Cover -- Title Page -- Copyright -- Contents -- List of Contributors -- Preface -- List of Abbreviations -- About the Companion Website -- Part I History, Education, Outcomes, and Science -- Chapter 1 History of Anesthesia for Congenital Heart Disease -- Introduction -- The first years: 1938-1954 -- The heart-lung machine: 1954-1970 -- The era of deep hypothermic circulatory arrest and the introduction of PGE1: 1970-1980 -- PDA and the introduction of PGE1 -- The story of HLHS: 1980-1990 -- Fontan and the catheterization laboratory: 1990-2000 -- Emergence of technology, including imaging (TEE, MRI) and ECMO: 2000-2010 -- 2011-2015 and the future -- CHD - a growing specialty from the fetus to the adult patient -- Selected references -- Chapter 2 Education for Anesthesia in Patients with Congenital Cardiac Disease -- Introduction -- Why teach and learn congenital cardiac anesthesia? -- The current model -- Curriculum for learning and teaching congenital cardiac anesthesia -- Problem identification and general needs assessment -- Targeted needs assessment -- Goals and objectives -- Educational strategies -- Implementation -- Evaluation and feedback -- Curriculum maintenance and enhancement -- Dissemination -- Role of professional societies -- Conclusion -- Selected References -- Chapter 3 Quality, Outcomes, and Databases in Congenital Cardiac Anesthesia -- Introduction -- Errors and outcomes in surgery and anesthesia -- The six "Cs": communication and teamwork -- Databases in pediatric cardiac surgery and anesthesiology -- Prospective risk assessment in pediatric cardiac surgery and cardiology -- Closed claims analysis in anesthesia -- Pediatric and congenital cardiac anesthesia morbidity and mortality -- CCAS and the Congenital Cardiac Anesthesia Network (CCAN) -- Joint CCAS-STS database initiative.

International efforts -- Selected References -- Chapter 4 Development of the Cardiovascular System and Nomenclature for Congenital Heart Disease -- Introduction -- Development of the cardiovascular system -- New concept of cardiac development -- Cardiovascular development: normal and abnormal -- Summary - development of the cardiovascular system -- Nomenclature for congenital heart disease -- Sequential segmental approach to diagnosis -- Morphologic and segmental anatomy -- Right atrium -- Left atrium -- Morphologic right ventricle -- Morphologic left ventricle -- Summary - nomenclature for CHD -- Selected References -- Chapter 5 Physiology and Cellular Biology of the Developing Circulation -- Introduction -- Development from fetus to neonate -- Circulatory pathways -- Myocardial contractility -- Development from neonate to older infant and child -- Gene expression in cardiac development -- The extracellular matrix -- Cell-to-cell connectivity -- Innervation of the heart -- Development from child to adult -- Normal values for physiologic variables by age -- Myocardial sequelae of longstanding CHD -- Cardiomyocyte receptor function in normal and diseased hearts -- The adrenergic receptor -- Developmental changes in adrenergic receptor signaling -- Calcium cycling in the normal heart -- Developmental changes in calcium cycling -- Thyroid hormone -- Regulation of vascular tone in systemic and pulmonary circulations -- Pulmonary circulation -- Systemic circulation -- Receptor signaling in myocardial dysfunction, CHD, and heart failure -- Receptor signaling in acute myocardial dysfunction -- Receptor signaling in CHD -- Receptor signaling in congestive heart failure and cardiomyopathy -- Myocardial preconditioning -- Selected References.

Chapter 6 Anesthetic Agents and Their Cardiovascular Effects -- Introduction -- Volatile agents -- Nitrous oxide (N2O) -- Opioids and benzodiazepines -- Propofol -- Ketamine -- Etomidate -- Dexmedetomidine -- Special conditions affecting anesthetic pharmacokinetics and pharmocodynamics in congenital cardiac anesthesia -- Intracardiac shunts -- Cardiopulmonary bypass -- Hypothermia -- Neuromuscular blocking agents and antagonists -- Succinylcholine -- Pancuronium -- Vecuronium -- Rocuronium -- Atracurium and cisatracurium -- Antagonists -- Selection of anesthetic regimen in CHD -- Selected References -- Chapter 7 Cardiopulmonary Bypass -- Introduction -- Basic bypass circuit setup -- Cannulation and tubing -- Pumps -- Oxygenator -- Priming -- Differences between pediatric and adult CPB circuitry -- Hemodilution -- Perfusion pressures -- Flow rates -- Blood gas management -- Aortopulmonary collaterals -- Temperature ranges -- Glucose management -- Management of pediatric CPB -- Stages of CPB -- Pre-bypass period -- Anticoagulation and hemostatic management -- Initiation of CPB and flow requirements -- Cooling and temperature management -- Aortic cross-clamping, myocardial ischemia, and protection -- Induction and maintenance of cardioplegic cardiac arrest -- Reperfusion -- Separation from CPB and post-bypass phase -- Conventional ultrafiltration and modified ultrafiltration -- Failure to separate from CPB -- Heparin reversal and transfusion management -- Antifibrinolytic therapy -- Effects of CPB on organ systems -- Neurological injury and protection -- Pulmonary effects -- Renal, hepatic, and gastrointestinal effects -- Endocrine and metabolic response to CPB -- Special CPB management issues -- Warm CPB -- Deep hypothermic circulatory arrest -- Regional cerebral perfusion.

