Congenital Heart Disease in Pediatric and Adult Patients: Anesthetic and Perioperative Management

Foreword
Preface
Contents
History of Pediatric Anesthesia and Pediatric Cardiac-Congenital Surgery
	The Birth of Pediatrics and “Children’s Hospitals” in the United States
	Surgical Pioneers in Pediatric Surgery
	Origins of Pediatric Anesthesiology
	Developments Leading to Pediatric Heart and Congenital Surgery
	Future Directions
	References
Cardiovascular System Embryology and Development
	Establishing Cardiac Crescent
	Formation of the Heart Tube
	The Looping of Cardiac Tissue
	The Formation of the Sinus Venosus
	The Next Steps in Differentiation: Atrial Development
	The Primordial Heart Septation
	Atrioventricular Valves
	Septum Development in the Truncus Arteriosus and Conus Cordis
	Semilunar Valves Formation
	Development of the Cardiac Conducting System in the Heart
	Atrioventricular (AV) Valve Anomalies
	Stenosis of Cardiac Semilunar Valves
	Cardiac Outflow Tract Septation Anomalies
	Vascular Formation
	Arterial System
		Development of Aortic Arches
		Development of Arterial Tree from Aortic Arches
	Fate of the Vitelline and Umbilical Arteries
	Dorsal Aorta Give Rise Lateral Branches
	Intersegmental Branches
	Formation of Limb Arteries
	The Formation of Coronary Arteries
	The Formation of Venous System
	The Formation of Lymphatic System
	Development of Lymph Sacs and Lymphatic Ducts
	The Development of Thoracic Duct
	Circulation Before and After Birth
		Fetal Circulation
		Circulatory Alterations after Birth
	Angiomas
	Abnormalities of the Ductus Arteriosus
	Aortic Coarctation
	Vena Cava Anomalies
	IVC Anomalies
	SVC Anomalies
		Left Superior Vena Cava
	Lymphatic System Anomalies
	References
Pediatric Cardiovascular Physiology
	Evolutional Transition in Cardiac Physiology
		Fetal Circulation and Its Developmental Changes
		Changes in Circulation at Birth
	Myocardial Electromechanical Function
		Electrical Function of the Myocardium: Action Potential
			Action Potential in Cardiac Cells
			Cardiac Automaticity and Its Mechanism(s)
		Excitation-Contraction Coupling: ECC
		Mechanical (Contractile) Function of the Myocardium
	Novel Therapeutic Agents Against Heart Failure
	Control Mechanisms of Cardiac Function
		Autonomic Control of the Heart
			Sympathetic and Parasympathetic Receptors
			Functions of the ANS in the Heart
			Inotropic Effects of the β1 and M2 Receptors Activation
			Chronotropic Effects of the β1 and M2 Receptors Activation
			Lusitropic Effects of the β1 Receptor Activation
	Developmental Changes in the Fetal Cardiac Contractile System
		Developmental Changes in Cardiac Ca2+ Homeostasis
		Developmental Changes in Cardiac Action Potential
		Developmental Changes in Mechanical Force Production and Contractile Function
		Developmental Changes in Myocardial Function
	Cardiac Cycle and Cardiac Work
		Normal Cardiac Cycle
		Cardiac Work
		Frank-Starling Relationship
	Cardiac Reflexes
		Baroreceptors Reflex (or Carotid Sinus Reflex)
		Bainbridge Reflex
		Bezold-Jarisch Reflex
		Chemoreceptor Reflex
		Valsalva Maneuver
		Cushing Reflex
