Critical Heart Disease in Infants and Children

Critical Heart Disease in Infants and Children 3e 2019......Page 0
Cover......Page 1
Critical Heart Disease in Infants and Children......Page 2
Copyright Page......Page 4
Dedication......Page 5
Contributors......Page 6
Preface......Page 18
Video Contents......Page 19
Whole Brain Leadership......Page 20
Others.......Page 21
Pacesetting.......Page 22
Super Reasonable.......Page 23
Avoid the Four Horsemen of the Apocalypse......Page 24
Flooding.......Page 25
Be Ratio Minded......Page 26
Inquiry Versus Advocacy.......Page 27
Commitment......Page 28
Selected References......Page 29
References......Page 30
Identifying Targets for Improvement......Page 31
Improving Care......Page 32
Team Structure and Dynamics......Page 33
Driving the Balanced Scorecard to Unit Levels......Page 35
Benchmarking and Collaboration......Page 36
Selected References......Page 37
References......Page 38
Overview of Real-Time Data Analytics......Page 39
Development of a Real-Time Analytics Platform in the Cardiac Intensive Care Unit......Page 40
Final Comments......Page 43
Selected References......Page 44
References......Page 45
Structural Measures......Page 46
Outcome Measures......Page 47
Importance of Disease- and Patient-Specific Factors......Page 48
Risk Modeling......Page 70
Alternative Approaches......Page 71
Deconstructing Outcomes Data: Pitfalls and Caveats......Page 72
Unintended Consequences......Page 76
Reference......Page 77
References......Page 78
Patient Population......Page 80
Philosophy and Approach......Page 81
Patient Care......Page 82
Communication.......Page 83
Conflict Resolution.......Page 84
Care Delivery......Page 85
Nursing......Page 89
Family-Centered Care......Page 90
Selected References......Page 91
References......Page 93
History of the Cardiac Intensive Care Unit......Page 94
Cardiac Intensive Care Training Pathways......Page 95
Curriculum......Page 96
Assessment......Page 97
Board Certification......Page 99
Recruitment......Page 102
References......Page 103
References......Page 104
Defining Patient Outcomes and Quality Metrics......Page 105
Risk Adjustment in Critical Care Outcomes and Quality Assessment......Page 106
Pediatric Cardiac Critical Care Consortium Clinical Registry......Page 107
Quality Collaboratives in Pediatric Cardiac Critical Care......Page 108
Value of Quality Collaboratives......Page 109
Selected References......Page 110
References......Page 111
8 Improving Safety......Page 112
Lessons From “High-Reliability Organizations”......Page 113
Defining and Building a Culture of Safety for Health Care......Page 114
Safety Reporting Systems and Approaches to Analyzing Patient Safety Events......Page 115
Surgical Handoff—Risk and Opportunity......Page 116
Nosocomial Infections in Children With Cardiac Disease......Page 117
Conclusion......Page 118
Selected References......Page 120
References......Page 121
Challenging Environment of Health Care Decisions......Page 123
Age and Cognitive Development......Page 124
Providing Informed Consent for a Patient With Complex Congenital Heart Disease......Page 125
Multidisciplinary Cardiac Conference in Management and Decision Making in Cardiac Care......Page 126
Social and Cognitive Bias and Unconscious Bias......Page 128
Improving Informed Consent, Decision Making, and Care of the Health Care Givers......Page 129
References......Page 131
References......Page 132
Pediatric Nurse Practitioners.......Page 133
Preparation for Admission From the Operating Room.......Page 134
Monitoring......Page 135
Arrhythmias......Page 137
Respiratory Complications......Page 138
Feeding Intolerance......Page 139
Initial Presentation and Diagnosis......Page 140
Developmental Needs of Children......Page 141
Conclusion......Page 143
Selected References......Page 144
References......Page 145
Intake......Page 147
Settling In......Page 148
Transparency of Information Sharing......Page 150
Anticipatory Guidance......Page 151
References......Page 152
References......Page 153
Sequential Segmental Approach......Page 154
Patent Ductus Arteriosus (Patent Arterial Duct)......Page 155
Ventricular Septal Defect......Page 156
Common Arterial Trunk (Also Known as Persistent Truncus Arteriosus)......Page 157
Tetralogy of Fallot......Page 158
Pulmonary Atresia With Intact Ventricular Septum......Page 159
Transposition of Great Arteries......Page 160
Aortic Stenosis......Page 161
Ebstein Malformation of Tricuspid Valve......Page 162
