Anesthesia for Congenital Heart Disease

Description

Anesthesia forCongenital Heart Disease An Extensive Reference Work Detailing the Procedures, Knowledge, and Approaches in Anesthesia for Congenital Heart Disease In this fourth edition of Anesthesia for Congenital Heart Disease, a distinguished group of expert authors in congenital heart disease (CHD) from all over the world provide a thorough, comprehensive, and fully updated overview of the specifics of congenital heart disease and the intricacies involved with administering anesthetic care to patients who suffer from the myriad of lesions encompassed by CHD. In the seven years since the third edition of Anesthesia for Congenital Heart Disease was published, an explosion of new procedures, approaches, and patients eligible for anesthetic care has occurred. The goal of Anesthesia for Congenital Heart Disease, Fourth Edition is to help readers understand all of the recent advancements and developments in the field while also imparting a foundation of essential historical knowledge. The book contains new chapters that reflect exciting new approaches in this rapidly changing field, and also includes: Updated information in the chapter Adult Congenital Heart Disease and Mechanical Support of the Circulation, reflecting the increasing prominence of these patients in CHD care New chapters on Informatics and Artificial Intelligence, Genetic Syndromes, Point of Care Ultrasound, and Cardiopulmonary Resuscitation in Congenital Heart Disease Thorough updates of all chapters with many new figures and tables, and hundreds of new recent references provide up to date information Anesthesia for Congenital Heart Disease, Fourth Edition serves as a thorough and in-depth reference work and is an essential resource for practitioners providing perioperative care to CHD patients. Cover Title Page Copyright Page Contents List of Contributors Preface to the Fourth Edition List of Abbreviations About the Companion Website CHAPTER 1 History of Anesthesia for Congenital Heart Disease Introduction The first years: 1938–1954 The heart–lung machine: 1954–1970 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 new technology – imaging (TEE, MRI) and ECMO: 2000–2010 2011–2021 and the future Congenital heart disease – a growing specialty from the fetus to the adult patient Acknowledgments Selected references CHAPTER 2 Education for Anesthesia in Patients with Congenital Cardiac Disease Introduction Why teach and learn congenital cardiac anesthesia? The current model Pediatric cardiac anesthesia training 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 Acknowledgments 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 Congenital Cardiac Anesthesia Society and the Congenital Cardiac Anesthesia Network Joint CCAS–STS database initiative International efforts Selected references CHAPTER 4 Multidisciplinary Collaboration, Team Functioning, and Communication in Congenital Cardiac Care Introduction Texas Children’s Heart Center multidisciplinary approach Heart Center leadership structure Clinical decision-making Heart Center Quality Improvement Conferences Heart Center Wide Communication Activities Anesthesiologist involvement in patient care and leadership activities Communication and team functioning in periprocedural areas: surgery and catheterization laboratory Communication in the operating room Communication during the operation Other intraoperative multidisciplinary collaborations Intensive care unit handoffs The cardiac catheterization laboratory Enhanced recovery after cardiac surgery TCH handbook Parent and family involvement in congenital cardiac care Heart Center functioning during the COVID-19 pandemic Conclusions Selected references CHAPTER 5 Informatics and Artificial Intelligence in Congenital Heart Disease Introduction History of medical informatics Principles, terminology, and technologies How to work with big data How is data represented Data types within a hospital Databases, data organization, and querying data Data analysis and processing pipelines Machine learning and artificial intelligence Supervised learning algorithms Unsupervised learning algorithms Deep learning algorithms Advanced monitors in intensive care units and operating rooms Limitations and challenges Future of clinical informatics in congenital heart disease Selected references