		Oculocardiac Reflex
	References
Cardiovascular Pharmacology in Pediatric Patients with Congenital Heart Disease
	Vasoactive Agents
		Pure Vasopressors, That Is, Pure Vasoconstrictors
			Phenylephrine
				The Common Adverse Effect of Phenylephrine
			Vasopressin
		Inoconstrictors
			Epinephrine
				Indications
				Routes of Administration and Dosage
			Dopamine
				Indications and the Clinical Effects of Dopamine
				Dopaminergic Activity: Its Effect on the Immunologic and Neurohormonal Systems
				Indications
				General Precautions
			Norepinephrine
				Indications
				Dose and Administration
				General Precautions
		Inodilators (Mainly Milrinone, Dobutamine, and Levosimendan)
			Milrinone
				Indications
				Drug Dose
				Routes of Administration
				Adverse Effects and Pharmaceutical Precautions
			Dobutamine
			Levosimendan
		Pure Vasodilators
			Nitroglycerin
			Hydralazine
			Alprostadil
			Sodium Nitroprusside
			Phentolamine Mesylate (Regitine)
	Antihypertensive Agents
		Drugs Used in Pulmonary Hypertension
			Nitric Oxide
			Sildenafil
			Endothelin Receptor Blockers (ET Blockers)
			Milrinone
	Antiarrhythmic Agents
	Analgesic Agents, Sedative Drugs, and Intravenous Anesthetic Agents
		Drugs for Pediatric Delirium
		Stress Ulcer Prevention and Treatment
		Anticoagulation and Thrombolysis Drugs
			Oral Anticoagulants
	Antiplatelet Agents
	Antifibrinolytic Agents
	Antibiotic Prophylaxis in the Perioperative Period
	References
Perioperative Care of the Congenital Cardiac Patient in the Cardiac Catheterization Laboratory
	Introduction
	The Cath Lab Environment
	The Goals of Congenital Cardiac Catheterization
	Procedural Vascular Access and the Approach
	Anesthetic Considerations in Diagnostic and Interventional Cardiac Cath Procedures
	Preoperative Assessment
	Intraoperative Management
	Anesthetic Agents Used in Congenital Cardiac Catheterization
		Volatile Anesthetic Agents
		Propofol
		Opiate Medications
		Benzodiazepines
		Ketamine
		Dexmedetomidine
	Complications in the Congenital Cath Lab
		Hybrid Procedures
	Conclusion
	References
Perioperative Imaging for the Pediatric Congenital Cardiac Patient
	Introduction
	Philosophic Approach and Intraoperative Decision Making in the Operating Room
	Technology
	Case Vignettes
	References
3D Printing and Modeling of Congenital Heart Disease for Pre-Surgical and Pre-Procedural Planning
	Introduction
	3D Printing Process
	Double Outlet Right Ventricle
	Abnormal Venous Connections
	Hybrid Surgery and Catheterization Procedures: Closure of Complex Ventricular Septal Defects
	Ventricular Assist Devices and Heart Transplantation
	Limitations of 3D Virtual and Printed Hearts
	References
Perioperative Imaging in the Adult Congenital Cardiac Patient
	Technical Considerations for Specific ACHD Lesions
		Atrial and Ventricular Septal Defects
		Tetralogy of Fallot
		Left-Ventricular Outflow Tract Obstruction
		D-Transposition of the Great Arteries
		Congenitally Corrected Transposition of the Great Arteries
		Ebstein Anomaly
		Single Ventricle
	Conclusion
	References
Pediatric Cardiovascular Monitoring