Double Outlet Right Ventricle......Page 163
References......Page 164
References......Page 165
Pressure, Flow, and Resistance......Page 166
Laminar and Turbulent Flow......Page 167
Vessel Wall Shear Stress......Page 168
Contractile Apparatus......Page 169
Cardiac Muscle Cell Contraction......Page 171
Excitation-Contraction Coupling......Page 172
Isotonic and Afterloaded Contractions......Page 173
Atrial Systole......Page 174
Ejection.......Page 175
Ventricular Pressure-Volume Relationship......Page 176
Stroke Volume......Page 177
Preload......Page 178
Afterload......Page 180
Venous Return......Page 181
Veno-Cardiac Coupling......Page 182
Mean Systemic Venous Pressure and Venous Capacitance......Page 183
Intrathoracic Pressure and Right Ventricular Preload......Page 184
Intrathoracic Pressure and Right Ventricular Afterload......Page 185
Special Considerations of Positive Pressure Ventilation in Cardiac Disease......Page 186
Effects of the Left Ventricle on the Right Ventricle......Page 187
Selected References......Page 188
References......Page 189
Compliance.......Page 194
Resistance.......Page 196
Work of Breathing.......Page 197
Gravity-Dependent Determinants of Blood Flow.......Page 198
Gravity-Independent Determinants of Blood Flow.......Page 199
Active Pulmonary Blood Flow......Page 200
Alveolar Ventilation......Page 201
Oxygen Diffusion/Transport.......Page 202
Fluid Transport.......Page 203
Airway Anatomy and Airway Resistance in Neonates......Page 204
Increased Pulmonary Blood Flow......Page 205
Bypass and Reperfusion......Page 206
Trauma of Intubation.......Page 207
Respiratory Infections......Page 208
References......Page 209
References......Page 210
Anatomy.......Page 212
Regulation of Hepatic Blood Flow.......Page 213
Hypotension and the Hepatic Artery Buffer Response.......Page 214
Physiologic Factors......Page 215
Congenital Malformations......Page 216
Etiology.......Page 217
Intestinal Ischemia and Necrotizing Enterocolitis......Page 218
Acute Hepatic Failure.......Page 220
Selected References......Page 221
References......Page 222
Renal Vasodilation......Page 226
Renal Vasoconstriction......Page 227
Pathophysiology of Salt and Water Retention......Page 228
Cardiorenal Interactions in Specific Populations......Page 229
Incidence and Risk Factors for Acute Kidney Injury......Page 230
Outcomes After Acute Kidney Injury......Page 231
Acute Kidney Injury Biomarkers......Page 232
Fluid Overload......Page 233
Aldosterone Receptor Antagonists/Potassium-Sparing Diuretics.......Page 234
Drug Dosing in Acute Kidney Injury and Renal Replacement Therapy......Page 235
Equipment and Initiation of Peritoneal Dialysis.......Page 236
Equipment and Initiation of Hemodialysis.......Page 237
Conclusion and Future Directions......Page 238
Selected References......Page 239
References......Page 240
Physiology of the Cerebral Vasculature......Page 245
Systemic Vasoconstrictive Response......Page 246
Neurovascular Coupling......Page 247
Carbon Dioxide, Oxygen, and Glucose Reactivity......Page 248
Neurologic Injury in Utero......Page 249
Perioperative Neurologic Injury......Page 250
Deep Hypothermia and Blood Gas Management......Page 251
Deep Hypothermic Circulatory Arrest or Selective Cerebral Perfusion......Page 252
Hemodilution and Transfusion During Cardiopulmonary Bypass......Page 253
Boston Circulatory Arrest Study......Page 254
Milwaukee Cohort......Page 255
Selected References......Page 256
References......Page 257
18 Endocrine Function in Critical Heart Disease......Page 262
Glycemic Control After Pediatric Cardiac Surgery......Page 263
Thyroid Disease and Thyroid Hormone Therapy After Cardiac Surgery......Page 264
Calcium Homeostasis and Hypocalcemia in Cardiac Surgery Patients......Page 265
Challenges of Defining Adrenal Insufficiency in Critical Illness......Page 266
References......Page 267
References......Page 268
Myocardial Beta1 and Beta2 Receptors.......Page 270
Adrenoreceptors in Heart Failure.......Page 271
Dopamine......Page 273
Epinephrine......Page 275
Milrinone......Page 276
Calcium......Page 279
Digoxin......Page 280
Esmolol......Page 282
Basic Principles......Page 283
Alprostadil (Prostaglandin E1)......Page 284
Indications.......Page 285
Nitroglycerin......Page 286
Angiotensin Receptor Blockers......Page 287
Arginine Vasopressin......Page 288
Drug Extraction by the Circuit......Page 289
References......Page 290
References......Page 291
Preoperative Management......Page 297
General Principles......Page 298