Blood gas management: pH-stat vs. alpha-stat -- Sickle cell disease -- Cold agglutinins -- Leukoreduction and irradiation of blood products -- Monitoring anticoagulation during and after CPB -- Complications and safety -- Conclusions and future perspectives -- Selected references -- Chapter 8 Multiorgan Effects of Congenital Cardiac Surgery -- Introduction -- The systemic inflammatory response and congenital cardiac surgery -- Systems mediating the systemic inflammatory response to CPB -- Mitigating the deleterious inflammatory effects of CPB -- Corticosteroids -- Modified ultrafiltration -- Serine protease inhibitors -- Heparin-bonded circuits -- Transfusion and inflammation -- Effects of congenital heart surgery on coagulation, hemostasis, and thrombosis -- Disturbances in the coagulation system in pediatric cardiac surgery -- Unique aspects of pediatric coagulation -- Hemostasis in CHD -- Pediatric patients and CPB -- Risk factors for hemorrhagic complications in pediatric cardiac surgery -- Optimization of transfusion management in pediatric cardiac surgery -- Thrombosis in pediatric cardiac surgery -- Pulmonary effects of congenital heart surgery -- Neonatal and pediatric pulmonary physiology: a compromised state -- Metabolic compromise -- Mechanical compromise -- Ventilatory compromise -- Compromise of neonatal oxygen delivery -- Compromise arising from CHD -- Effects of CPB on pulmonary physiology in children -- Renal effects of congenital heart surgery -- Introduction, incidence, and definition of the problem -- Emerging biomarkers of renal dysfunction -- Association of renal injury with clinical outcomes -- Renal failure and renal replacement therapy following cardiac surgery in children -- Peritoneal dialysis -- Continuous veno-venous hemofiltration.

Gastrointestinal and hepatic consequences of cardiac surgery in children -- Splanchnic circulation -- Hepatic dysfunction -- Endocrine response and pediatric cardiac surgery -- Diagnosis of CIRCI -- Management of CIRCI -- Stress hyperglycemia -- Thyroid hormone -- Selected References -- Chapter 9 Anesthetic and Sedative Neurotoxicity in the Patient with Congenital Heart Disease -- Introduction -- Preclinical studies in animal and tissue culture models -- Mechanisms of anesthesia-induced neurotoxicity -- Mitochondria, oxidative stress, and the intrinsic apoptosis pathway -- The extrinsic apoptosis pathway -- Brain-derived neurotrophic factor -- Cell age-specific vulnerability -- Summary of human cardiac cohorts -- Experimental therapy in preclinical studies -- Anesthetic preconditioning -- Dexmedetomidine -- Erythropoietin -- Lithium -- Vitamins, anti-inflammatory agents, and antioxidants -- Hypothermia -- Carbon monoxide -- Xenon -- Future therapy -- Preconditioning -- Dexmedetomidine -- Hypothermia -- Low-flow anesthesia -- Directions for future research -- Conclusion -- Selected references -- Part II Monitoring -- Chapter 10 Vascular access and monitoring -- Introduction -- Venous access -- Peripheral venous access -- Central venous access -- Arterial access -- Radial artery -- Femoral artery -- Brachial artery -- Axillary artery -- Umbilical artery -- Temporal artery -- Dorsalis pedis/posterior tibial arteries -- Ulnar artery -- Arterial cutdown -- Ultrasound guidance for vascular access in congenital heart surgery -- Sonoanatomy -- Ultrasound-guided peripheral vein access -- Ultrasound-guided central vascular access -- Ultrasound-guided arterial catheterization -- Percutaneous PA catheterization -- Interpretation of intravascular pressure waveforms -- Arterial pressure waveform.

Central venous and left atrial waveforms.