	Noninvasive Monitoring
	Pulse Oximetry
	5-Lead Electrocardiogram
	Blood Pressure
	Near-Infrared Spectroscopy Monitors
	Invasive Monitoring
	Arterial Line
	Central Venous Lines
	Pulmonary Artery Catheter
	Minimally Invasive Cardiac Output Monitors
	Pulse Contour Analysis
	Ultrasound
	Bioimpedance/Bioreactance
	References
Electrocardiography: Basic Knowledge with Focus on Fetal and Pediatric ECG
	Heart Rate
	Rhythm
	QRS Axis
	Conduction Intervals
		PR Interval
		QT Interval
	Morphology
		P-Wave
		QRS Complex
		T-Wave
		ST Segment
	Chamber Hypertrophy
		Right Ventricular Hypertrophy
		Left Ventricular Hypertrophy
		Biventricular Hypertrophy
	Conduction Abnormalities
		Atrioventricular Block
		Intraventricular Conduction Defect
			Right Bundle Branch Block
			Left Bundle Branch Block
			Left Fascicular Block
			Multifascicular Block
	Cardiac Tachyarrhythmias
		Supraventricular Tachycardias
		Ventricular Tachycardias
	Congenital Heart Disease
		Atrial Septal Defect
		Ventricular Septal Defect
		Atrioventricular Septal Defect
		Patent Ductus Arteriosus
		Truncus Arteriosus
		Partially and Totally Anomalous Pulmonary Venous Returns
		Tricuspid Atresia
		Pulmonary Valve Stenosis and Pulmonary Valve Atresia
		Tetralogy of Fallot
		Ebstein Anomaly
		Aortic Valve Stenosis
		Coarctation of the Aorta
		Transposition of the Great Arteries
		Congenitally Corrected Transposition of the Great Arteries
	Cardiomyopathy
		Hypertrophic Cardiomyopathy
		Dilated Cardiomyopathy
		Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia
		Restrictive Cardiomyopathy
	Dystrophies
		Duchenne Muscular Dystrophy
		Myotonic Muscular Dystrophy
	Inflammatory Conditions
		Kawasaki Disease
		Pericarditis
		Myocarditis
		Acute Rheumatic Fever
	Cardiac Tumors
		Rhabdomyoma
		Fibroma
		Myxoma
	References
Central Nervous System Monitoring in Pediatric Cardiac Surgery
	Introduction: Role of CNS Monitoring in Pediatric Cardiac Surgery
	Clinical Assessment of Pain and Sedation in the Postoperative Period in Children
		Assessment of Pain
			The Children’s Hospital of Eastern Ontario Pain Scale (CHEOPS)
			The Children’s Hospital of Eastern Ontario Pain Scale: CHEOPS
			Face, Legs, Activity, Cry, Consolability Scale (FLACC)
			COMFORT Scale
			Toddler Preschool Postoperative Pain Scale (TPPPS)
			Parents’ Postoperative Pain Measure (PPPM)
		Assessment of Sedation
			Ramsay Sedation Scale (Ramsay)
			Ramsay Sedation Scale (Ramsay)
			Richmond Agitation Sedation Scale (RASS)
			Sedation Agitation Scale (SAS)
	Near-Infrared Spectroscopy (NIRS)
	Electroencephalography (EEG) Including Traditional EEG, qEEG, and CCEEG
		History of EEG
		What Are the Applications of EEG in the Perioperative Period?
		How EEG Works (Including the 10/20 System and the Waves)
			Interpretation of EEG
			How to Report EEG
			Limitations of EEG
			EEG Considerations in the Pediatric and Neonatal Group
			Amplitude Integrated EEG: aEEG
	Transcranial Doppler: TCD
		Windows for TCD Signal Acquisition:
		What Are the Main Limitations of TCD?
	Jugular Venous Oxygen Saturation (SjVO2)
		What Does SjVO2 Monitoring Show Us?