Physiologic Monitoring......Page 299
Induction and Maintenance of Anesthesia......Page 300
Temperature......Page 301
Ultrafiltration......Page 302
Specific Anesthetic Considerations......Page 303
Anesthesia for Simple Open-Heart Procedures......Page 304
Anesthesia for Complex Open-Heart Procedures......Page 308
Anesthesia for Closed-Heart Procedures......Page 309
Anesthesia for Heart and Lung Transplantation......Page 310
Anesthesia for Diagnostic and Interventional Cardiac Catheterization Procedures......Page 312
Anesthesia for Noncardiac Procedures......Page 313
Conclusion......Page 314
Selected References......Page 315
References......Page 316
Pain Assessment......Page 320
Analgesia......Page 321
Sedation......Page 323
Etiology of Delirium......Page 325
Delirium Assessment......Page 326
Iatrogenic Factors.......Page 328
Sleep......Page 329
Promoting Sleep Hygiene and Acute Rehabilitation in the Cardiac Intensive Care Unit......Page 330
Selected References......Page 332
References......Page 333
Electrocardiographic Monitoring......Page 338
Pulse Oximetry......Page 339
Near-Infrared Spectroscopy......Page 341
Temperature......Page 343
Invasive Monitoring......Page 344
Transpulmonary Thermodilution and Pulse Contour Analysis......Page 345
Venous Oximetry......Page 346
Arterial Blood Pressure.......Page 347
Central Venous Pressure.......Page 348
Capnometry......Page 349
Acid-Base Status......Page 350
Cardiac Biomarkers......Page 351
Urine Output and Biomarkers of Renal Perfusion......Page 352
Selected References......Page 353
References......Page 354
Inspiratory Flow.......Page 359
Spontaneous Breaths.......Page 360
Flow Graphics.......Page 361
Volume Graphics.......Page 362
Alarms.......Page 363
Carbon Dioxide Elimination.......Page 365
Effect of Nitric Oxide Administration......Page 366
Effect of Ventilatory Manipulations on Right Ventricular Function......Page 367
Perioperative Management......Page 368
Ventilator Mode.......Page 370
Inadequate Oxygen Delivery.......Page 371
Inadequate Carbon Dioxide Elimination.......Page 372
Right Ventricular Dysfunction.......Page 373
Pulmonary Artery Hypertension.......Page 374
Weaning From Positive Pressure Ventilation......Page 375
Carbon Dioxide Elimination.......Page 376
Criteria for Extubation.......Page 377
Selected References......Page 379
References......Page 380
Initiation Phase......Page 383
Fibrinolysis......Page 385
Tests of Platelet Function......Page 386
Thromboelastography and Thromboelastometry......Page 387
Hemostasis and Coagulation in Congenital Heart Disease......Page 388
Platelets......Page 389
Warfarin......Page 390
Thrombosis in Pediatric Cardiac Disease and Use of Anticoagulation......Page 391
Use of Anticoagulation in Pediatric Heart Disease (Table 24.1)......Page 392
Activation of Coagulation......Page 395
Inflammation......Page 396
Platelet Dysfunction......Page 397
Heparinization......Page 398
Diagnosis and Management of Bleeding After Cardiopulmonary Bypass in Infants and Children......Page 399
Pharmacologic Methods to Reduce Bleeding......Page 400
Anticoagulation on ECMO......Page 401
Selected References......Page 402
References......Page 404
Iron Replacement......Page 411
General Strategies.......Page 412
Antifibrinolytics.......Page 413
Transfusion Algorithms/Coagulation Studies.......Page 414
Blood Utilization and Blood Conservation—Postoperative Management......Page 415
Red Blood Cell Transfusions—Restrictive Transfusion Practices.......Page 417
Red Blood Cell Storage Age and Modification......Page 418
Blood Cell Conservation—Reinfusion of Cell Saver Blood.......Page 419
Selected References......Page 420
References......Page 421
l-Carnitine Deficiency and Fatty Acid Oxidation Defects.......Page 425
Vitamin D and Calcium Deficiency.......Page 427
Heart Failure......Page 428
Intestinal Malabsorption and Nutrient Loss (Protein-Losing Enteropathy, Chylothorax)......Page 429
Biochemical Measurements......Page 431
Preoperative Nutritional Management......Page 432
Energy Requirements.......Page 433
Immunologic Benefits of Enteral Nutrition......Page 434
Parenteral Nutrition......Page 435
Management of Dysfunctional Swallowing......Page 436
References......Page 437
References......Page 438
Mechanisms of Arrhythmia......Page 443
Bedside Telemetry......Page 445
Pharmacologic Treatment of Tachyarrhythmias......Page 446
Class IC Antiarrhythmic Medications (Flecainide, Propafenone).......Page 448
Adenosine.......Page 449