CHAPTER 6 Development of the Cardiovascular System Introduction Contemporary concepts of cardiac development Cardiovascular development: normal and abnormal Cardiogenic fields Formation of the heart tube Cardiac looping Cardiac septation Epicardium and coronary artery development Development of the conduction system Development of the aortic arches Development of the pulmonary and systemic veins Development of cardiac innervation Development of the cardiac lymphatics Summary Selected references CHAPTER 7 Nomenclature and Anatomic Evaluation in Congenital Heart Disease Introduction Congenital heart surgery nomenclature and database project Approaches for describing congenital heart disease The “Van Praaghian” and “Andersonian” nomenclature systems The Van Praagh notation Segmental approach to diagnosis of congenital heart disease Cardiac position and apex orientation Visceral and atrial situs Great veins and atria Ventricles and ventricular septum Atrioventricular junction Atrioventricular valves Atrioventricular connections Ventriculoarterial junction Conal or infundibular anatomy Semilunar valves Ventriculoarterial connections Great arteries and their relationships Branch pulmonary arteries and ductus arteriosus Aortic arch Coronary arteries Description of associated malformations Summary Selected references CHAPTER 8 Genetic Syndromes and Associations in Congenital Heart Disease Introduction Chromosome 22q11 deletion syndrome Trisomy 21 Trisomy 18 and 13 Williams-Beuren syndrome and Elastin arteriopathies Noonan syndrome Turner syndrome Goldenhar syndrome Mucopolysaccharidoses and glycogen storage diseases Aortopathy syndromes Marfan syndrome Loeys-Dietz syndrome Ehlers-Danlos syndrome VACTERL association CHARGE syndrome Heterotaxy syndrome Table of genetic syndromes and associations Genetic testing in CHD Chromosomal microarray (CMA) Fluorescence in-situ hybridization (FISH) Chromosomal karyotype Single gene sequencing Whole exome sequencing Ethics in congenital heart disease Ethical considerations for treatment Palliative care in CHD Conclusions Selected references CHAPTER 9 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 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 Stem cell and other cell-based therapy for congenital heart disease Selected references CHAPTER 10 Anesthetic Agents and Their Cardiovascular Effects Introduction Volatile agents Nitrous oxide (N2O) Opioids and benzodiazepines Propofol Ketamine Etomidate Dexmedetomidine Neuromuscular blocking agents and antagonists Succinylcholine Pancuronium Vecuronium Rocuronium Atracurium and cisatracurium Antagonists Special conditions affecting anesthetic pharmacokinetics and pharmacodynamics in congenital cardiac anesthesia Intracardiac shunts Cardiopulmonary bypass Hypothermia Selection of anesthetic regimen in CHD Selected references CHAPTER 11 Cardiopulmonary Bypass Introduction Basic bypass circuit setup Cannulation and tubing Pumps Oxygenator Priming Differences between pediatric and adult CPB Hemodilution Perfusion pressures Flow rates 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 Emergency cardiopulmonary bypass Conclusions and future perspectives Selected references CHAPTER 12 Multiorgan Effects of Congenital Cardiac Surgery Introduction The systemic response to congenital cardiac surgery Systems mediating the systemic inflammatory response to CPB Mitigating the deleterious effects of CPB Corticosteroids Modified ultrafiltration Protease inhibitors Biocompatible CPB circuits Clinical effects of congenital heart surgery on hemostasis and thrombosis Unique aspects of pediatric coagulation Hemostasis in congenital heart disease Risk factors for hemorrhagic complications in pediatric cardiac surgery Transfusion, inflammation, and outcomes Transfusion in pediatric cardiac surgery Antifibrinolytics in pediatric cardiac surgery MUF in pediatric cardiac surgery Other methods to minimize transfusion Other hemostatic agents Thrombosis in pediatric cardiac surgery Risk factors for thrombosis in children with CHD Pulmonary effects of congenital heart surgery Neonatal and pediatric pulmonary physiology: a compromised state Effects of CPB on pulmonary physiology in children CPB and lung parenchyma Techniques to improve respiratory function 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 GI and hepatic consequences