		SjVO2 Catheters and the Technique Used for Their Insertion
		Anatomic Approach
		Limitations of Data Interpretation for SjVO2 and Methods to Correct It
	Depth of Anesthesia and Sedation
	Evoked Potentials: Somatosensory Evoked Potential (SSEP), Motor Evoked Potential (MEP), Auditory Evoked Potential (AEP), and Visual Evoked Potential (VEP)
	References
Perioperative Respiratory Monitoring in Congenital Heart Disease Patients
	Introduction
	Clinical Assessment
	Inspection
		Respiratory Rate and Other Vital Signs
		The Pattern of Breathing
	Work of Breathing (WOB)
	Nasal Flaring
	Recession
		Accessory Muscle Use
	Skin Color
	Auscultation
	Respiratory Sounds
		Normal Breath Sounds
		Adventitious or Abnormal Sounds
	Palpation
	Percussion
	Cardiopulmonary Interactions
	Respiratory Monitoring Adjuncts
		Pulse Oximetry
		Capnography and Capnometry
		Blood Gas Analysis
		Monitoring the Ventilator and Mechanical Ventilator Support Systems
	References
Perioperative Coagulation Monitoring in Congenital Heart Disease Patients
	Introduction
	Pediatric Hemostasis
		Comparison Between Neonatal and Adult Hemostasis
	Congenital Heart Disease and Coagulation
	Clinical Evaluation and Preoperative Laboratory Testing
	Coagulopathy and Cardiac Bypass
	Monitoring of Coagulation
		Laboratory-Based Coagulation Test
		Point of Care Monitoring
			Thromboelastography (TEG®) and Thromboelastometry (ROTEM®)
				History and Nomenclature
				Operation Principles
				Graph Analysis and Parameters
			Types of Testing
				Thromboelastometry (ROTEM®)
				Thromboelastography (TEG®)
				Results Interpretation
				Indications and Limitations of Thromboelastography and Thromboelastometry
			Platelet Function Testing
				PFA-100
				VerifyNow System
				The Multiplate analyzer
				TEG Platelet Mapping
				The ROTEM platelet
	Monitoring Integration in Clinical Care
	References
Preoperative Evaluation
	Introduction
	Medical History
	Physical Examination
	Central Nervous System Assessment
	Paraclinical Diagnostic Tests
		Chest Radiography
		Electrocardiography
		Echocardiography
		Computerized Tomography
		Cardiac Magnetic Resonance Imaging (cMRI)
		Cardiac Catheterization and Angiography
	Conclusion
	References
Multi-Systemic Consequences of CHD and the Impact on Perioperative Care
	The Impact of Multi-Systemic Organ Dysfunction on the CHD Patient
	Percentage of Patients with Congenital Heart Disease Who Reach Adulthood
		Pediatric Vs. Adult Percentages in the United States
	Renal Disease in the CHD and ACHD Patients
	Management of Renal Impairment in CHD
	Liver Impairment in CHD
	Management of Liver Disease in CHD
	Pulmonary Disease in CHD
	Management of Restrictive Lung Disease in CHD Patients
	Immunologic Impairment in CHD and Protein-Losing Enteropathy
	Infectious Disease Concerns in CHD
	Hematologic Derangements in CHD
	Thrombotic and Hemostatic Considerations in CHD
	Management of Coagulation Abnormalities
	Endocrine Abnormalities in CHD
	Thyroid Disease in CHD
	Calcium Metabolism and Bone Disorders in CHD
	Diabetes in the CHD Population
	Obesity, Metabolic Syndrome, and Hyperlipidemia in CHD Patients
	Vascular and Cerebrovascular Disease in CHD
	Peripheral Venous Disease
	Aortopathy and Arteriopathy in CHD
	Hypertension in the CHD Population
	Oncologic Complications in CHD
	Mental Health, Well-Being, and Social Impact of CHD
	Summary
	References
Anesthetic Management of Adults with Congenital Heart Disease
	Epidemiology of Adult Congenital Heart Disease
	Major Categories of Congenital Cardiac Structural Defects
		Shunt Lesions