Pharmacologic Agents for Treatment of Bradyarrhythmias......Page 450
Premature Ventricular Complexes......Page 451
Atrioventricular Block......Page 452
Atrioventricular Reciprocating Tachycardia.......Page 453
Atrial Flutter.......Page 454
Permanent Junctional Reciprocating Tachycardia.......Page 456
Ventricular Tachycardia.......Page 457
Torsades de Pointes.......Page 458
Wolff-Parkinson-White (Ventricular Preexcitation) Syndrome......Page 459
Catecholaminergic Polymorphic Ventricular Tachycardia......Page 460
Dilated Cardiomyopathy......Page 461
Classification of Modes......Page 462
Methods of Temporary Pacing......Page 463
Maintenance and Safety......Page 464
Troubleshooting......Page 465
Treatment of Tachyarrhythmias With the Pacemaker......Page 466
Selected References......Page 467
References......Page 468
Etiology.......Page 471
Cardiac Tamponade.......Page 472
Management.......Page 473
Etiology......Page 475
Overview......Page 476
Pathogenesis.......Page 477
Clinical Manifestations.......Page 478
Diagnostic Approach.......Page 480
Pharmacologic and General Supportive Care.......Page 482
Immunosuppressive Therapy.......Page 483
References......Page 484
References......Page 485
Ultrasound Screening for Congenital Cardiac Defects......Page 490
Support for Lower Levels of Newborn Care—Undiagnosed Cardiac Defects......Page 491
Cardiac Defects......Page 492
Summary......Page 493
Selected Readings......Page 494
Developmental Differences in Myocardial Structure and Excitation-Contraction Coupling.......Page 496
Neonatal Ventricular Performance.......Page 497
Renal and Gastrointestinal......Page 498
Effects of Cardiopulmonary Bypass on Neonates......Page 499
Cardiac Surgery at Low Birth Weight and Very Low Birth Weight......Page 500
Surgical Repair of Congenital Diaphragmatic Hernia.......Page 501
Surgical Management.......Page 502
Selected References......Page 503
References......Page 504
Risk Factors......Page 507
Vasopressors......Page 509
Recognition and Management of Shunt Obstruction.......Page 511
Unique Challenges in Cardiopulmonary Resuscitation in the Patient With Patent Ductus Arteriosus or Shunted Single-Ventricle Physiology.......Page 512
Unique Challenges in Cardiopulmonary Resuscitation in Patients With a Superior Cavopulmonary Anastomosis or Fontan.......Page 513
Maximizing the Provider’s Performance—Mechanical Feedback......Page 514
Optimizing the Patient’s Response— Physiologic Feedback......Page 515
Direction of Resuscitative Efforts.......Page 516
Detection of Return of Spontaneous Circulation.......Page 517
Prognostication.......Page 518
Mechanical Support—Extracorporeal Life Support and Extracorporeal Cardiopulmonary Resuscitation......Page 519
Post Cardiac Arrest......Page 520
Post–Cardiac Arrest Care......Page 521
References......Page 522
References......Page 523
Esophageal Atresia and Tracheoesophageal Fistula......Page 530
Other Congenital Pediatric Surgical Conditions......Page 531
Necrotizing Enterocolitis......Page 532
Vascular Access......Page 536
Feeding Dysfunction......Page 537
Selected References......Page 540
References......Page 541
Postoperative Cardiac Dysfunction—Pathophysiology......Page 542
Postoperative Cardiac Dysfunction— Medical Management......Page 543
Shunt Malfunction......Page 544
Pleural and Pericardial Diseases......Page 545
Pulmonary Hypertension......Page 546
Arrhythmias......Page 547
Central Nervous System Injuries......Page 548
Acute Kidney Injury and Fluid Management......Page 549
Infectious Complications......Page 550
Quality Improvement Initiatives in Postoperative Care......Page 551
References......Page 552
References......Page 553
Signal Processing and Image Generation......Page 556
Transducer Selection: Tradeoff Between Resolution and Penetration......Page 559
Doppler Analysis......Page 560
Continuous Wave Doppler and High Pulse Repetition Doppler.......Page 561
Theoretical Concepts.......Page 562
Clinical Assessment of Systolic Function.......Page 566
Assessment of Right Ventricular Systolic Function......Page 567
Echocardiographic Indicators of Tamponade Physiology......Page 568
Balloon Atrial Septostomy......Page 569
Assessment of Central Line Placement......Page 570
Echocardiography and Extracorporeal Membrane Oxygenation......Page 571
Echocardiography on Extracorporeal Membrane Oxygenation......Page 572
Echocardiography Before Ventricular Assist Device Placement......Page 573
Indications......Page 574
Selected References......Page 575
References......Page 576