of cardiac surgery in children Splanchnic circulation Endocrine response and pediatric cardiac surgery Critical illness-related corticosteroid insufficiency Stress hyperglycemia The risk of hypoglycemia Thyroid hormone Selected references CHAPTER 13 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 Newer techniques in pediatric intravascular monitoring Cardiac output monitoring Central venous oxygen saturation monitoring Complications of vascular access Incidence and risk factors Thrombosis Infection Malposition/perforation Pneumothorax/hemothorax Cardiac tamponade Inadvertent arterial puncture or catheter placement Arrhythmias Systemic venous air embolus Foreign bodies Complications related to intracardiac catheters Other complications Conclusions Selected references CHAPTER 14 Neurological Monitoring and Outcome Introduction Cerebrovascular physiology during cardiac surgery Cooling and rewarming pH-stat vs. alpha-stat blood gas management Hemodilution and transfusion practices during CPB Temperature management Glucose management Circulatory arrest Selective cerebral perfusion Selective cerebral perfusion and flow rates Neurological monitoring during congenital heart surgery Electroencephalographic technologies Transcranial Doppler ultrasound Monitors of cerebral oxygenation Monitors of cerebral autoregulation Anesthetic and sedative neurotoxicity Longer term neurodevelopmental testing outcomes after congenital heart surgery Boston circulatory arrest study Children’s hospital of Philadelphia cohort Western Canadian study Hearts and minds study Milwaukee cohort Texas children’s hospital cohort Single ventricle reconstruction trial International cardiac collaborative on neurodevelopment (ICCON) investigators cohort Zurich cohort Conclusions Does a neuroprotective strategy during CPB improve outcome? Selected references CHAPTER 15 Transesophageal and Epicardial Echocardiography in Congenital Heart Disease Introduction Indications for TEE in CHD Contraindications to TEE TEE hardware Echocardiographic system TEE imaging probes TEE examination in CHD Structural examination Three-dimensional TEE Functional assessment Hemodynamic evaluation Applications of TEE in CHD TEE safety and complications Evaluation of selected congenital heart defects before and after intervention Atrial septal defects Ventricular septal defects Atrioventricular septal defects LV outflow obstruction RV outflow obstruction Tetralogy of Fallot Double-outlet right ventricle Transposition of the great arteries Congenitally corrected transposition of the great arteries Truncus arteriosus Single ventricle Applications of TEE during mechanical support Epicardial echocardiography Summary Acknowledgments Selected references CHAPTER 16 Coagulation, Cardiopulmonary Bypass, and Bleeding Introduction Coagulation Mechanisms of hemostasis Developmental hemostasis Influence of congenital heart disease on coagulation Preoperative considerations Predictors of bleeding post-CPB Preoperative laboratory testing Preoperative anemia Autologous donation CPB-associated coagulation changes Exposure to CPB circuit Hemodilution and CPB prime Dilutional anemia Dilution of coagulation factors Composition of CPB prime Anticoagulation Heparin-induced thrombocytopenia and alternatives to heparin Ultrafiltration Management of bleeding Cell salvage Use of coagulation tests and transfusion algorithms Blood transfusion Whole blood Red blood cells Platelet transfusion Fibrinogen supplementation Recombinant factor VIIa Factor concentrates Pharmacologic therapies Antifibrinolytics Desmopressin Sickle cell disease Summary Selected references CHAPTER 17 Point-of-Care Ultrasound for Congenital Heart Disease Patients Introduction What is POCUS? Pediatric cardiac POCUS Basic physics of POCUS Performing cardiac POCUS Parasternal long-axis view Parasternal short-axis view Apical four-chamber view Subcostal four-chamber and inferior vena cava views Individual structure assessment using cardiac POCUS Assessment of the left ventricle Assessment of the right ventricle Assessment of pericardial space and early tamponade physiology Assessment of the atria and valves Assistance during cardiac arrest Assistance during central line placement Specific clinical situations and corresponding limitations POCUS during assessment of shock Performing lung POCUS Structures by