Viviane G. Nasr, MD graduated from Medical School at the American University of Beirut (AUB), Lebanon in 2003, and pursued a residency in anesthesiology at AUB and Tufts Medical Center, Boston. She completed a pediatric anesthesia fellowship at Children's National Medical Center (CNMC) in Washington DC and a pediatric cardiac anesthesia fellowship at Boston Children's Hospital. She is American Board certified in Anesthesiology and Pediatric Anesthesiology and an active member of the Congenital Cardiac Anesthesia Society.

James A. DiNardo, MD graduated from Dartmouth College and Dartmouth Medical School (with honors). He completed his anesthesia residency and fellowship in Cardiac Anesthesia at Beth Israel Hospital in Boston. He joined faculty at Boston Children's Hospital in 1999 where he currently serves as the Chief of the Division of Cardiac Anesthesia. He spends approximately one third of this clinical time as an Attending in the Cardiac ICU.
Dr DiNardo is certified in perioperative TEE and is former Chairman of the PTEeXAM Committee. He is also a Senior Oral Board Examiner for the ABA, a member of the ABA Pediatric Examination Writing Committee, and Chair of the Pediatric MOCA Examination Committee. He is Past-President of both the Congenital Cardiac Anesthesia Society and the Association of Cardiac Anesthesiologists. He is currently the Anesthesia and Analgesia Executive Editor for Pediatric Anesthesia.
He is the author of a major textbook on cardiac anesthesia and of more than 160 peer-reviewed articles and book chapters.

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, Single¿Ventricle Physiology, and Inter¿Circulatory Mixing 9

Shunting 9

Effective Blood Flow 10

Total Pulmonary Blood Flow/Total Systemic Blood Flow 10

Single¿Ventricle Physiology 10

Intercirculatory Mixing 13

Classification of Anatomic Shunts 15

Simple Shunts 15

Complex Shunts 15

Pulmonary Vascular Pathophysiology 18

Causes of Pre¿Capillary PH in CHD 18

Causes of Post¿Capillary 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

End¿Tidal CO2 32

Central Venous Pressure 32

Temperature 33

Near¿InfräRed 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

Right¿Heart Catheterization 39

Right Atrial Pressure (RAP) Waveform 39

Right Ventricular Pressure (RVP) Waveform 39

Pulmonary Artery Pressure (PAP) Waveform 40

Left¿Heart 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

Veno¿Venous (VV)¿ECMO 59

Veno¿Arterial (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 Pre¿Bypass Management 64

Post¿VAD 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

Non¿Cardiac Surgery 71

9 Aortopulmonary Window 73

Introduction 73

Anatomy 73

Physiology 73

Surgical Management 73

Catheterization Laboratory Intervention 74

Anesthetic Management 75

Non¿Cardiac 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 Cross¿Clamp Removal 82

Non¿Cardiac 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

Post¿CPB Management 86

Non¿Cardiac 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 Canal¿Type Defects 89

Muscular Defects 89

Physiology 89

Surgical Therapy 91

Catheterization Laboratory Intervention 91

Anesthetic Management 91

Induction and Maintenance 91

Post¿CPB Management 91

Non¿Cardiac 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

Post¿CPB Management 96

Non¿Cardiac Surgery 96

14 Double Outlet Right Ventricle 97

Introduction 97

Anatomy 97

Physiology 99

Surgical Therapy 100

Anesthetic Management 101

Induction and Maintenance 101

Post¿CPB 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

Post¿CPB 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

Post¿CBP Management 110

Goals 110

Long¿Term 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

Post¿CPB 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

Pre¿Induction 119

Induction and Maintenance 119

Post¿Cardiopulmonary 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

Post¿CPB Management 123

19 Pulmonary Atresia/Intact Ventricular Septum (PA/IVS) 125

Introduction 125

Anatomy 125

Physiology 125

Surgical and Catheter¿Based Therapy 126

Anesthetic Management 129

Goals 129

Post¿CPB 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

Post¿CPB Management 138

Goals 138

Postoperative Junctional Ectopic Tachycardia 139

Non¿Cardiac Surgery 139

21 Tetralogy of Fallot with Pulmonary Atresia (TOF/PA) 141

Anatomy 141

Physiology 141

Surgical Therapy 141

Anesthetic Management 143

Post¿CPB Management 143

22 Tetralogy of Fallot with Absent Pulmonary Valve (TOF/APV) 145

Anatomy 145

Physiology...