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Critical Heart Disease in Infants and Children E-Book

Critical Heart Disease in Infants and Children E-Book

David G. Nichols | William J. Greeley | Dorothy G. Lappe | Ross M. Ungerleider | Duke E. Cameron | Philip J. Spevak | Randall C. Wetzel

(2006)

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Book Details

Abstract

Pediatric intensivists, cardiologists, cardiac surgeons, and anesthesiologists from the leading centers around the world present the collaborative perspectives, concepts, and state-of-the-art knowledge required to care for children with congenital and acquired heart disease in the ICU. Their multidisciplinary approach encompasses every aspect of the relevant basic scientific principles, medical and pharmacologic treatments, and surgical techniques and equipment. From the extracardiac Fontan procedure, and the Ross procedure through new pharmacologic agents and the treatment of pulmonary hypertension to mechanical assist devices, heart and lung transplantation, and interventional cardiac catheterization—all of the developments that are affecting this rapidly advancing field are covered in depth.

  • Employs well-documented tables, text boxes, and algorithms to make clinical information easy to access.
  • Features chapters each written and reviewed by intensivists, surgeons, and cardiologists.
  • Integrates the authors' extensive experiences with state-of-the-art knowledge from the literature.
  • Offers four completely new chapters: Cardiac Trauma, Congenital Heart Disease in the Adult, Congenitally Corrected Transposition of the Great Arteries, and Outcome Evaluation.
  • Describes the basic pharmacology and clinical applications of all of the new pharmacologic agents.
  • Details important refinements and developments in surgical techniques, including the Ross pulmonary autograft replacement of the aortic valve, video-assisted fluoroscopy, and the extracardiac Fontan connection, and discusses their indications and potential complications.
  • Explores the latest advances in the treatment of pulmonary hypertension, new developments in mechanical assist devices, heart and lung transplantation, and interventional cardiac catheterization.
  • Examines issues affecting adults with congenital heart disease.

Table of Contents

Section Title Page Action Price
Front Cover Cover
Critical Heart Disease in Infants and Children i
Copyright Page ii
Table of Contents xiii
Dedication iii
Contributors v
Preface xi
PART ONE: Basic Principles 1
Chapter 1. The Segmental Approach to Congenital Heart Disease 3
INTRODUCTION 3
POSITION OF THE HEART 4
EMBRYOLOGIC CONSIDERATIONS 4
THE ATRIA 5