Vascular Access......Page 577
Cardiac......Page 579
Qualitative Evaluation of Cardiac Function and Cardiac Views......Page 580
Intravascular Volume Status......Page 581
Cardiac Arrest......Page 583
Lung......Page 584
Pleural Effusions......Page 585
Diaphragm......Page 587
Abdomen......Page 588
Abdominal Assessment of Free Fluid......Page 589
Future Directions......Page 590
Selected References......Page 591
References......Page 592
Gradient Echo.......Page 594
First-Pass Perfusion Imaging.......Page 595
Four-Dimensional Flow Magnetic Resonance Imaging.......Page 596
Case 2.......Page 597
Case 5.......Page 599
Estimation of Shunt Fraction (Qp:Qs).......Page 601
When Sedation Is Needed......Page 602
Case 4.......Page 603
Case 6.......Page 605
Selected References......Page 606
Tetralogy......Page 607
Computed Tomography......Page 608
Assessment of Pressure Data......Page 609
Estimating Shunts......Page 610
Indications for Diagnostic Catheterization......Page 613
To Assess Pulmonary Vascular Resistance.......Page 614
To Evaluate Sources of Pulmonary Blood Flow in Patients With Pulmonary Atresia and Ventricular Septal Defect.......Page 615
Therapeutic Cardiac Catheterization......Page 616
Aortic Valve Stenosis......Page 617
Native and Recurrent Coarctation of the Aorta......Page 618
Ductal Stenting......Page 619
Hybrid Stage I Palliation for Hypoplastic Left Heart Syndrome......Page 620
Risk Scores......Page 621
Cardiac Catheterization in Children Requiring Mechanical Circulatory Support......Page 622
Selected References......Page 623
References......Page 624
Brain Natriuretic Peptide Structure.......Page 626
Normative Pediatric Values.......Page 627
Perioperative and Postoperative Levels.......Page 628
Soluble ST2 Structure and Function.......Page 629
Cardiac Ischemia in the Intensive Care Unit.......Page 630
Other Biomarkers......Page 631
Key Points and Conclusion......Page 632
Selected References......Page 633
References......Page 634
Perioperative Support......Page 636
Biventricular Circulation......Page 637
Equipment......Page 638
Extracorporeal Membrane Oxygenation Cannulation and Initial Management......Page 639
Rapid-Deployment Extracorporeal Membrane Oxygenation......Page 641
Respiratory Support......Page 642
Anticoagulation and Blood Product Management......Page 643
Weaning From Extracorporeal Membrane Oxygenation......Page 644
Survival......Page 645
Neurofunctional and Quality-of-Life Outcomes......Page 646
Selected References......Page 647
References......Page 648
Pediatric Heart Failure......Page 652
Extracorporeal Membrane Oxygenation......Page 653
Temporary Ventricular Assist Device Cannulation......Page 654
Intraaortic Balloon Counterpulsation......Page 656
Biventricular Failure and Evaluation of Ventricular Assist Device Support......Page 657
Ventricular Assist Device for Single-Ventricle Hearts......Page 658
Left Ventricular Assist Device......Page 659
Device Management.......Page 660
Bleeding......Page 662
Ventricular Assist Device Weaning......Page 663
Selected References......Page 664
References......Page 665
Cardiopulmonary Bypass in Neonates and Infants......Page 668
Basic Cardiopulmonary Bypass Terms, Conditions, and Strategies......Page 669
Postoperative Low Cardiac Output Syndrome......Page 670
Myocardial Protection......Page 671
Postoperative Strategies......Page 672
Hemostasis and Cardiopulmonary Bypass......Page 673
Neurologic Injury......Page 674
Hypothermic Injury to the Brain......Page 675
Cerebral Oximetry......Page 677
Goal-Directed Therapy and Near-Infrared Spectroscopy......Page 678
References......Page 679
References......Page 680
Presentation......Page 685
Ductal Dependent Systemic Circulation.......Page 686
Ensure Adequate Pulmonary and Systemic Output......Page 687
Stabilize Hemodynamics......Page 688
Transport Modes......Page 689
Transport Intake.......Page 691
Elective Intubation Before Transfer: Risks and Benefits.......Page 692
Transport Complications......Page 693
Summary......Page 694
Selected References......Page 695
References......Page 696
Anatomy and Embryology......Page 697
Pathophysiology......Page 698
Medical Management......Page 699
Open Surgical Approach......Page 700
Surgical Complications......Page 702
Conclusion......Page 703
References......Page 704
Embryology......Page 705
Presentation and Diagnosis......Page 706
Double Aortic Arch......Page 707
Pulmonary Artery Sling......Page 708
Innominate Artery Compression......Page 709
Selected References......Page 711
