Note: Intro -- Preface -- Rhodes' Postulates and the Reasons Why Pediatric and Congenital Cardiologists Should Study Exercise Physiology -- Clinical Value of Cardiopulmonary Exercise Testing in Patients with CHD and Other Disorders -- Contents -- Contributors -- Part I: The Normal Cardiopulmonary Response to Exercise -- 1: Biochemistry of Exercise -- Suggested Readings -- 2: Oxygen Delivery -- Heart Rate -- Stroke Volume -- Oxygen Extraction -- Kinetics of Oxygen Delivery and Oxygen Debt -- References -- 3: Central Hemodynamics and Coronary Blood Flow During Exercise -- References -- 4: CO2 Elimination (V̇CO2) -- Tidal Volume -- Respiratory Rate -- VD/VT Ratio -- Anaerobic Threshold: Implications for CO2 Elimination (and Oxygen Delivery) -- References -- Part II: Conduct of the Cardiopulmonary Exercise Test -- 5: Laboratory Setup, Equipment, and Protocols -- Environment -- Equipment -- Protocols -- The 6-Minute Walk Test (6MWT) -- Exercise Testing with Electrocardiographic Monitoring -- Cardiopulmonary Exercise Testing (CPET) -- Other Protocols -- References -- 6: Exercise Stress Echocardiography -- Background -- Assessment of Myocardial Ischemia -- Assessment of Patients with Known or Suspected Hypertrophic Cardiomyopathy -- Assessment of Patients with Known or Suspected Pulmonary Hypertension -- Conclusions -- References -- 7: Other Modalities: Assessment of Pulmonary Response and Measurement of Cardiac Output -- Exercise Flow-Volume Loops -- Measurement of Cardiac Output -- Noninvasive Measurement of Cardiac Output: Inert Gas Rebreathing -- Invasive Measurements of Cardiac Output -- Blood Sampling During Exercise -- Exercise Oscillatory Ventilation -- References -- 8: Supervision and Safety Precautions for Exercise Testing -- References -- 9: Special Considerations for Children -- Introduction -- Pediatric Patients and Their Families. , Test Equipment -- Imaging Studies -- Conclusion -- References -- 10: Special Considerations for Adults with Congenital Heart Disease -- Introduction -- Comorbidities -- Exercise Testing -- Conclusion -- Reference -- Part III: Interpretation of the Cardiopulmonary Exercise Test -- 11: Peak Exercise Parameters -- Peak V̇O2 -- Peak Work Rate and Endurance Time -- Heart Rate -- The Oxygen Pulse (O2P) -- Respiratory Exchange Ratio (RER) -- Blood Pressure -- Arterial O2 Saturation -- Respiratory Measurements -- References -- 12: Parameters from Submaximal Exercise -- Ventilatory Anaerobic Threshold (VAT) -- V̇E/V̇CO2 Slope -- End-Tidal pCO2 -- Oxygen Uptake Efficiency Slope -- Oxygen Uptake-Work Rate Relationship: ΔV̇O2/ΔWR -- The Exercise Electrocardiogram -- References -- 13: Putting It All Together -- References -- Part IV: Prototypical Lesions -- 14: Repaired Tetralogy of Fallot -- Basic Anatomy -- Hemodynamics -- Exercise Capacity -- V˙E/V˙CO2 Slope -- Other Abnormalities -- Spirometric Abnormalities -- Boston Children's Hospital Experience -- Pulmonary Valve Replacement -- Prognostic Value of CPET Data in Tetralogy of Fallot -- Prototypical Patients -- References -- 15: Fontan Circulation -- Basic Anatomy -- Exercise Hemodynamics -- Exercise Capacity -- Other Abnormalities -- Natural History of Exercise Function in Fontan Patients -- Boston Children's Hospital Experience -- Causes of Exercise Limitation in Fontan Patients -- Cardiac Function -- The Pulmonary Vascular Bed -- Chronotropic Insufficiency -- Other Causes of Exercise Dysfunction -- Fenestration Closure -- The Fontan Circulation as a Benchmark for Other Congenital Heart Defects -- Prognostic Value of Cardiopulmonary Exercise Testing Data in Patients with Fontan Circulations -- Prototypical Patient -- References -- 16: Aortic Valve Disease -- Basic Anatomy. , Exercise Hemodynamics: Aortic Regurgitation -- Exercise Hemodynamics: Aortic Stenosis -- Exercise Function -- Clinical Implications -- Prototypical Patient -- References -- 17: Coarctation of the Aorta -- Basic Anatomy -- Hemodynamics -- Exercise Function -- Clinical Implications -- Boston Children's Hospital Experience -- Prototypical Patient -- References -- 18: Systemic Right Ventricles with a Biventricular Circulation (L-Transposition and D-Transposition After Atrial Switch Operation) -- Anatomy -- D-Looped Transposition of the Great Arteries -- L-Looped Transposition of the Great Arteries -- Hemodynamics -- Exercise Function -- Myocardial Ischemia -- Boston Children's Hospital Experience -- Prototypical Patient -- References -- 19: D-Transposition s/p Arterial Switch Operation -- Basic Anatomy -- Hemodynamics -- Exercise Function -- Myocardial Ischemia -- Boston Children's Hospital Experience -- Prototypical Patient -- References -- 20: Ebstein's Anomaly -- Basic Anatomy -- Hemodynamics -- Exercise Function -- Clinical Implications -- Boston Children's Hospital Experience -- Prototypical Patient -- References -- 21: Pulmonary Vascular Disease -- Basic Anatomy and Pathophysiology -- Hemodynamics -- Exercise Function -- Ventilatory Function -- Clinical Implications -- Prototypical Patient -- References -- 22: Exercise Testing in Pediatric Dilated Cardiomyopathy -- Pathophysiology -- Exercise Testing in Children with Dilated Cardiomyopathy -- Oxygen Consumption During Exercise -- Ventilatory Abnormalities -- Blood Pressure and Electrocardiography Response -- Cardiopulmonary Exercise Testing as a Prognostic Tool -- 6-Minute Walk Test -- Prototypical Patient -- References -- 23: Hypertrophic Cardiomyopathy -- Basic Anatomy and Physiology -- Exercise Hemodynamics in Hypertrophic Cardiomyopathy. , Clinical Value of Exercise Testing in Hypertrophic Cardiomyopathy -- Risks Associated with Exercise Testing -- Prototypical Patient -- References -- 24: Coronary Anomalies -- Basic Anatomy and Physiology -- Diagnosis of Ischemia -- Anomalous Coronary Artery Origin -- Acquired Coronary Artery Disease in Pediatrics -- Postoperative Coronary Artery Assessment After Congenital Heart Surgery -- Coronary Artery Fistulae -- References -- 25: Metabolic Disorders -- Mitochondrial Defects -- Glycogen Storage Diseases -- Prototypical Patient: Mitochondrial Defect -- Prototypical Patient: Glycogen Storage Disease -- References -- 26: Exercise Testing After Pediatric Heart Transplantation -- Pathophysiology -- Exercise Testing in Pediatric Heart Transplant Recipients -- Electrocardiography and Heart Rate Changes -- Oxygen Consumption During Exercise -- Ventilatory Response -- Prototypical Patient -- Conclusion -- References -- 27: Cardiac Rehabilitation and Exercise Training -- Components of Cardiac Rehabilitation Programs -- Barriers to Success -- Healthcare System Barriers: Referral of Appropriate Patients -- Patient- and Community-Related Factors -- Boston Children's Hospital Experience -- Cardiac Rehabilitation Experience in Madrid -- References -- 28: Summary of Lesions -- Reference -- Part V: Electrophysiologic Issues -- 29: Syncope, Orthostatic Intolerance, and Exertional Symptoms -- Exercise-Associated Syncope -- Mid-exertional Syncope -- Peri-exertional and Post-exertional Syncope -- Orthostatic Intolerance and Postural Orthostatic Tachycardia -- Boston Children's Hospital Experience and Context -- Conclusion -- References -- 30: Exercise Stress Testing: Diagnostic Utility in the Evaluation of Long QT Syndrome -- Introduction -- Genetics -- Exercise Testing and Long QT Syndrome -- Conclusion -- References -- 31: Wolff-Parkinson-White Syndrome. , Introduction -- Electrocardiogram Findings in Wolff-Parkinson-White -- Accessory Pathway Characteristics and Clinical Manifestation -- Clinical Presentation -- Risk Stratification -- Anatomic Risk -- Electrophysiologic Risk -- Exercise Testing -- Symptomatic Patients and Exercise Testing -- Conclusion -- Conclusions Include -- References -- 32: Exercise Testing in the Management of Arrhythmias -- Overview -- Evaluation of Bradycardia -- Sinus Node Dysfunction -- Atrioventricular Block -- Implantable Cardiac Defibrillators -- Evaluation of Tachycardia -- Supraventricular Arrhythmias -- Ventricular Arrhythmias -- Overview -- Isolated Ventricular Ectopy -- Idiopathic Ventricular Tachycardias -- Ventricular Arrhythmias in Congenital Heart Disease -- Catecholaminergic Polymorphic Ventricular Tachycardia -- Arrhythmogenic Cardiomyopathy -- T-Wave Alternans -- Conclusion -- References -- Part VI: Interesting/Instructive Cases -- 33: Patients with Physiologically Normal Hearts and Lungs -- Case 33.1: "Fatigue" (Normal Study) -- Case 33.2: Anxiety-Related Hyperventilation -- Case 33.3: Chronic Metabolic Alkalosis -- Case 33.4: Anemia -- Case 33.5: Mitochondrial Myopathy s/p Heart Transplant -- Case 33.6: Complete Heart Block -- Case 33.7: Obesity -- Case 33.8: An Athlete with Shortness of Breath -- Case 33.9: Athlete with Post-viral Chronic Fatigue Syndrome -- Reference -- 34: Patients with Unusual Congenital Heart Defects and/or Intracardiac Shunts -- Case 34.1: Fontan Patient with Sinus Node Dysfunction -- Case 34.2: Hypoplastic Left Heart Syndrome, s/p Fontan, and Second-Degree Heart Block -- Case 34.3: Complex/Failing Fontan -- Case 34.4: A 1.5 Ventricle Repair -- Case 34.5: Corrected Transposition with Second-Degree AV Block -- Case 34.6: l-TGA with Heart Block -- Case 34.7: d-TGA, s/p Atrial Switch, and Progressive Right Ventricular Dysfunction. , Case 34.8: Tricuspid Atresia and s/p Bidirectional Glenn Shunt.