VENTRICLES AND ATRIOVENTRICULAR CONNECTIONS 7
GREAT VESSELS AND VENTRICLES 9
THE UNUSUAL CASE: RELEVANCE IN THE INTENSIVE CARE SETTING 13
SUMMARY 13
References 13
Chapter 2. Cardiovascular Physiology and Shock 17
INTRODUCTION 18
CONTRACTILE AND METABOLIC PROPERTIES OF THE CARDIOVASCULAR SYSTEM 18
DETERMINANTS OF CARDIAC OUTPUT 20
DETERMINANTS OF STROKE VOLUME 21
REGULATION OF SYSTEMIC VASCULAR TONE 31
REGULATION OF VASCULAR TONE BY THE ENDOTHELIUM 32
NEURAL REGULATION OF THE SYSTEMIC VASCULATURE 35
HUMORAL CONTROL OF THE SYSTEMIC VASCULATURE 37
DISTRIBUTION OF CARDIAC OUTPUT 41
CARDIOVASCULAR DYSFUNCTION 41
CONGESTIVE FAILURE 42
ACUTE CIRCULATORY DYSFUNCTION IN SHOCK 45
CAUSES OF SHOCK 49
THERAPEUTIC STRATEGIES 57
THE POST–CARDIOPULMONARY BYPASS CIRCULATION 64
CONCLUSION 65
References 66
Chapter 3. Regulation of Pulmonary Vascular Resistance and Blood Flow 73
INTRODUCTION 74
DEVELOPMENT OF THE PULMONARY CIRCULATION 74
REGULATION OF PULMONARY BLOOD FLOW DISTRIBUTION 86
PULMONARY VASCULAR DISEASE 99
THERAPY OF PULMONARY HYPERTENSION 103
CONCLUSIONS 106
References 106
Chapter 4. Renal Function and Heart Disease 113
INTRODUCTION 113
VASOMOTOR BALANCE IN THE KIDNEY 114
RENAL RESPONSES DURING CARDIAC DYSFUNCTION 116
ACUTE RENAL FAILURE 119
INVASIVE MANAGEMENT OF ACUTE RENAL FAILURE 121
POTENTIAL OUTCOMES AND FUTURE DIRECTIONS 127
References 127
Chapter 5. Splanchnic Function\rand Heart Disease 131
INTRODUCTION 131
BASIC SCIENCE OF THE SPLANCHNIC CIRCULATION 131
EFFECTS OF CARDIOPULMONARY BYPASS ON THE SPLANCHNIC CIRCULATION 134
CLINICAL CONDITIONS 135
References 139
Chapter 6. Cerebral Function and Heart Disease 143
INTRODUCTION 143
CENTRAL NERVOUS SYSTEM DISEASE AND NONOPERATED CONGENITAL HEART DISEASE 144
SEQUELAE OF CARDIAC SURGERY 151
PATHOGENESIS AND CEREBROPROTECTION 155
NEUROLOGIC MONITORING AND MANAGEMENT 161
CONCLUSIONS 166
References 167
Chapter 7. Pharmacology of Cardiovascular Drugs 173
INTRODUCTION 174
AUTONOMIC CONTROL OF THE CARDIOVASCULAR SYSTEM 174
INOTROPIC DRUGS 178
BETA BLOCKADE 190
VASODILATION 191
VASOCONSTRICTORS 198
CONCLUSION 198
References 198
PART TWO: Special Problems 205
Chapter 8. Pediatric Arrhythmias 207
INTRODUCTION 208
THE NORMAL CARDIAC RHYTHM AND ELECTROCARDIOGRAM 208
MECHANISMS OF ARRHYTHMIAS 208
PEDIATRIC CLINICAL ARRHYTHMIAS 210
CLINICAL ENTITIES FREQUENTLY ASSOCIATED WITH ARRHYTHMIAS 220
DIAGNOSTIC METHODS FOR PEDIATRIC ARRHYTHMIAS 224
MANAGEMENT OF PEDIATRIC ARRHYTHMIAS 227
INTENSIVE CARE UNIT APPROACH TO THE DIAGNOSIS AND TREATMENT OF ARRHYTHMIAS 233
References 238
Chapter 9. Pericardial Effusion and Tamponade 243
INTRODUCTION 243
ANATOMY AND PHYSIOLOGY OF THE PERICARDIUM 243
PATHOPHYSIOLOGY OF PERICARDIAL EFFUSION AND TAMPONADE 247