artifact visualization Incorporation of lung ultrasound into anesthesia practice POCUS training and credentialing Selected references CHAPTER 18 Preoperative Evaluation and Preparation Introduction The patient with congenital heart disease Terminology and classification Multidisciplinary approach Consent Preoperative evaluation History and physical examination Congestive heart failure Pulmonary arterial hypertension The neonates and premature infants Medications Electrocardiographic evaluation, pacemakers, and defibrillators Laboratory evaluation Preoperative imaging studies Chest radiography Echocardiography Cardiac catheterization Magnetic resonance imaging Computed tomography Choice of echocardiography, CT, or MRI for non-invasive assessment of CHD Head ultrasound and other brain imaging modalities Preoperative preparation Preoperative fasting Preoperative psychological preparation and premedication Infective endocarditis antibiotic prophylaxis Sickle cell disease Risk stratification Selected references CHAPTER 19 Approach to the Fetus, Premature, and Full-Term Neonate Approach to treatment in the neonate Early palliation Systemic-to-pulmonary artery shunt Transcatheter ductus arteriosus stent Banding of the pulmonary artery The case for early complete repair Special considerations for the neonate Limited physiologic reserve Stress response Systemic inflammatory response to CPB Neurologic injury Premature infants and low birth weight neonates Pulmonary function Cardiac function Necrotizing enterocolitis Intraventricular hemorrhage Outcome after congenital heart surgery Patent Ductus arteriosus occlusion in premature neonates Fetal cardiac intervention Transplacental pharmacotherapy of the fetus Open fetal cardiac interventions Catheter-based fetal cardiac interventions Delivery room emergencies in complex congenital heart disease Selected references CHAPTER 20 Anesthesia for Adults with Congenital Heart Disease Introduction Noncardiac sequelae of CHD Pulmonary sequelae Hematological sequelae Renal sequelae Neurological sequelae Hepatic sequelae Vascular access considerations Unrestricted shunts Pregnancy Relevant physiologic changes during pregnancy Cardiac risk assessment before and during pregnancy Maternal obstetric and fetal risks Care during pregnancy Cardiac procedures during pregnancy Delivery planning Adults with down syndrome Psychological issues Cardiac lesions Atrial septal defects and Shunts Ventricular septal defect (VSD) Patent ductus arteriosus Coarctation of the Aorta Pulmonary valve stenosis (PS) Tetralogy of Fallot Pulmonary atresia Congenitally corrected transposition of the great arteries Dextro-transposition of the great arteries (D-TGA) Ebstein’s anomaly Single-ventricle anatomy/Fontan physiology Heart failure in ACHD Noncardiac surgery in the adult with CHD Conclusions Selected references CHAPTER 21 Hemodynamic Management Introduction: Hemodynamic management following congenital heart disease surgery: a goal-directed approach The VO2/DO2 balance following CHD surgery Mechanisms underlying increases in VO2 following CHD surgery Mechanisms underlying reduced DO2 following CHD surgery Improving postoperative oxygen transport balance: preventive and therapeutic interventions Inotropic agents Vasoconstrictors Systemic vasodilators Pulmonary vasodilators Other strategies to improve circulatory function Current practices Inotropic and vasoactive scores Individualized perioperative hemodynamic management Cardiopulmonary interactions Effects of changes in intrathoracic pressure Individualized management of the ventilation parameters and cardiopulmonary interactions Monitoring Clinical hemodynamic variables and standard perioperative monitoring Assessment of cardiac output Near infra-red spectroscopy Mixed venous saturation Blood lactate Central venous to arterial CO2 difference Current practices Conclusion Selected references CHAPTER 22 Arrhythmias: Diagnosis and Management Introduction Cardiac rhythm disturbances Sinus bradycardia Low atrial rhythm Sinus node dysfunction Sinus tachycardia Junctional rhythm Conduction disorders Supraventricular arrhythmias Ventricular arrhythmias Pharmacologic therapy of cardiac arrhythmias Class I agents Class IA agents Class II agents Class III agents Class IV agents Other agents Pacemaker therapy in children Pacemaker nomenclature Permanent