEASE GENE DISCOVERY BY LINKAGE ANALYSIS......Page 184
DCM AND HCM—SHARED DISEASE GENES, DIVERGENT CARDIAC REMODELING PATHWAYS......Page 186
A NEW MECHANISM FOR FAMILIAL DCM—DEFECTIVE MYOCELLULAR CALCIUM REGULATION......Page 188
CONCLUSIONS......Page 189
BACKGROUND......Page 198
CLINICAL FINDINGS ASSOCIATED WITH FETAL CHF......Page 202
ETIOLOGIES ASSOCIATED WITH FETAL HEART FAILURE......Page 208
TREATMENT STRATEGIES AND OUTCOME......Page 221
INTRODUCTION......Page 236
Acute Heart Failure Syndrome......Page 237
Maturation of Cardiac Function: Molecular and Cellular Aspects......Page 238
Neonatal Pulmonary Function......Page 243
ETIOLOGIES OF NEONATAL HEART FAILURE......Page 244
Clinical Manifestations of Neonatal Heart Failure......Page 246
General Approaches to Management Based on Physiological Considerations......Page 247
Chronic Heart Failure Syndrome......Page 253
Diuretics......Page 254
Vasodilators......Page 255
Neurohormonal Modulation......Page 256
Nutritional Therapy......Page 258
Education and Counseling......Page 261
APPENDIX A......Page 262
INTRODUCTION......Page 268
CLINICAL PRESENTATION......Page 270
INVESTIGATIONS......Page 272
INITIAL TREATMENT......Page 275
MITOCHONDRIAL CARDIOMYOPATHIES......Page 276
OXPHOS Defects......Page 277
Fatty Acid Oxidation Defects......Page 281
Mitochondrial Apoptosis......Page 285
Other Unclassified Defects......Page 286
STORAGE DISEASES ASSOCIATED WITH CARDIAC FAILURE Mucopolysaccharidoses......Page 288
Additional Lysosomal Storage Diseases with Cardiac Presentations......Page 292
ACKNOWLEDGMENT......Page 296
OVERVIEW......Page 300
INTRODUCTION......Page 301
NORMAL CARDIAC STRUCTURE......Page 303
Z-DISC ORGANIZATION......Page 307
DISORDERS OF VENTRICULAR SYSTOLIC DYSFUNCTION Dilated Cardiomyopathy......Page 309
Cardiac catheterization: This is not typically used as a......Page 311
ARRHYTHMIAS AND CONDUCTION SYSTEM DISEASE IN DILATED CARDIOMYOPATHY......Page 312
HEART FAILURE WITH PRESERVED SYSTOLIC FUNCTION......Page 313
SYNDROME OF HEART FAILURE......Page 314
CLINICAL GENETICS OF DILATED CARDIOMYOPATHY......Page 315
MOLECULAR GENETICS OF DILATED CARDIOMYOPATHY......Page 316
X-LINKED CARDIOMYOPATHIES X-Linked Dilated Cardiomyopathy (XLCM)......Page 317
BARTH SYNDROME......Page 319
AUTOSOMAL DOMINANT DILATED CARDIOMYOPATHY......Page 320
LAMIN A=C......Page 324
MUSCLE IS MUSCLE: CARDIOMYOPATHY AND SKELETAL MYOPATHY GENES OVERLAP......Page 325
Therapy of Dilated Cardiomyopathy......Page 326
Etiology......Page 327
Epidemiology......Page 328
Clinical Manifestations......Page 330
Echocardiogram: A dilated and dysfunctional left ventri-cle......Page 332
Endomyocardial biopsy: A cardiac catherization and......Page 333
The ‘‘Dallas criteria’’: In order to minimize the discre-pancy......Page 334
Molecular diagnostics: In 1986, the first molecular diag-nostic......Page 335
The Role of Cytokines in Myocarditis and DCM......Page 337
The Role of Cell Adhesion Molecules in Myocarditis and DCM......Page 338
Apoptosis......Page 339
Long-Term Sequelae......Page 340
Treatment......Page 341
Clinical Presentation......Page 343
Clinical Evaluation......Page 344
Electrocardiography: The diagnosis is most definitive......Page 346
Genetics of ARVD=ARVC......Page 347
DISEASES OF VENTRICULAR DIASTOLIC FUNCTION Hypertrophic Cardiomyopathy......Page 349
Echocardiography: The primary modality for the diagno-sis......Page 350
Metabolic studies: Similar to DCM, metabolic and mito-chondrial......Page 352
THERAPY IN HYPERTROPHIC CARDIOMYOPATHY......Page 360
Chest radiography: Chest x-rays commonly demonstrate......Page 361
Cardiac catheterization: Elevated atrial and ventricular......Page 362
Genetics of RCM......Page 363
OVERLAP DISORDERS Left Ventricular Noncompaction......Page 364
Metabolic testing: Blood and urine studies, as well as......Page 365
Echocardiography: Two-dimensional echocardiography......Page 366
Therapy and Outcome......Page 367
