Preface xvii Part I The Basics 1 1 Cardiovascular Development 3 Embryology 3 Cardiovascular Physiology 3 Fetal Circulation 3 Closure of the Ductus Arteriosus 5 Closure of the Foramen Ovale 6 Closure of the Ductus Venosus 6 Pulmonary Vascular Changes 6 Myocardial Performance in the Neonate 7 Hemodynamic Changes at Birth 7 2 Important Concepts in Congenital Heart Disease 9 Pathophysiology of CHD 9 Concepts of Shunting, SingleVentricle Physiology, and InterCirculatory Mixing 9 Shunting 9 Effective Blood Flow 10 Total Pulmonary Blood Flow/Total Systemic Blood Flow 10 SingleVentricle Physiology 10 Intercirculatory Mixing 13 Classification of Anatomic Shunts 15 Simple Shunts 15 Complex Shunts 15 Pulmonary Vascular Pathophysiology 18 Causes of PreCapillary PH in CHD 18 Causes of PostCapillary PH in CHD 18 Control of Pulmonary Vascular Resistance 19 PaO2 19 PaCO2 19 pH 19 Variation in Lung Volumes 19 Vasodilator Agents 20 Sympathetic Nervous System Stimulation 20 Myocardial Ischemia 21 Aortic Diastolic Pressure 21 Subendocardial Pressure 21 Heart Rate 22 Anatomic Coronary Artery Lesions 22 3 Preoperative Evaluation 23 Clinical History 23 Physical Examination 23 Associated Congenital Abnormalities 24 Laboratory Data 25 Pregnancy 26 Psychological Considerations 26 Preoperative Preparation 27 Preoperative Fluid Therapy 27 Preoperative Medications 29 4 Intraoperative Management 31 Intraoperative Monitoring 31 ECG and Blood Pressure 31 Systemic Oxygen Saturation 32 EndTidal CO2 32 Central Venous Pressure 32 Temperature 33 NearInfraRed Spectroscopy 33 Transcranial Doppler 34 Transesophageal Echocardiography (TEE) 35 Airway Management 35 Anesthesia Induction and Maintenance 36 Induction of Anesthesia 36 Mask Induction 36 Intravenous Induction 36 Maintenance of Anesthesia 37 5 Interpretation of Cardiac Catheterization Data 39 RightHeart Catheterization 39 Right Atrial Pressure (RAP) Waveform 39 Right Ventricular Pressure (RVP) Waveform 39 Pulmonary Artery Pressure (PAP) Waveform 40 LeftHeart Catheterization 40 Left Atrial Pressure (LAP) Waveform 41 Left Ventricular Pressure (LVP) Waveform 41 Aortic Pressure Waveform 41 Left Ventriculography 41 Coronary Angiography 43 Coronary Anatomy 43 Anatomic Coronary Lesions 44 Coronary Collaterals 45 Cardiac Output Determination 45 Thermodilution Technique 45 Fick Determination 45 Evaluation of Cardiac Shunts 46 Shunt Location 46 Shunt Quantification 46 Saturation Data 48 Resistances 48 Assessment of Pulmonary Vascular Anatomy 48 Evaluation of the Pulmonary Vasculature 48 Pulmonary Artery Wedge Angiogram 48 Pulmonary Vein Wedge Angiogram 49 Assessment of Pulmonary Arterial Hypertension 49 Assessment of Valve Lesions 50 Stenotic Lesions 50 Regurgitant Lesions 50 6 Cardiopulmonary Bypass 51 The Cardiopulmonary Bypass Circuit 51 Differences Between Adult and Pediatric CPB 53 CPB Flow Rates 53 Anesthesia During CPB 53 Monitoring During CPB 54 Hemodilution 54 Priming Solution 54 Ultrafiltration 55 Conventional Ultrafiltration 55 Modified Ultrafiltration 55 Blood Gas Management 55 Deep Hypothermic Circulatory Arrest and Regional Perfusion Techniques 57 Anticoagulation 57 Protamine Reversal 58 7 Mechanical Support Devices 59 Extracorporeal Membrane Oxygenation (ECMO) 59 Indications 59 Relative Contraindications 59 Modes of ECMO Support 59 VenoVenous (VV)ECMO 59 VenoArterial (VA)ECMO 59 Device and Patient Complications 60 Common Terminology Used During an ECMO Run 61 Intraoperative Considerations for Patients on ECMO 61 Ventricular Assist Devices (VADs) 62 Indications 62 Relative Contraindications 62 Modes of VADs 62 Complications 62 Anesthetic Considerations for Patients Receiving VAD 62 Induction of Anesthesia and PreBypass Management 64 PostVAD Placement Management 65 BiVAD Insertion 65 Part