cardiac pacing Temporary cardiac pacing External cardiac (transcutaneous) pacing Transesophageal overdrive pacing Implantable cardioverter-defibrillators Summary Selected references CHAPTER 23 Airway and Ventilatory Management Introduction Choosing the appropriate endotracheal tube (ETT) Orotracheal vs. nasotracheal intubation The difficult airway Intubation of the patient with a difficult airway Fiberoptic-guided tracheal intubation Emergency cricothyrotomy The difficult extubation Airway and ventilatory management for thoracic surgery Ventilation / perfusion in the lateral decubitus position Single-lung ventilation (SLV) Ventilatory management during thoracic surgery Changes in lung function in children with CHD Changes in lung function from CPB Cardiopulmonary interactions Mechanical ventilation for children with CHD Lung management during CPB Volume control vs. pressure control ventilation Monitoring ventilation Anesthesia ventilators Operating room to ICU transition Specialized problems Hypoxic gas mixture and inspired CO2 Nitric oxide Tracheostomy in congenital heart disease Placement of tracheostomy tubes Cardiac surgery in patients with pre-existing tracheostomy Summary Selected references CHAPTER 24 Early Tracheal Extubation, Enhanced Recovery After Pediatric Cardiac Surgery, Regional Anesthesia and Postoperative Pain Management Introduction Background and history Enhanced recovery after pediatric cardiac surgery Feasibility of fast-tracking in congenital heart surgery Patient selection Implementation and maintaining fast-tracking Anesthesia technique Surgery and CPB considerations Failed extubation and prolonged mechanical ventilation Benefits of fast-tracking Concerns and safety of fast-tracking Postoperative considerations Neuraxial techniques Single-shot neuraxial techniques Catheter-based neuraxial techniques Potential benefits of neuraxial techniques Risks and complications of neuraxial techniques Regional blocks Conclusions Selected references CHAPTER 25 Cardiopulmonary Resuscitation in the Patient with Congenital Heart Disease Epidemiology Overview of current CPR guidelines CPR techniques Advanced airway interventions during CPR Drug administration during CPR Management of ventricular fibrillation and/or pulseless ventricular fibrillation Pediatric resuscitation in patients with suspected or confirmed COVID-19 infection Phases of cardiac arrest Post-cardiac arrest care Cardiopulmonary resuscitation for the congenital heart disease patient Left-heart lesions The underlying disease states Adjunctive CPR techniques for the CHD patient Simulation/education Team training Selected references CHAPTER 26 Anesthesia for Left-to-Right Shunt Lesions Introduction Patent ductus arteriosus Incidence, anatomy, and natural history Pathophysiology Surgical and transcatheter approaches and outcomes Anesthetic considerations Aortopulmonary window Incidence, anatomy, and natural history Pathophysiology Surgical and transcatheter approaches and outcomes Anesthetic considerations Atrial septal defects Incidence Anatomy Natural history Pathophysiology Surgical and transcatheter approaches and outcomes Anesthetic considerations Ventricular septal defects Incidence Anatomy Natural history Pathophysiology Surgical and transcatheter approaches and outcomes Anesthetic considerations Atrioventricular canal Incidence Anatomy Natural history Pathophysiology Surgical approaches and outcomes Anesthetic considerations Double outlet right ventricle Incidence Anatomy Natural history and pathophysiology Surgical approaches and outcomes Anesthetic considerations Truncus arteriosus Incidence Anatomy Natural history Pathophysiology Surgical and transcatheter approaches and outcomes Anesthetic considerations Partial and total anomalous pulmonary venous return Incidence Anatomy Natural history and pathophysiology Partial anomalous pulmonary venous return Total anomalous pulmonary venous return Surgical approaches and outcomes Anesthetic considerations Selected references CHAPTER 27 Anesthesia for Left–sided Obstructive Lesions Introduction Aortic valve stenosis Incidence, anatomy, and natural history Pathophysiology Surgical and transcatheter approaches and outcomes Anesthetic considerations Subvalvular AS Incidence, anatomy, and natural history Pathophysiology Surgical and transcatheter approaches and outcomes