References......Page 712
Pathophysiology......Page 713
Presentation......Page 714
Medical Management......Page 715
Anesthetic Management......Page 717
Respiratory Complications.......Page 718
Postoperative Systemic Hypertension.......Page 720
Survival.......Page 721
Evaluation......Page 722
Surgical Repair......Page 723
Interventional Therapy......Page 724
Stents.......Page 725
References......Page 726
References......Page 727
Embryology......Page 730
Diagnostic Assessment......Page 731
Indications and Timing of Surgery......Page 732
Primary Repair of Interrupted Aortic Arch With Ventricular Septal Defect......Page 733
Strategies for Treatment of Left Ventricular Outflow Tract Obstruction at the Time of Interrupted Aortic Arch Repair......Page 735
References......Page 736
References......Page 737
Ostium Primum Atrial Septal Defect......Page 738
Natural History......Page 742
Presentation and Evaluation......Page 743
Indication for Closure......Page 744
Catheter-Based Device Closure of Atrial Septal Defects......Page 745
Secundum Atrial Septal Defect......Page 746
Sinus Venosus Atrial Septal Defect......Page 747
Ostium Primum Atrial Septal Defect......Page 748
Result of Surgery and Long-Term Prognosis......Page 750
References......Page 752
References......Page 753
Associated Defects and Genetics......Page 754
Diagnostic Assessment......Page 756
Surgical Intervention......Page 758
Postoperative Critical Care......Page 761
Outcomes......Page 762
Selected References......Page 763
References......Page 764
Pathophysiology and Natural History......Page 766
Echocardiography......Page 767
Cardiac Catheterization......Page 768
Operative Management......Page 770
Interventional Therapy......Page 772
Selected References......Page 773
References......Page 775
Classification and Anatomy......Page 777
Pathophysiology and Natural History......Page 778
Diagnostic Assessment......Page 779
Incomplete Atrioventricular Septal Defects......Page 780
Palliative Procedures......Page 781
Complete Atrioventricular Septal Defect With Tetralogy of Fallot......Page 783
Postoperative Critical Care Management......Page 784
Selected References......Page 785
References......Page 786
Critical Neonatal Aortic Stenosis......Page 788
Valvar Aortic Stenosis in Older Children......Page 792
Subvalvar Aortic Stenosis......Page 797
Supravalvar Aortic Stenosis......Page 800
References......Page 804
References......Page 805
Embryology......Page 808
Anatomy......Page 809
Mitral Rings......Page 810
Parachute Mitral Valve......Page 811
Diagnosis of Mitral Valve Obstruction......Page 812
Postoperative Critical Care......Page 813
Double-Orifice Mitral Valve......Page 814
Diagnosis of Mitral Insufficiency......Page 815
Surgical Management......Page 816
Selected References......Page 817
References......Page 818
Marfan Syndrome......Page 820
Loeys-Dietz Syndrome......Page 821
Ehlers-Danlos Syndrome......Page 822
Mitral Valve Disease......Page 823
Aortic Root Procedures in Connective Tissue Diseases......Page 824
Bicuspid Aortic Valves and Valve Repair......Page 826
Mitral Valve Procedures in Connective Tissue Diseases......Page 827
Preoperative and Postoperative Considerations......Page 828
References......Page 829
References......Page 830
Associated Lesions......Page 832
History......Page 834
Differential Diagnosis......Page 835
Transpulmonary Approach.......Page 836
Anomalous Origin of a Pulmonary Artery From the Ascending Aorta......Page 838
Postoperative Management......Page 839
References......Page 840
References......Page 841
Classification......Page 842
Associated Anomalies......Page 843
Physiology......Page 845
Preoperative Management......Page 846
Surgical Technique......Page 847
Postoperative Management......Page 848
Selected References......Page 850
References......Page 851
Anatomy and Embryology......Page 853
Pathophysiology......Page 854
Diagnostic Imaging......Page 855
Surgical Techniques......Page 856
Postoperative Management......Page 857
Anatomy......Page 859
Cardiopulmonary Exercise Testing.......Page 860
Coronary Artery Fistula......Page 861
Anatomy......Page 862
Surgical Techniques.......Page 863
References......Page 865
References......Page 866
Pathophysiology and Clinical Presentation......Page 869
Timing of Surgery.......Page 871
Coronary Transfer Techniques......Page 872
d-Transposition With Left Ventricular Outflow Tract Obstruction......Page 874
Low Cardiac Output Syndrome and Left Ventricular Dysfunction......Page 875