ETIOLOGY OF PERICARDIAL EFFUSION AND TAMPONADE 254
CLINICAL MANIFESTATIONS 256
TREATMENT 258
THE FUTURE: PERICARDIAL THERAPEUTICS 261
References 261
Chapter 10. Anesthesia for Pediatric Cardiac Surgery 267
INTRODUCTION 267
PREOPERATIVE MANAGEMENT 267
INTRAOPERATIVE MANAGEMENT 268
INDUCTION AND MAINTENANCE OF ANESTHESIA 270
CARDIOPULMONARY BYPASS 271
MODIFIED ULTRAFILTRATION 276
ANTICOAGULATION, HEMOSTASIS, AND BLOOD CONSERVATION 277
POSTOPERATIVE PAIN MANAGEMENT 280
SPECIFIC ANESTHETIC CONSIDERATIONS 280
References 287
Chapter 11. Applied Respiratory Physiology 291
INTRODUCTION 291
DISTRIBUTION OF PULMONARYBLOOD FLOW 292
DISTRIBUTION OF VENTILATION 295
PHYSIOLOGY OF GAS AND FLUID EXCHANGE 299
DEVELOPMENTAL CONSIDERATIONS 302
ALTERATIONS IN RESPIRATORY PHYSIOLOGY SECONDARY TO CONGENITAL HEART DISEASE 303
References 304
Chapter 12. Respiratory Support for the Child with Critical Heart Disease 307
RESPIRATORY SUPPORT 307
CARDIORESPIRATORY INTERACTIONS 315
RESPIRATORY SUPPORT FOR CHILDREN WITH HEART DISEASE: A SYSTEMATIC APPROACH 318
CONCLUSIONS 330
References 330
Chapter 13. Cardiac Arrest and Cardiopulmonary Resuscitation 333
EPIDEMIOLOGY OF CARDIOPULMONARY RESUSCITATION IN CHILDREN WITH HEART DISEASE 334
PHYSIOLOGY OF CARDIOPULMONARY RESUSCITATION 335
PHARMACOLOGY OF CARDIOPULMONARY RESUSCITATION 343
NEUROLOGIC OUTCOME FOLLOWING CARDIOPULMONARY RESUSCITATION 354
References 358
Chapter 14. Coagulation Disorders in Congenital Heart Disease 367
INTRODUCTION 367
THE FLUID PHASE 368
HEMOSTASIS: THE PLATELET PHASE 369
FIBRINOLYSIS 369
CONTROL OF COAGULATION 369
TESTS OF COAGULATION 369
TESTS OF PLATELET FUNCTION 370
TESTS OF FIBRINOLYSIS 370
DEVELOPMENTAL CHANGES IN HEMOSTASIS 371
HEMOSTASIS AND COAGULATION IN CONGENITAL HEART DISEASE 371
HEMOSTATIC ALTERATIONS CAUSED BY CARDIOPULMONARY BYPASS 371
HEMATOLOGIC MANAGEMENT DURING CARDIOPULMONARY BYPASS IN CHILDREN 374
DIAGNOSIS AND MANAGEMENT OF BLEEDING AFTER CARDIOPULMONARY BYPASS IN INFANTS AND CHILDREN 375
BLOOD CONSERVATION DURING CONGENITAL HEART SURGERY 376
PHARMACOLOGIC METHODS TO REDUCE BLEEDING 377
SUMMARY 378
References 378
Chapter 15. Nutrition and Metabolism in the Critically Ill Child with Cardiac Disease 379
INTRODUCTION 379
PROTEIN-ENERGY MALNUTRITION 380
NUTRITIONAL DEFICIENCY AS A CAUSE OF HEART DISEASE 380
HEART DISEASE AS A CAUSE OF MALNUTRITION 384
MUTIFACTORIAL NUTRITIONAL SYNDROMES IN CHILDREN WITH CRITICAL HEART DISEASE 386
NUTRITIONAL ASSESSMENT 389
NUTRITIONAL MANAGEMENT OF THE CARDIAC SURGICAL PATIENT 390
NUTRITIONAL SUPPORT TECHNIQUES 391
ORAL FEEDING, SWALLOWING, AND DYSPHAGIA IN THE CHILD WITH CONGENITAL HEART DISEASE 394
MANAGEMENT OF DYSFUNCTIONAL SWALLOWING 397