Anesthetic considerations Supravalvular AS Incidence, anatomy, and natural history Pathophysiology Surgical and transcatheter approaches and outcomes Anesthetic considerations Hypertrophic cardiomyopathy Incidence, anatomy, and natural history Pathophysiology Surgical and transcatheter approaches and outcomes Anesthetic considerations Coarctation of the aorta Incidence, anatomy, and natural history Pathophysiology Surgical and transcatheter approaches and outcomes Anesthetic considerations Interrupted or hypoplastic aortic arch Incidence, anatomy, and natural history Pathophysiology Surgical and transcatheter approaches and outcomes Anesthetic considerations Shone’s anomaly Incidence, anatomy, and natural history Pathophysiology Surgical and transcatheter approaches and outcomes Anesthetic considerations Mitral stenosis Incidence, anatomy, and natural history Pathophysiology Surgical and transcatheter approaches and outcomes Anesthetic considerations Cor triatriatum Selected references CHAPTER 28 Anesthesia for Right-Sided Obstructive Lesions Introduction Ebstein anomaly Incidence, anatomy, and natural history Pathophysiology Surgical and transcatheter approaches and outcomes Anesthetic considerations Tetralogy of Fallot Incidence, anatomy, and natural history Pathophysiology Surgical and transcatheter approaches and outcomes Anesthetic considerations Pulmonary stenosis with intact ventricular septum Incidence, anatomy, and natural history Pathophysiology Surgical and transcatheter approaches and outcomes Anesthetic considerations Pulmonary atresia with intact ventricular septum Incidence, anatomy, and natural history Pathophysiology Surgical and transcatheter approaches and outcomes Anesthetic considerations Pulmonary atresia/ventriculoseptal defect/major aortopulmonary collateral arteries Incidence, anatomy, and natural history Pathophysiology Surgical and transcatheter approaches and outcomes Anesthetic considerations Summary Acknowledgements Selected references CHAPTER 29 Anesthesia for Transposition of the Great Arteries Introduction Physiologically uncorrected TGA Incidence Anatomy Coronary artery anatomy Natural history and pathophysiology Shunting and effective pulmonary and systemic blood flow in TGA Intercirculatory mixing Clinical presentation and diagnostic features Outcomes from balloon atrial septostomy Anesthesia for balloon atrial septostomy Surgical options Indication and timing of ASO Arterial switch operation Other surgical procedures Outcomes for ASO Anesthetic considerations and perioperative management Congenitally corrected TGA (L-TGA) Incidence Anatomy Natural history Pathophysiology Clinical presentation and diagnostic features Surgical options Outcomes Anesthetic considerations and perioperative management Preoperative assessment Postoperative course and complications Selected references CHAPTER 30 Anesthesia for the Patient with a Single Ventricle Introduction Hypoplastic left heart syndrome Incidence, anatomy, and natural history Pathophysiology of HLHS Surgical and transcatheter approaches and outcomes Anesthetic considerations Tricuspid atresia Incidence, anatomy, and natural history Pathophysiology of TA Surgical and transcatheter approaches and outcomes Anesthetic considerations Other forms of univentricular hearts Double inlet ventricle Mitral atresia with VSD Unbalanced complete atrioventricular septal defect Single functional ventricle with heterotaxy syndrome Superior cavopulmonary connection Surgical procedure Anesthetic considerations Outcomes Fontan completion Surgical procedure Anesthetic considerations Long term complications and outcomes Transplantation for single-ventricle patients Heart transplantation Post-Fontan heart-liver transplantation Non-cardiac surgery and anesthesia for the patient with a single ventricle Summary Selected references CHAPTER 31 Anesthesia for Miscellaneous Cardiac Lesions Introduction Vascular rings Classification, anatomy, and incidence Diagnosis of vascular rings Vascular rings due to double and right aortic arches: anatomy and natural history Innominate artery compression of the trachea: anatomy, diagnosis, and natural history Pulmonary artery sling with tracheal stenosis: anatomy and natural history Anomalies of the coronary arteries Anomalous pulmonary origins of the coronary arteries: incidence, anatomy, and natural