Coronary Artery Insufficiency or Obstruction......Page 876
Mortality After the Arterial Switch Operation......Page 877
Neurodevelopmental Outcomes......Page 878
References......Page 879
References......Page 880
Genetics......Page 882
Associated Obstructive Lesions......Page 883
Classification......Page 884
Surgical Procedures......Page 885
TGA-Type (Subpulmonary VSD, With PS)......Page 887
Residual Anatomic Defects......Page 888
Hemodynamic Monitoring and Support......Page 889
Left Ventricular Dysfunction......Page 890
Atrial Tachyarrhythmia......Page 891
Selected References......Page 892
References......Page 893
Tetralogy of Fallot With Pulmonary Atresia......Page 896
Clinical Manifestations......Page 898
Initial Medical Management......Page 899
Tetralogy of Fallot With Pulmonary Stenosis......Page 900
Systemic to Pulmonary Artery Shunt.......Page 901
Tetralogy of Fallot With Pulmonary Atresia......Page 902
Postoperative Care......Page 906
Considerations for the Challenging Neonate......Page 907
Tetralogy of Fallot With Pulmonary Stenosis......Page 908
Longer-Term Follow-Up and the Adult With Tetralogy of Fallot......Page 909
Selected References......Page 910
References......Page 911
Pulmonary Valve Replacement Options (Box 60.3)......Page 915
Bioprosthetic Stented Valve.......Page 916
Mechanical Valve.......Page 917
Transcatheter Pulmonary Valve Replacement......Page 919
References......Page 920
References......Page 921
Embryology......Page 923
Physiology......Page 924
Preprocedural Care and Evaluation......Page 925
One-and-One-Half-Ventricle Palliation......Page 927
Postoperative Considerations in the ICU......Page 928
References......Page 929
References......Page 930
Decision Making......Page 931
Left Ventricular Hypoplasia and Outflow Obstruction......Page 932
Congenitally Corrected Transposition of the Great Arteries......Page 934
Palliative Procedures......Page 935
Pulmonary Artery Banding......Page 936
Systemic to Pulmonary Artery Shunt......Page 937
The Hybrid Procedure......Page 939
Superior Cavopulmonary Anastomosis......Page 940
Summary......Page 942
Recruitment of the Borderline Left Ventricle......Page 943
Selected References......Page 944
References......Page 945
Preoperative Assessment......Page 947
Pre-Fontan Staging......Page 948
Indications for Surgery......Page 949
Lateral Tunnel Fontan Completion After the Hemi-Fontan Procedure.......Page 950
Extracardiac Fontan Completion After the Bidirectional Glenn.......Page 951
Left Pulmonary Artery Stenosis.......Page 952
Atrioventricular Valve Regurgitation.......Page 953
Early Complications After Fontan Completion......Page 954
Late Complications After Fontan Completion......Page 955
Neurodevelopmental Outcome......Page 956
Selected References......Page 957
References......Page 958
Pathophysiology......Page 960
Indications for Surgery......Page 961
Evolution of Principles of Surgical Management......Page 962
Our Surgical Technique......Page 963
Results of Our Approach......Page 964
Selected References......Page 966
References......Page 967
Postnatal Circulation......Page 968
Diagnostic Assessment......Page 970
Management of the Newborn With Decreased Pulmonary Blood Flow......Page 971
The Modified Blalock-Taussig Shunt......Page 972
Management of the Newborn With Increased Pulmonary Blood Flow......Page 973
Bidirectional Glenn (Cavopulmonary Anastomosis)......Page 974
Postoperative Management After a Bidirectional Glenn/Hemi-Fontan......Page 976
The Fontan Operation......Page 977
Complications of Systemic Venous Hypertension......Page 978
Conclusion......Page 979
Selected References......Page 980
References......Page 981
Overview......Page 984
Physical Findings......Page 985
Prostaglandin E1 Infusion......Page 986
Augmenting Systemic Output in the Setting of Excessive Pulmonary Blood Flow.......Page 987
Nutrition.......Page 988
Historical Overview......Page 989
Development of the Staged Repair of Hypoplastic Left Heart Syndrome......Page 990
Surgical Reconstructive Techniques......Page 991
Options for Pulmonary Blood Flow......Page 994
Optimization of Systemic Cardiac Output.......Page 995
Hypoxemia.......Page 997
Myocardial Dysfunction.......Page 998
Late Postoperative Issues......Page 999
Neurologic Outcome......Page 1000
References......Page 1001
References......Page 1002
Atrial Appendages......Page 1006
Cardiac Morphology......Page 1007
Conduction System and Arrhythmias......Page 1008
Impact on Surgical Palliation......Page 1009