FINAL COMMON PATHWAY HYPOTHESIS......Page 368
RELEVANCE OF THE FINAL COMMON PATHWAY HYPOTHESIS......Page 369
RHEUMATIC FEVER......Page 398
Scope of the Problem......Page 399
Etiology and Pathogenesis......Page 401
Diagnosis and Evaluation......Page 403
Testing......Page 405
RHEUMATIC HEART DISEASE Mitral Regurgitation......Page 407
Aortic Regurgitation......Page 411
Myocarditis......Page 413
Mitral Stenosis......Page 414
Aortic Stenosis......Page 417
Right Heart Involvement......Page 418
TREATMENT OF RHEUMATIC HEART DISEASE......Page 419
Secondary Prophylaxis......Page 421
Medical Management of Acute Carditis......Page 422
Medical Management of Acute Rheumatic Carditis and Heart Failure......Page 423
Medical Management of Chronic RHD......Page 424
Surgical Treatment of RHD......Page 425
KAWASAKI DISEASE......Page 427
Myocarditis......Page 428
Coronary Artery Disease......Page 429
Diagnosis and Evaluation......Page 430
Heart Failure......Page 432
TREATMENT OF HEART FAILURE IN PATIENTS WITH KD Acute Phase of KD......Page 434
Chronic Phase of KD......Page 436
INTRODUCTION......Page 460
PATHOPHYSIOLOGY OF ARRHYTHMIAS AND SUDDEN DEATH IN HEART FAILURE Dilated Cardiomyopathies: Cause or Effect?......Page 462
Tachycardia-Related Cardiomyopathies......Page 464
ARRHYTHMIAS IN SPECIFIC CONDITIONS ASSOCIATED WITH PEDIATRIC HEART FAILURE Dilated Cardiomyopathy......Page 465
Tachycardia-Induced Cardiomyopathies......Page 471
Cardiomyopathy in Association with Isolated Congenital Complete AV Block......Page 475
Arrhythmias and Sudden Death in Restrictive Cardiomyopathy......Page 478
Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC)......Page 479
Congenital Heart Disease......Page 482
Anticongestive Measures......Page 486
Class I Antiarrhythmic Agents......Page 488
Amiodarone......Page 489
Implantable Devices......Page 490
CONCLUSIONS......Page 495
12 Single Ventricle Lesions......Page 508
HEART FAILURE IN THE NEWBORN PERIOD......Page 509
ESTABLISHMENT OF A SERIES CONNECTION OF THE SYSTEMIC AND PULMONARY CIRCUIT IN THE SINGLE VENTRICLE PATIENT—FONTAN PHYSIOLOGY......Page 514
OPTIMIZATION OF CARDIAC FUNCTION—THE TIMING AND MECHANISM OF VENTRICULAR VOLUME UNLOADING......Page 520
ATRIOVENTRICULAR VALVE REGURGITATION......Page 523
OPTIMIZATION OF CARDIAC FUNCTION—THE MANIPULATION OF VENTRICULAR PRELOAD......Page 524
OPTIMIZATION OF CARDIAC FUNCTION—THE INFLUENCE OF AFTERLOAD......Page 527
PULMONARY VASCULAR RESISTANCE......Page 529
OPTIMIZATION OF CARDIAC FUNCTION—THE DESIGN OF THE PATHWAY OF SYSTEMIC VENOUS RETURN TO THE PULMONARY ARTERIES......Page 531
OPTIMIZATION OF CARDIAC FUNCTION-ASSOCIATED DISTURBANCES OF CARDIAC RHYTHM......Page 534
SINUS NODE DYSFUNCTION......Page 535
INTRA-ATRIAL REENTRANT TACHYCARDIA (IART)=ATRIAL FLUTTER......Page 536
OPTIMIZATION OF CARDIAC FUNCTION— MYOCARDIAL ISSUES......Page 537
TREATMENT OF CARDIAC FAILURE IN THE SINGLE VENTRICLE PATIENT......Page 539
CARDIAC TRANSPLANTATION......Page 540
13 Right-Sided Heart Failure......Page 560
ETIOLOGY......Page 561
PATHOPHYSIOLOGY......Page 563
DIAGNOSIS Clinical Findings......Page 571
ECG......Page 573
Transthoracic Echocardiography......Page 575
Cardiac Catheterization......Page 581
MEDICAL MANAGEMENT General......Page 582
Pulmonary Vasodilators......Page 583
Myocardial Performance......Page 584
Supplemental Oxygen......Page 585
Atrial Septostomy......Page 586
Transplantation......Page 587
SUMMARY......Page 588
INTRODUCTION......Page 594
ETIOLOGIES OF HEART FAILURE IN CONGENITAL HEART DISEASE......Page 595
Ventricular Volume or Pressure Load and Normal Myocardium......Page 596
Diastolic Dysfunction......Page 597
MANIFESTATIONS OF HEART FAILURE IN CHILDREN WITH CONGENITAL HEART DISEASE Clinical Features......Page 598
Neurohormonal Abnormalities......Page 600
Laboratory Assessment of Heart Failure......Page 604
Volume Overload Lesions......Page 606
Primary Myocardial Dysfunction in the Postoperative Patient......Page 608