		Mixing Lesions
		Obstructive Lesions
		Regurgitant Lesions
	Acyanotic Congenital Heart Disease
		Dextrocardia
		Congenitally Corrected Transposition of the Great Vessels
		Congenitally Bicuspid Aortic Valve
		Coarctation of the Aorta
		Pulmonic Stenosis
		Primary Pulmonary Hypertension and Pulmonary Vascular Disease
	Left-to-Right Shunt Lesions
		Patent Ductus Arteriosus
		Atrial Septal Defects
		Ventricular Septal Defects
	Cyanotic Congenital Heart Disease
		Tetralogy of Fallot
		Ebstein’s Anomaly
		Transposition of the Great Arteries
		Eisenmenger Syndrome
		Single-Ventricle Physiology and Complex Cyanotic Congenital Heart Disease
	Perioperative Concerns and Anesthetic Management
		Syndromes and Associated Anomalies
		Perioperative Monitoring
		Airway and Ventilatory Management
		Intravenous Access Considerations
		Perioperative Fluid Management and NPO Intervals
		Hematocrit and Perioperative Transfusion Management
		Antibiotic Prophylaxis
		Pacemakers and Arrhythmias
		Postoperative Considerations
	Conclusion
	References
Medical Facility Infrastructure Considerations
	Identification of Pediatric and Adults Patients and Referral Management for Congenital Disease
		Barriers to Care
	Evaluation Coordination
	Surgical and Cardiologic Interventional Issues
		Team Structure
		Pediatric Surgeon Versus Adult Surgeon
		Quality Assurance
		“Hybrid” Suites
	References
Electrophysiology in Patients with Congenital Heart Disease
	Introduction
	Electrophysiology Study and Catheter Ablation in CHD
		Atrial and Supraventricular Tachyarrhythmias
		Ventricular Tachycardias
		Procedural Considerations
	Cardiac Implantable Electronic Devices
		Implantable Cardioverter-Defibrillators
		Pacemakers
	References
Cardiopulmonary Bypass in Children and Infants
	Introduction
	Components of CPB
		Vascular Access
		Tubing
		Blood Pumps
		Oxygenator
	Priming and Hemodilution
	Metabolism During CPB
	Systemic Inflammation During CPB
	Controlling Host Response
	Conclusions
	References
Atrioventricular Septal Defect (AVSD)
	Introduction
	Cardiac Anatomy and Embryology
	Pathologic Findings and Associated Anomalies
	Classification of AVSD
	Associated Cardiac Anomalies
	Clinical Presentation and Diagnosis of the Disease
	Echocardiography
	Surgical Repair
	Intraoperative Management of Anesthesia
	Postoperative Care for AVSD
	Reoperation
	Outcome
	References
Atrial Septal Defect, Ventricular Septal Defect
	Atrial Septal Defect
		Introduction
		Embryology
		Classification
		Pathophysiology of ASD
		Diagnosis of ASD
		Diagnosis of ASD
			Clinical Presentation and the Course of the Disease
			Exercise Intolerance
			Pulmonary Hypertension
			Electrocardiography (ECG)
			Chest X-Ray (CXR)
		Imaging Techniques in the Diagnosis of Atrial Septal Defects
			Echocardiography
		Common Views for Detection of ASD
		Other Imaging Studies
			Cardiac Magnetic Resonance (CMR)
			Cardiac Computed Tomography (CT)
			Cardiac Catheterization
		Treatment
		Anesthetic Management During Atrial Septal Defect Repair
			Anesthesia for Surgical Treatment
			Anesthesia for Interventional Treatment
	Ventricular Septal Defect
		Embryology and Classification of Ventricular Septal Defects
		Pathophysiology of Ventricular Septal Defects
		Diagnosis of Ventricular Septal Defects
			Clinical Findings
				General Findings
				Respiratory Findings
				Cardiovascular Findings
			Chest X-Ray
			Electrocardiography
			Cardiac Catheterization
			Echocardiography
		Treatment of Ventricular Septal Defects
		Anesthetic Management
		Outcome
	References
Tetralogy of Fallot