history Anomalous aortic origins of the coronary arteries Congenital atresia of the left main coronary artery Coronary arteriovenous fistulas: incidence, anatomy, and natural history Coronary artery bridging, aneurysms, and stenosis Pericardial effusion and tamponade Incidence, anatomy, and natural history Pathophysiology and diagnosis of cardiac tamponade Surgery for pericardial effusion and cardiac tamponade Anesthesia for pericardial effusion and cardiac tamponade Mitral regurgitation Incidence, anatomy, and natural history of mitral regurgitation Normal anatomy and function of the mitral valve Classification of mitral regurgitation Mechanisms of mitral valve regurgitation Pathophysiology of MR Surgical approaches and outcomes for MR in children Anesthetic considerations for the repair of MR Cardiac tumors in childhood Incidence, anatomy, and natural history Pathophysiology of cardiac tumors Surgical approaches and outcomes for cardiac tumors Anesthesia considerations for cardiac tumors Aortic aneurysm and aortopathy in children Classification and definition of aortic aneurysms (and aortopathy) Incidence, anatomy, and natural history of aortic aneurysms in children Genetic conditions associated with aortic aneurysm Sinus of Valsalva aneurysms Aortopathy and aortic dilation with CHD Pathophysiology of aortic aneurysms Surgical approaches for aortic aneurysms in children Anesthesia for aortic aneurysm surgery Mediastinal masses Incidence, anatomy, and natural history of mediastinal masses in children Diagnosis of anterior mediastinal masses Pathophysiology Surgery for anterior mediastinal masses Anesthesia for patients with an anterior mediastinal mass Selected references CHAPTER 32 Anesthesia for Cardiac and Pulmonary Transplantation Heart transplantation Diagnostic indications for heart transplantation ABO-incompatible heart transplantation Recipient evaluation Recipient pretransplant management Donor management Surgical technique Anesthetic management Failing Fontan transplants Combined heart and liver transplantation Heart–lung transplantation Immunosuppression Anesthetic management of children who have undergone heart transplantation Future prospects Pediatric lung transplantation Indications, contraindications, and listing criteria in children Lung transplant listing criteria Donor management and lung preservation Bridge to transplantation Anesthetic management and surgical approach Immediate perioperative complications Primary graft failure Physiological changes and growth of the transplanted lungs Surgical complications Medical complications Selected references CHAPTER 33 Anesthesia for Pulmonary Hypertension Incidence, anatomy, natural history Definition Classification Epidemiology Pathophysiology of pulmonary hypertension in congenital heart disease Assessment of pulmonary hypertension Medical management of pulmonary hypertension Calcium channel blockers (CCBs) Prostanoids Endothelin receptor antagonists (ERA) Phosphodiesterase-5 inhibitors (PDE-5i) Atrial septostomy and lung transplantation Perioperative risk considerations Pulmonary hypertensive crisis Anesthetic management Hemodynamic effects of anesthetic drugs Perioperative pulmonary vasodilators Airway and ventilation management Monitoring Postanesthesia disposition Conclusions Selected references CHAPTER 34 Anesthesia for the Cardiac Catheterization Laboratory Introduction Diagnostic catheterization Procedure overview Endomyocardial biopsy Pulmonary hypertension study Cardiac magnetic resonance imaging-guided catheterization Interventional catheterization Device closure of cardiovascular communications Balloon dilation of cardiac valves Balloon angioplasty and/or stent placement Creation of cardiovascular communications Transcatheter valve replacement Hybrid procedures Pericardiocentesis Percutaneous mechanical circulatory support Adverse events Risk assessment Anesthetic management Operator-directed sedation versus anesthesia care Periprocedural planning Anesthetic techniques Anesthetic drugs Monitoring Intravascular volume management Recovery Electrophysiology procedures Electrophysiology studies and ablations Anesthetic considerations Anesthetic drugs and the cardiac conduction system Implantation of pacemakers and defibrillators Elective cardioversion Transvenous lead extractions Conclusions Selected references CHAPTER 