CONCLUSIONS 398
References 398
Chapter 16. Heart and Lung Transplantation 405
INTRODUCTION 405
INDICATIONS 405
CONTRAINDICATIONS 407
PREOPERATIVE MANAGEMENT 408
OPERATIVE TECHNIQUES 409
SHORT-TERM COMPLICATIONS 412
LONG-TERM COMPLICATIONS 419
OUTCOMES 420
CONCLUSION 421
References 422
PART THREE: Equipment and Techniques 425
Chapter 17. Noninvasive Diagnosis of Congenital and Acquired Pediatric Heart Disease 427
INTRODUCTION 427
ECHOCARDIOGRAPHY 427
MAGNETIC RESONANCE IMAGING 443
References 458
Chapter 18. Diagnostic and Therapeutic Cardiac Catheterization 463
INTRODUCTION 463
THERAPEUTIC CATHETERIZATION AS AN ALTERNATIVE TO SURGERY 463
CARDIAC CATHETERIZATION IN PREPARATION FOR CARDIAC SURGERY 468
CARDIAC CATHETERIZATION AFTER CARDIAC SURGERY 471
USE OF INTRACARDIAC MONITORING LINES 474
PERCUTANEOUS DRAINAGE OF PERICARDIAL OR PLEURAL EFFUSIONS 475
DECOMPRESSION OF THE LEFT ATRIUM IN PATIENTS ON EXTRACORPOREAL MEMBRANE OXYGENATION 475
THERAPY FOR ACQUIRED HEART DISEASE 475
CONCLUSION 477
References 477
Chapter 19. Perioperative Monitoring 479
INTRODUCTION 480
ELECTROCARDIOGRAPHIC MONITORING 480
NONINVASIVE METHODS OF BLOOD PRESSURE MEASUREMENT 481
INTRAVASCULAR PRESSURE MONITORING 483
INVASIVE METHODS OF ARTERIAL BLOOD PRESSURE MEASUREMENT 486
CENTRAL VENOUS PRESSURE 489
PULMONARY ARTERY PRESSURE 491
LEFT ATRIAL PRESSURE 495
CARDIAC OUTPUT 496
INTRAOPERATIVE ECHOCARDIOGRAPHY 498
PULSE OXIMETRY 498
CENTRAL NERVOUS SYSTEM MONITORING 499
ACID-BASE MANAGEMENT 499
INFLUENCE OF TEMPERATURE ON PARTIAL PRESSURE OF BLOOD GASES 500
RESPIRATORY GAS AND ANESTHETIC MONITORING 502
TEMPERATURE 503
URINARY OUTPUT 503
CONCLUSION 503
References 503
Chapter 20. Cardiopulmonary Bypass in Infants and Children 507
INTRODUCTION 507
CARDIOPULMONARY BYPASS FOR INFANTS VERSUS ADULTS 508
PHYSIOLOGY OF CARDIOPULMONARY BYPASS 509
HYPOTHERMIC INJURY TO THE BRAIN 515
THE SYSTEMIC INFLAMMATORY RESPONSE 517
THE EXTRACORPOREAL CIRCUIT 518
ANTICOAGULATION AND BLEEDING MANAGEMENT 520
WEANING FROM CPB 521
MODIFIED ULTRAFILTRATION 522
POST-CARDIOTOMY MECHANICAL SUPPORT 522
CONCLUSION 523
References 524
Chapter 21. Mechanical Circulatory Support in Infants and Children 529
INTRODUCTION 529
GENERAL INDICATIONS AND CONTRAINDICATIONS 530
THE VENTRICULAR ASSIST DEVICE 532
EXTRACORPOREAL MEMBRANE OXYGENATION (ECMO) 535
ECMO AS A BRIDGE TO TRANSPLANTATION 538
OUTCOME OF VAD AND ECMO IN CHILDREN 539
VAD VERSUS ECMO FOR CHILDREN 541
COST 542
LONG-TERM SUPPORT 542
OTHER SUPPORT SYSTEMS 542
Acknowledgments 543
References 543
Chapter 22. Nursing Care of the Child with Congenital Heart Disease 545
INTRODUCTION 545
PEDIATRIC CARDIAC NURSING SKILLS AND KNOWLEDGE 545
PSYCHOSOCIAL IMPACT OF CONGENITAL HEART DISEASE IN THE CHILD 554