	Introduction
	Embryology and Anatomy
	Uncomplicated TOF
		Presentation and Anatomic Correlation
		Historical Therapeutic Approaches and Outcomes
		Contemporary Surgical Management and Outcomes
		Timing of Surgery
		Surgical Technique
		Natural History of Operative Correction
		Comorbidities
		Anesthesia Considerations
		Anatomic Variants of TOF
	PDA-Dependent Lesions
		Pulmonary Atresia and Critical Pulmonary Stenosis
	Pulmonary Atresia and Major Aortopulmonary Collaterals
	Non-PDA-Dependent Lesions
		Absent Pulmonary Valve Syndrome
		Coronary Artery Anomalies
		Other Anomalies Requiring Palliative Shunting Prior to Complete Repair
		Additional Congenital Cardiac Anomaly
		Additional Noncardiac Congenital Anomaly
		Physics and Physiology of Systemic Pulmonary Shunting
		Anesthesia Issues of Systemic Pulmonary Shunting
		Anesthesia Issues of the Patient with a BTS for Complete Repair
	Conclusion
	References
Transposition of the Great Arteries
	Embryology
	Terminology and Anatomy
		Simple Transposition of the Great Arteries
	Clinical Presentation
	Historical Therapeutic Approaches and Outcomes
	Contemporary Management and Interventions Prior to Surgery
	Contemporary Surgical Management and Outcomes
	Comorbidities
	Additional Anesthetic Considerations
		Complex TGA
	TGA with Aortic Coarctation
	TGA with Conal Septal Deviation
	Conal Septal Deviation-TGA/VSD and LVOT Obstruction
	Conal Septal Deviation-Taussig Bing Anomaly
	Corrected Transposition of the Great Arteries
	Addendum on Invasive Monitoring Catheter Insertion: Author’s Opinion
	Ultrasound for Central Venous Line (CVL) Placement and Arterial Cannulation
	References
Truncus Arteriosus
	Introduction
	Embryology
	Classification
	Anatomy and Associated Anomalies
	Diagnosis
	Natural History
	Perioperative Evaluation and Management
	Preoperative Assessment and Preparation
	Intraoperative Anesthesia Management
	Operative Technique in Truncus Arteriosus
		General Principles
		Cannulation and Conduct of Cardiopulmonary Bypass (CPB)
		Separation of Pulmonary Arteries
		VSD Closure
		Reconstruction of RV to PA Pathway
		Truncal Valve Repair
		Weaning from CPB
		Risk Factors
	Postoperative Care
	References
Right-Sided Obstructive Lesions
	Tetralogy of Fallot
		Introduction
		Anatomy
		Presentation
		Treatment
		Anesthetic Considerations
	Congenital Pulmonic Valve Stenosis
		Introduction
		Anatomy
		Presentation
		Treatment
		Anesthetic Considerations
	Pulmonary Atresia with Intact Ventricular Septum
		Introduction
		Anatomy
		Presentation
		Treatment
		Anesthetic Considerations
	Pulmonary Atresia with VSD
		Introduction
		Anatomy
		Presentation
		Treatment
		Anesthetic Considerations
	Ebstein’s Anomaly
		Introduction
		Anatomy
		Presentation
		Treatment
		Anesthetic Considerations
	References
Congenital Mitral Valve Anomalies
	Introduction
	Embryology
	Classification of Congenital Mitral Valve Anomalies
		Anatomical Classification Systems
		Surgical Classification
		Complex Congenital MV Lesions
	Clinical Features
		Congenital Mitral Stenosis
		Congenital Mitral Regurgitation
	Diagnosis
		Congenital Mitral Stenosis
			Electrocardiography
			Chest Radiography
			Echocardiography
			Transesophageal Echocardiography
			Dynamic Three-Dimensional (3D) Transthoracic and Transesophageal Echocardiography
		Congenital Mitral Regurgitation
			Chest Radiography
			Electrocardiography
			Transthoracic Echocardiography
			Transesophageal Echocardiography (TEE)
			3D Echocardiography
			Cardiac Magnetic Resonance Imaging
			Cardiac Catheterization