35 Anesthesia for Noncardiac Surgery and Magnetic Resonance Imaging Introduction Risk assessment and stratification Preoperative preparation for noncardiac surgery Multidisciplinary planning Preoperative cardiology visit Interpretation of imaging and hemodynamics Nil per os Continuation of medications Endocarditis prophylaxis Pacemakers and defibrillators The patient on ventricular assist device support Management strategies for the stages of single-ventricle palliation High-risk patient groups Ductal dependent unpalliated neonates Shunt-dependent single-ventricle patients Palliated single-ventricle patients with atrioventricular valve regurgitation Pulmonary hypertension with systemic or supra-systemic PA pressure Severe aortic stenosis (peak gradient >60 mmHg) Williams syndrome Hypertrophic cardiomyopathy Transplant coronary artery disease Eisenmenger syndrome Adult congenital heart disease Intraoperative care Monitoring Anesthetic technique Surgery Complex surgical situations Radiologic procedures Magnetic resonance imaging and computed tomography Lymphatic mapping and intervention Postoperative considerations Intensive care unit vs. general inpatient unit Home discharge criteria Selected references CHAPTER 36 Cardiac Intensive Care Introduction Pathophysiology of specific congenital cardiac defects and implications Intercirculatory mixing, complete mixing, and streaming Shunts Outflow obstruction Airway and ventilation management Airway management Mechanical ventilation Cardiorespiratory interactions Influence of lung volume Influence of intrathoracic pressure Positive end-expiratory pressure Alternative modes of ventilation and respiratory support Early extubation Weaning from mechanical ventilation Myocardial dysfunction and hemodynamic monitoring Assessment of cardiac output Surgical factors Cardiopulmonary bypass and the systemic inflammatory response Dysrhythmias Low preload High afterload Decreased myocardial contractility Delayed sternal closure Cardiopulmonary resuscitation in the cardiac intensive care unit Prearrest phase: monitoring and event risk reduction Arrest phase: no-flow and low-flow states Postresuscitation phase: patient-focused care Postresuscitation phase: team debriefing Management of postoperative complications Patient safety and quality improvement in the cardiac intensive care unit Mechanical support of the circulation Extracorporeal membrane oxygenation Ventricular assist devices Hemostasis Infection control Neurologic monitoring/assessment and complications Fluid management and renal dysfunction Nutrition and gastrointestinal complications SARS-CoV-2 infection and multisystem inflammatory syndrome in children Selected references CHAPTER 37 Mechanical Circulatory Support Background, introduction, and history Indications for mechanical support Preoperative stabilization and support Failure to wean from CPB or LCOS after cardiac surgery Resuscitation of cardiac arrest Respiratory failure and lung transplantation Sepsis Myocarditis, cardiomyopathy, and cardiac transplantation Arrhythmias with hemodynamic compromise Cardiac catheterization instability Pulmonary hypertension Intoxicants Mechanical support to assist organ donation Contraindications to mechanical support in children Devices Extracorporeal membrane oxygenation (ECMO) Ventricular assist devices Role of echocardiography in mechanical support Extracorporeal membrane oxygenation Ventricular assist devices Weaning from circulatory support ECMO weaning VADs: weaning from CPB to VAD VADs: weaning from VAD to recovery Anesthesia, analgesia, and sedation for mechanical support Drug disposition changes on mechanical support Anesthesia, analgesia, and sedation for mechanical support patients undergoing NonCardiac surgery Preoperative management Intraoperative management Postoperative management Anticoagulation, antifibrinolytics, and platelet antiaggregation therapies Anti-infective therapy Outcomes and complications of extracorporeal support ECMO for cardiac support ECMO for respiratory support ECMO complicated by sepsis Overall long-term survival Neurological outcome Quality of life Comparing outcomes and complications of VAD and ECMO Other outcome issues The future Tomorrow ECMO Tomorrow VAD Selected references Appendix: Pediatric Cardiovascular Anesthesia Drug Sheet (September 2022) References Index EULA