FAMILY-CENTERED CARE 556
References 558
PART FOUR: Congenital Heart Defects 559
Chapter 23. Perioperative Management of Patients with Congenital Heart Disease: A Multidisciplinary Approach 561
BASIC PRINCIPLES 561
GENERAL PRINCIPLES OF OXYGEN DELIVERY AND UTILIZATION 562
GENERAL PRINCIPLES OF ANATOMY AND PATHOPHYSIOLOGY AFFECTING PREOPERATIVE AND POSTOPERATIVE MANAGEMENT 563
PREOPERATIVE STABILIZATION, SURGICAL PLANNING 564
POSTOPERATIVE CARE 566
FAMILY-CENTERED CARE 575
References 576
Chapter 24. Atrial Septal Defects and Ventricular Septal Defects 579
ATRIAL SEPTAL DEFECTS 579
VENTRICULAR SEPTAL DEFECTS 585
References 591
Chapter 25. Atrioventricular Septal Defects 593
DEFINITION 593
EMBRYOLOGY 593
CLASSIFICATION AND ANATOMY 594
ASSOCIATED DEFECTS 595
NATURAL HISTORY 596
PATHOPHYSIOLOGY 597
DIAGNOSTIC ASSESSMENT 598
PREOPERATIVE CRITICAL CARE MANAGEMENT 599
SURGICAL CORRECTION 600
POSTOPERATIVE CRITICAL CARE 605
OUTCOME 606
References 606
Chapter 26. Left Ventricular Outflow Tract Obstruction 609
VALVULAR AORTIC STENOSIS 609
SUBVALVULAR STENOSIS 617
SUPRAVALVULAR STENOSIS 618
References 622
Chapter 27. Coarctation of the Aorta and Interrupted Aortic Arch 625
DEFINITIONS 625
COARCTATION OF THE AORTA 625
INTERRUPTED AORTIC ARCH 641
References 645
Chapter 28. Mitral Valve Diseases 649
HISTORY 649
EMBRYOLOGY 650
ANATOMY 650
OBSTRUCTIVE (STENOTIC) LESIONS OF THE MITRAL VALVE 651
REGURGITANT LESIONS OF THE MITRAL VALVE 656
RESULTS 659
MITRAL VALVE REPLACEMENT 659
CONCLUSIONS 660
References 660
Chapter 29. Aortopulmonary Septal Defects and Patent Ductus Arteriosus 663
AORTOPULMONARY SEPTAL DEFECTS 663
PATENT DUCTUS ARTERIOSUS 668
References 673
Chapter 30. Anomalous Origin of the Coronary Arteries 675
DEFINITION 675
EMBRYOLOGY 675
NORMAL VARIATION IN CORONARY ANATOMY 676
ANOMALOUS ORIGIN OF THE LEFT CORONARY ARTERY FROM THE PULMONARY ARTERY 677
ANOMALOUS ORIGIN OF THE RIGHT CORONARY ARTERY FROM THE PULMONARY TRUNK 684
ANOMALOUS ORIGIN OF A MAIN CORONARY ARTERY FROM THE AORTA 685
References 686
Chapter 31. Persistent Truncus Arteriosus 689
INTRODUCTION AND HISTORY 689
CLASSIFICATION 689
EMBRYOLOGY 689
ANATOMY AND ASSOCIATED ANOMALIES 690
NATURAL HISTORY 691
HEMODYNAMICS 691
DIAGNOSIS 692
SURGICAL TECHNIQUE 692
POSTOPERATIVE MANAGEMENT 695
CONCLUSIONS 696
References 696
Chapter 32. Total Anomalous Pulmonary Venous Return 699
INTRODUCTION 699
EMBRYOLOGY 699
ANATOMY AND CLASSIFICATION 700
GENETICS 701
ASSOCIATED DEFECTS 702
PATHOPHYSIOLOGY 702
PRESENTATION 702
DIAGNOSTIC ASSESSMENT 703
PREOPERATIVE CRITICAL CARE MANAGEMENT 704
SURGICAL INTERVENTION 705
POSTOPERATIVE CRITICAL CARE MANAGEMENT 708
OUTCOME 709
References 712