Lung Function and Cardiopulmonary Exercise Testing......Page 1010
Risk of Bacteremia......Page 1011
Neurodevelopmental Delay......Page 1012
References......Page 1013
References......Page 1014
Cox-Maze IV Procedure......Page 1017
Pulmonary Vein Isolation......Page 1018
Left Atrial Lesion Set......Page 1019
Supraventricular Arrhythmias......Page 1021
Prophylactic Arrhythmia Surgery......Page 1022
Postoperative Management......Page 1024
Selected References......Page 1025
References......Page 1026
Unobstructed Pulmonary Outflow......Page 1028
Diagnosis......Page 1029
Physiologic Repair......Page 1030
Double-Switch Procedure.......Page 1031
Special Considerations......Page 1033
Pulmonary Artery Banding......Page 1034
Outcomes......Page 1035
Bibliography......Page 1037
Bibliography......Page 1038
Lesion-Specific Congenital Heart Defects......Page 1039
Microorganisms......Page 1040
Pathogenesis......Page 1041
Diagnostic Criteria......Page 1042
Blood Cultures......Page 1043
Echocardiography......Page 1044
General Principles of Antimicrobial Therapy......Page 1046
Streptococci......Page 1047
Route of Administration and Duration of Therapy......Page 1049
Prevention......Page 1050
Selected References......Page 1051
References......Page 1052
Challenges Specific to Congenital Heart Disease......Page 1055
Perioperative Risk in Patients With PH......Page 1057
Echocardiography for Pulmonary Hypertension......Page 1058
Catheterization Procedure......Page 1060
Catheter-Based Interventions for Pulmonary Hypertension......Page 1061
Pulmonary Hypertensive Crisis Management......Page 1062
Phosphodiesterase Type 5 Inhibitors (PDE-5 Inhibitors)......Page 1064
Endothelin Receptor Antagonists......Page 1066
Transitions to Home Care With Pulmonary Hypertension......Page 1067
Animal Models of Pulmonary Hypertension......Page 1068
Molecular Models......Page 1069
Selected References......Page 1070
References......Page 1071
Prostacyclin and Prostacyclin Analogues for Pediatric Pulmonary Hypertension......Page 1079
Clinical Manifestations of Pediatric Heart Failure......Page 1085
Pediatric Cardiomyopathies and Heart Failure......Page 1087
Hypertrophic Cardiomyopathy......Page 1088
Cardiac Evaluation.......Page 1089
Outcomes of Heart Failure in Children With Cardiomyopathy......Page 1090
Congenital Heart Disease and Heart Failure......Page 1091
Cyanosis......Page 1092
Infection......Page 1093
Medical Management of Heart Failure in Children......Page 1094
Fluid Management......Page 1095
Hypertrophic Cardiomyopathy......Page 1096
Management of Psychosocial Stresses of Heart Failure in Children......Page 1099
References......Page 1100
References......Page 1101
Lung Transplantation......Page 1107
Recipient Evaluation......Page 1109
Recipient Management......Page 1110
Donor Management......Page 1111
Donor Organ Retrieval......Page 1112
Heart-Lung Block.......Page 1113
Lung Implantation......Page 1114
Graft Failure......Page 1116
Acute Rejection......Page 1117
Cardiac Rhythm......Page 1120
Heart Transplantation......Page 1121
Lung Transplantation......Page 1122
References......Page 1123
References......Page 1124
Venous Thromboembolism......Page 1128
Incidence of Pulmonary Embolism......Page 1129
Diagnostic Workup for Pulmonary Embolism......Page 1130
Treatment of Pulmonary Embolism......Page 1131
Conclusion......Page 1132
Selected References......Page 1134
References......Page 1135
75 Syndromes, Genetics, and Heritable Heart Disease......Page 1137
Down Syndrome......Page 1138
22q11.2 Deletion Syndrome......Page 1139
Turner Syndrome......Page 1141
Noonan Syndrome......Page 1142
Trisomy 18 (Edwards Syndrome)......Page 1143
Heterotaxy Syndrome......Page 1144
Diagnostic Considerations in Cardiovascular Genetics......Page 1145
Additional Cardiac Syndromes......Page 1146
Illustrative Case.......Page 1147
Autonomy.......Page 1148
References......Page 1149
References......Page 1150
Adult Congenital Heart Disease Patient in the Intensive Care Unit: Systems Factors......Page 1154
Systemic Right Ventricle......Page 1155
Right Ventricular Dysfunction......Page 1156
Heart Failure......Page 1157
Vasopressors.......Page 1158
Selected References......Page 1159
References......Page 1160
A......Page 1162
B......Page 1164
C......Page 1165
D......Page 1169
E......Page 1170
F......Page 1171
H......Page 1172
I......Page 1173
L......Page 1174
M......Page 1175
N......Page 1176
P......Page 1177
R......Page 1180
S......Page 1181
T......Page 1182
V......Page 1183
Z......Page 1184