	Management of Congenital Mitral Valve Anomalies
		Timing of Surgery
	Anesthetic Management
		Mitral Regurgitation
		Surgical Techniques
		Surgical Outcomes
	Conclusion
	References
Congenital Anomalies of the Aortic Valve
	Valvular Aortic Stenosis
		Anatomy
		Pathophysiology
	Critical Aortic Stenosis
		Treatment
		Anesthetic Management
	The Child or Adolescent with Valvular Aortic Stenosis
		Treatment
		Anesthetic Management
	Supravalvular Aortic Stenosis
		Anatomy and Pathophysiology
		Treatment
		Risk Assessment
		Anesthetic Management
	Emerging Therapies in the Management of Congenital Aortic Stenosis
		Fetal Intervention
		Application of the Ozaki Procedure to the Pediatric Population
	Conclusions
	References
Anomalies of the Aortic Arch, Aortic Coarctation, Interrupted Aortic Arch, and Vascular Rings
	Coarctation of Aorta
		Introduction and Background
		Embryology and Anatomic Features
		Etiology and Mechanism of the Disease
		Pathologic Findings
		Clinical Features
			Prenatal Period (Fetal Period)
			Neonatal Period
			Infancy Period
			Older Childhood and Adolescence Period
			Adult Features
	Echocardiography in CoA
	Echocardiographic Views Used in CoA
		Echo Protocol
		Associated Anomalies of CoA
			Associated Cardiac Anomalies of CoA
				Aortic Valve Lesions
				Hypoplasia of the Left Heart Structures
				Hypoplasia of the Aortic Arch
				Ventricular Septal Defect (VSD)
				Patent Ductus Arteriosus (PDA)
				Atrial Septal Defect (ASD)
				Bovine Aortic Arch
				Other Cardiac Anomalies
			Associated Non-cardiac Anomalies of CoA
				CNS
				Gastrointestinal System
				Urogenital System
				Skeletal Anomalies
				Chromosomal Anomalies
				Urogenital System
				Skeletal Anomalies
		Therapeutic Approaches
			Surgical Correction
			Balloon Dilatation
			Stenting
		Anesthesia for CoA
			Preoperative Evaluation
				Preoperative Care in Neonatal and Infantile Period
				Preoperative Care in Older Children and Adults
			Intraoperative Anesthesia Management
				Anesthesia Induction and Maintenance
				Monitoring
				CNS Monitoring
				Vasoactive Drugs
				Spinal Cord Protection Strategies
				Patient Positioning
			Postoperative Care
		Clinical Outcome
			Classification of IAA
			Associated Anomalies
			Clinical Findings
			Treatment
	Intraoperative Management
	References
Patent Ductus Arteriosus Devices
	PDA Closure Small Birthweight Infants
		Introduction
			Procedural Indications
			Procedural Overview
			Risks and Potential Complications
			Procedural Anesthesia
	PDA Device Closure Outside of the Newborn Period
		Introduction
			Procedural Indications
			Procedural Overview
			Risks and Potential Complications
			Procedural Anesthesia
	PDA Stenting for the Provision and Maintenance of Systemic Blood Flow
		Introduction
			Procedural Overview
			Procedural Risks
			Procedural Anesthesia
	PDA Stenting for the Provision and Maintenance of Pulmonary Blood Flow
		Introduction
			Procedural Overview
			Procedural Risks
			Procedural Anesthesia
	References
Hypoplastic Left Heart Syndrome: Treatment Options
	Introduction
	Pathology
	Diagnosis
	Pre-operative Care
	Stage 1 Options
		Modified Norwood Procedure
		Sano Procedure
		Hybrid Procedure
		Transplantation
	Single Ventricle Reconstruction Trial
	Hybrid Procedure
	Hybrid Procedure
	Individualized Surgical Approach
	Summary
	References
Double Outlet Right Ventricle
	Clinical Vignettes
	Background
	Embryology
	Anatomy
		Coronary Arteries
	Clinical Presentation
		VSD-Type and TOF-Type DORV
		TGA-Type DORV
		DORV with Remote VSD
		DORV with AVSD