Chapter 33. Transposition of the Great Arteries and the Arterial Switch Operation 715
INTRODUCTION 715
EMBRYOLOGY AND ANATOMIC FEATURES 715
PHYSIOLOGY AND NATURAL HISTORY 718
DIAGNOSIS 718
PREOPERATIVE CRITICAL CARE MANAGEMENT 718
SURGICAL TREATMENT OF THE INFANT WITH TRANSPOSITION OF THE GREAT ARTERIES 719
ANATOMIC FEATURES REQUIRING SPECIAL CONSIDERATION 720
POSTOPERATIVE CRITICAL CARE MANAGEMENT 724
OUTCOME 725
CONCLUSIONS 727
References 727
Chapter 34. Double-Outlet Right Ventricle and Double-Outlet Left Ventricle 731
INTRODUCTION 731
DEFINITION 731
HISTORICAL PERSPECTIVE 732
EMBRYOLOGY AND GENETICS 732
CLASSIFICATION 733
ANATOMY 733
PATHOPHYSIOLOGY 736
DIAGNOSTIC ASSESSMENT 737
PREOPERATIVE CRITICAL CARE MANAGEMENT 740
SURGICAL PROCEDURE 741
POSTOPERATIVE CRITICAL CARE MANAGEMENT 745
OUTCOME 749
DOUBLE-OUTLET LEFT VENTRICLE 750
References 750
Chapter 35. Tetralogy of Fallot with and without Pulmonary Atresia 755
INTRODUCTION 755
ANATOMY 755
GENETICS 757
PHYSIOLOGY 757
DIAGNOSIS 758
INITIAL MEDICAL MANAGEMENT 760
SURGICAL MANAGEMENT 760
SURGICAL TECHNIQUES 762
POSTOPERATIVE CARE 762
OUTCOME 763
SUMMARY 764
References 764
Chapter 36. Pulmonary Atresia with Intact Ventricular Septum 767
INTRODUCTION/DEFINITIONS 767
ANATOMY/MORPHOLOGIC CONSIDERATIONS 767
EMBRYOLOGY 770
NATURAL HISTORY 770
DIAGNOSTICS 770
PREOPERATIVE MANAGEMENT 770
PRINCIPLES OF TREATMENT 771
CATHETER-BASED INTERVENTIONS 771
SURGICAL INTERVENTIONS 772
POSTOPERATIVE MANAGEMENT 773
OUTCOME 775
References 775
Chapter 37. Ebstein’s Malformation 777
HISTORY 777
DEFINITION 777
EMBRYOLOGY 777
ASSOCIATED DEFECTS 778
PATHOPHYSIOLOGY 778
PRESENTATION AND DIAGNOSIS 779
MEDICAL MANAGEMENT 781
SURGICAL TECHNIQUES 782
POSTOPERATIVE CRITICAL CARE 786
RESULTS 786
References 786
Chapter 38. Single-Ventricle Lesions 789
ANATOMY OF SINGLE-VENTRICLE COMPLEXES 789
BALANCING THE PARALLEL CIRCULATIONS 790
GENERAL PRINCIPLES OF NEWBORN SURGICAL MANAGEMENT 793
IMMEDIATE POSTOPERATIVE MANAGEMENT OF PALLIATIVE PROCEDURES 795
POSTOPERATIVE SURGICAL COMPLICATIONS 795
CONCLUSIONS 796
References 796
Chapter 39. Tricuspid Atresia 799
DEFINITION 799
EPIDEMIOLOGY 799
EMBRYOLOGY 800
ANATOMY AND CLASSIFICATION 800
PATHOPHYSIOLOGY 802
DIAGNOSTIC ASSESSMENT 803
MANAGEMENT OF TRICUSPID ATRESIA 805
POSTOPERATIVE PROBLEMS AFTER THE FONTAN OPERATION 815
CONCLUSION 820
References 820
Chapter 40. Hypoplastic Left Heart Syndrome 823
INTRODUCTION 823
PATHOPHYSIOLOGY 824
DIAGNOSTIC ASSESSMENT 825
PREOPERATIVE CRITICAL CARE MANAGEMENT 826
SURGICAL INTERVENTION 829
INITIAL PALLIATIVE RECONSTRUCTION (STAGE I) 830
ANESTHESIA FOR NONCARDIAC SURGERY IN CHILDREN WITH HLHS 839
OUTCOME 840
CONCLUSIONS 841
References 841