II Specific Lesions 67 8 Patent Ductus Arteriosus 69 Introduction 69 Anatomy 69 Physiology 69 Surgical Therapy 69 Catheterization Laboratory Intervention 70 Anesthetic Management 70 Induction and Maintenance 70 NonCardiac Surgery 71 9 Aortopulmonary Window 73 Introduction 73 Anatomy 73 Physiology 73 Surgical Management 73 Catheterization Laboratory Intervention 74 Anesthetic Management 75 NonCardiac Surgery 75 10 Coarctation of the Aorta 77 Introduction 77 Anatomy 77 Physiology 77 Surgical Therapy 80 Catheterization Laboratory Intervention 80 Anesthetic Management 81 Induction and Maintenance 81 Management after CrossClamp Removal 82 NonCardiac Surgery 82 11 Atrial Septal Defect 83 Introduction 83 Anatomy 83 Ostium Secundum ASD 83 Ostium Primum ASD 83 Sinus Venosus Defects 84 Coronary Sinus Defect 84 Physiology 85 Surgical Therapy 85 Catheterization Laboratory Intervention 85 Anesthetic Management 86 Induction and Maintenance 86 PostCPB Management 86 NonCardiac Surgery 87 12 Ventricular Septal Defect 89 Introduction 89 Anatomy 89 Subpulmonary or Supracristal Defects 89 Membranous or Perimembranous Defects 89 Conoventricular Defects 89 Inlet or CanalType Defects 89 Muscular Defects 89 Physiology 89 Surgical Therapy 91 Catheterization Laboratory Intervention 91 Anesthetic Management 91 Induction and Maintenance 91 PostCPB Management 91 NonCardiac Surgery 92 13 Atrioventricular Canal Defects 93 Introduction 93 Anatomy 93 Partial AVC 93 Transitional AVC 93 Complete AVC (CAVC) 93 Unbalanced AVC 94 Physiology 94 Surgical Therapy 95 Anesthetic Management 95 Induction and Maintenance 95 PostCPB Management 96 NonCardiac Surgery 96 14 Double Outlet Right Ventricle 97 Introduction 97 Anatomy 97 Physiology 99 Surgical Therapy 100 Anesthetic Management 101 Induction and Maintenance 101 PostCPB Management 101 15 Truncus Arteriosus 103 Introduction 103 Anatomy 103 Physiology 104 Surgical Therapy 104 Catheterization Laboratory Intervention 105 Anesthetic Management 105 Goals 105 Induction and Maintenance 105 PostCPB Management 105 Goals 106 16 Total Anomalous Pulmonary Venous Return 107 Introduction 107 Anatomy 107 Physiology 108 Surgical Therapy 109 Anesthetic Management 109 Goals 109 Induction and Maintenance 110 PostCBP Management 110 Goals 110 LongTerm Outcomes 110 17 Left Ventricular Outflow Tract Obstruction 113 Introduction 113 Anatomy 113 Physiology 113 Surgical Therapy 114 Catheterization Laboratory Intervention 114 Anesthetic Management 114 Goals 114 Induction and Maintenance 115 PostCPB Management 115 18 Mitral Valve 117 Mitral Valve Stenosis 117 Introduction 117 Anatomy 117 Physiology 118 Surgical Therapy 118 Catheterization Laboratory Intervention 118 Anesthetic Management 118 Goals 118 PreInduction 119 Induction and Maintenance 119 PostCardiopulmonary Bypass (CPB) Management 120 Mitral Valve Regurgitation 121 Introduction 121 Anatomy 121 Physiology 121 Surgical Management 122 Catheterization Laboratory Intervention 122 Anesthetic Management in Mitral Regurgitation 122 Goals 122 Premedication 122 Induction and Maintenance 122 PostCPB Management 123 19 Pulmonary Atresia/Intact Ventricular Septum (PA/IVS) 125 Introduction 125 Anatomy 125 Physiology 125 Surgical and CatheterBased Therapy 126 Anesthetic Management 129 Goals 129 PostCPB Management 129 20 Tetralogy of Fallot (TOF) 131 Introduction 131 Anatomy 131 Physiology 131 Hypoxic or Hypercyanotic Episodes (''Tet Spells'') 132 Surgical Therapy 134 Palliative Shunts 134 Definitive Repair 136 Catheterization Laboratory Intervention 136 Anesthetic Management 137 Goals 137 Induction and Maintenance 137 PostCPB Management 138 Goals 138 Postoperative Junctional Ectopic Tachycardia 139 NonCardiac Surgery 139 21 Tetralogy of Fallot with Pulmonary Atresia (TOF/PA) 141 Anatom.