Heart Transplantation......Page 609
15 Medical Management of Chronic Systolic Left Ventricular Dysfunction in Children......Page 616
Clinical Consequences......Page 617
Medical Management......Page 618
16 Nutritional Aspects of Pediatric Heart Failure......Page 648
ENERGY EXPENDITURE......Page 651
ENERGY INTAKE......Page 658
NUTRITIONAL SUPPORT......Page 660
SUMMARY......Page 661
BACKGROUND......Page 668
MECHANISMS......Page 670
EARLY GRAFT FAILURE......Page 675
DIAGNOSIS OF ACUTE REJECTION......Page 677
CLINICAL PRESENTATION OF ACUTE REJECTION......Page 678
LATE HEART FAILURE POST TRANSPLANTATION......Page 679
ADOLESCENT RECIPIENTS......Page 681
LATE ALLOGRAFT REJECTION......Page 682
NONIMMUNE CAUSES OF LATE HEART FAILURE......Page 685
INTRODUCTION......Page 692
THE PATHOPHYSIOLOGY OF CARDIOTOXICITY......Page 693
TYPES OF ANTICANCER THERAPIES Anthracyclines......Page 695
Other Chemotherapeutic Agents......Page 702
Radiation......Page 723
MANAGEMENT OF TREATMENT-RELATED CARDIOTOXICITIES......Page 731
Primary Prevention of Anthracycline Cardiotoxicity......Page 732
Treating Cancer-Related Cardiomyopathy......Page 736
Evaluating the Pediatric Cancer Survivor......Page 742
INTRODUCTION......Page 766
NORMAL CORONARY ANATOMY......Page 767
CONGENITAL ANOMALIES—ISOLATED Anomalous Left Coronary Artery Connected to the Pulmonary Artery (ALCAPA)......Page 769
Single Coronary Artery=Anomalous Coronary Origin......Page 772
Ostial Stenosis=Atresia of Left Coronary Artery......Page 775
Coronary Artery Fistula......Page 776
MYOCARDIAL BRIDGE......Page 778
Intramural Coronary Course......Page 781
KAWASAKI DISEASE......Page 783
CORONARY STENOSIS=IATROGENIC......Page 786
HEART TRANSPLANT CORONARY ARTERY DISEASE......Page 788
MISCELLANEOUS CAUSES OF PREMATURE CORONARY DISEASE......Page 790
CONCLUSIONS......Page 792
20 Heart Failure in the Postoperative Patient......Page 800
FREQUENCY AND RISK FACTORS......Page 801
ETIOLOGY......Page 802
DIAGNOSIS=DETECTION OF LCOS Clinical and Laboratory Data......Page 803
Serum Lactate......Page 804
Use of Modified Ultrafiltration (MUF)......Page 805
RULE OUT RESIDUAL LESIONS......Page 806
PHARMACOLOGICAL SUPPORT......Page 807
Phosphodiesterase Type III Inhibitors......Page 808
MANAGEMENT OF PULMONARY HYPERTENSION......Page 812
Inhaled Nitric Oxide......Page 813
ARRHYTHMIA AFTER CPB......Page 814
FLUID OVERLOAD AFTER CPB......Page 815
CREATION OF A RIGHT-TO-LEFT SHUNT......Page 816
MECHANICAL SUPPORT......Page 817
CONCLUSION......Page 818
21 Ventricular Assist Devices......Page 828
TYPES OF PUMPS......Page 829
Positive displacement pumps: A positive displacement......Page 830
Centrifugal pumps: These pumps contain a mechanism......Page 832
TYPES OF CIRCULATORY SUPPORT SYSTEMS Extracorporeal Life Support (ECLS)......Page 833
Cannulas: Cannulas for ECLS systems have been......Page 834
Oxygenators: Two types of membrane oxygenators are......Page 835
Ventricular Assist Devices (VADs) Cannulas: Aside from the type of pump used for......Page 836
INDICATIONS FOR SUPPORT......Page 837
Indications for Short-Term Support in Children (<10 Days)......Page 839
Indications for Longer-Term Support in Children (2–6 Weeks)......Page 843
PATIENT MANAGEMENT ON CIRCULATORY SUPPORT......Page 845
Initial Stabilization Phase......Page 846
Intermediate Support Phase......Page 847
Weaning Phase of Support......Page 850
RESULTS WITH VAD SYSTEMS......Page 852
FUTURE DIRECTIONS......Page 854
22 Psychosocial Aspects of Acute and Chronic Heart Failure in Children......Page 860
MEASUREMENT ISSUES......Page 863
HEALTH-RELATED QUALITY OF LIFE......Page 864
NEUROCOGNITIVE IMPACT......Page 871
PSYCHOLOGICAL AND BEHAVIORAL FUNCTIONING......Page 873
IMPACT ON THE FAMILY......Page 879
TRANSPLANTATION......Page 880
IMPLICATIONS FOR RESEARCH AND PRACTICE......Page 885
23 Heart Failure in the Adult with Congenital Heart Disease (ACHD)......Page 896
Index......Page 916