Chapter 41. Separating the Circulations: Cavopulmonary Connections (Bidirectional Glenn, Hemi-Fontan) and the Modified Fontan Operation 845
GENERAL PRINCIPLES OF SUPERIOR AND TOTAL CAVOPULMONARY CONNECTIONS 846
BIDIRECTIONAL GLENN/HEMI-FONTAN 846
THE MODIFIED FONTAN OPERATION 853
EARLY OUTCOME AFTER THE FONTAN PROCEDURE 862
References 864
PART FIVE: Medical Conditions 869
Chapter 42. The Critical Appraisal and Use of Evidence about Therapy 871
TRYING TO SORT OUT WHAT WORKED: A CASE STUDY 871
THE ERA OF EVIDENCE-BASED MEDICINE 872
STEPS IN THE PRACTICE OF EVIDENCE-BASED MEDICINE 872
BUILDING A BODY OF EVIDENCE 873
PITFALLS IN DATA INTERPRETATION 875
CRITICAL APPRAISAL OF THE CLINICAL TRIAL 876
CAUTIONS REGARDING OBSERVATIONAL STUDIES OF THERAPY 879
IMPORTANT ANALYSIS ISSUES RELATED TO APPRAISAL OF OUTCOMES OF THERAPY 880
SUMMARY 882
Suggested Readings 882
References 883
Chapter 43. Pulmonary Hypertension 885
DEFINITION/NOMENCLATURE 885
ETIOLOGY 886
BIOPHYSICS/MEASUREMENT 886
CLINICAL PRESENTATION 888
PATHOPHYSIOLOGY 888
RISK FACTORS 891
PRINCIPLES OF MANAGEMENT 892
GENERAL PHARMACOLOGIC MEASURES 893
CONCLUSIONS 896
References 896
Chapter 44. Inflammatory Heart Disease 899
INTRODUCTION 899
PERICARDITIS 899
MYOCARDITIS 904
SUMMARY 921
References 921
Chapter 45. Infective Endocarditis 927
INTRODUCTION 927
INCIDENCE 928
PATHOGENESIS OF INFECTIVE ENDOCARDITIS 928
PATHOLOGY 928
THE PATTERN OF INFECTIVE ENDOCARDITIS IN CHILDREN 929
NEONATAL ENDOCARDITIS 932
PROSTHETIC VALVE ENDOCARDITIS 933
NOSOCOMIAL ENDOCARDITIS 933
INFECTIVE ENDOCARDITIS IN INTRAVENOUS DRUG ABUSERS 934
FUNGAL ENDOCARDITIS 935
DIAGNOSIS OF INFECTIVE ENDOCARDITIS 936
DIFFERENTIAL DIAGNOSIS 938
MANAGEMENT 939
MANAGEMENT OF COMPLICATIONS 943
OUTCOME 944
RESPONSE TO THERAPY 944
RELAPSE 944
PREVENTION OF INFECTIVE ENDOCARDITIS 944
References 946
Chapter 46. Syndromes and Congenital Heart Defects 951
INTRODUCTION 951
DOWN SYNDROME OMIM 1906851 952
NOONAN SYNDROME OMIM 163950 954
CHROMOSOME 22Q11.2 DELETION SYNDROMES: DIGEORGE (OMIM 188400), VELOCARDIOFACIAL (OMIM 192430), AND CONOTRUNCAL ANOMALY FACE (OMIM 217095) SYNDROMES 956
THE VISCERAL HETEROTAXY SYNDROMES (SPLENO-CARDIAC OR ISOMERISM SYNDROMES, IVEMARK SYNDROME, OMIM 208530, 306955) 959
OTHER CARDIAC SYNDROMES 963
ETHICAL CONSIDERATIONS 964
References 967
Chapter 47. Heritable Heart Disease as Part of Multisystem Illness 973
INTRODUCTION 973
CONNECTIVE TISSUE DISEASES 973
STORAGE DISEASES 975
NEUROMUSCULAR DISEASES 977
References 979
Chapter 48. Cardiomyopathy 981
INTRODUCTION 981
DEFINITIONS OF CARDIOMYOPATHY 981
DILATED CARDIOMYOPATHY 982
HYPERTROPHIC CARDIOMYOPATHY 986
RESTRICTIVE CARDIOMYOPATHY 989
GENETICS OF CARDIOMYOPATHY 990
SUMMARY 991
References 991
Index 995