	Surgical Repair
		VSD-Type and TOF-Type DORV
		Mid-Spectrum DORV or TGA-Type DORV with PS
		TGA-Type DORV
		Aortic Outflow Tract Obstruction and Aortic Arch Hypoplasia
	Outcomes
	Anesthetic Management of Clinical Cases
	References
Double Outlet Left Ventricle
	Background
	Nomenclature
	Embryology
	Anatomy
		Situs
		VSD
		Great Vessels
		Conus
		Coronaries
		Conduction System
		Tricuspid Valve
		Associated Anomalies
	Physiology
	Physical Exam
	Electrocardiogram
	Chest Roentgenogram
	Diagnosis
	Surgical Treatment
		Subaortic VSDs
		Aortic Outflow Tract Obstruction
		Patent Pulmonary Outflow
		Single Ventricle
		Historical Operations
	Postoperative Considerations
	Outcomes
	References
Pulmonary Hypertension in Congenital Heart Diseases
	Introduction
	Definition
	Classification
	Clinical Features
	Paraclinical Studies
	Chest X-Ray
	Electrocardiography (Fig. 1)
		Echocardiography
		Cardiac MR (CMR) and Multi-Slice CT Scan
		Radioisotope Ventilation/Perfusion Scan
		Laboratory Tests (BNP/NT-proBNP)
		Genetic Studies
			Cardiopulmonary Exercise Test (CPET)
			Definition of PH During Exercise
	Management
	Pharmacological Treatment
	Anesthesia for Patients with Congenital Heart Disease and Pulmonary Hypertension
	References
Right Ventricular Failure
	Anatomy and Physiology of the Right Ventricle
	Definition
	Etiology
	Pathophysiology
	Diagnosis
		Echocardiographic Evaluation of the RV
	Medical Management
		How to Protect the RV?
		What to Do When the RV Is Failing?
			Preload Optimization
			Improvement in RV Contractility, Reduction in RV Afterload, and Increase in RV Coronary Perfusion Pressure
	Surgical and Interventional Management of Right Ventricular Failure
		Mechanical Circulatory Support Device Options
			Temporary Support
			Long-Term Support
	Transplantation
	RV Failure in the Pediatric Population with CHD
		MCS in the Pediatric Population with RV Failure
	Summary
	References
Coronary Artery Anomalies
	Introduction
	Embryology
	Classification (Table 1)
	Pathology
		Anomalous Left Coronary Artery from the Pulmonary Artery (ALCAPA)
		Anomalous Right Coronary Artery from the Pulmonary Artery (ARCAPA)
		Anomalous Aortic Origin of a Coronary Artery (AAOCA)
		Stenosis or Atresia of the Left Main Coronary Artery
		Myocardial Bridges (MB)
		Coronary Fistulae
		Acquired Coronary Diseases
			Inflammatory Diseases
			Coronavirus 2 Related Multisystem Inflammatory Syndrome in Children (MIS-C) and Coronary Disease
			Coronary Allograft Vasculopathy (CAV)
	Diagnostic Tests
		Electrocardiogram (EKG)
		Echocardiography
			Chest Radiography
			Computed Tomography Angiography (CTA)
			Cardiac Magnetic Resonance Imaging (CMRI)
			Exercise Stress Testing
			Cardiac Catheterization
	Management Algorithm
	Surgical Management
	Anesthetic Considerations
	Outcome
	Case-Based Contents
	References
Fontan and Single Ventricle Patients Undergoing Heart-Liver Transplantation as the Final Palliative Intervention
	Physiology and Transplant Outcomes
	Evaluation and Listing for Combined Heart Liver Transplantation
	Perioperative Optimization
	Intraoperative Considerations
		Liver Transplantation Considerations
	Phases of Liver Transplantation
	Anesthetic Considerations for Combined Liver Transplant and Heart Transplant (OLT/OHT)
	Mechanical Circulatory Support Options for Liver Transplantation
	Intraoperative and Perioperative Hemodialysis
	The Management of Intraoperative Vasoplegia and Massive Transfusion for Combined OHT/OLT
	Intensive Care Unit (ICU) Considerations
	Periprocedural Concerns Post-CHLT
	Conclusion
	References
Heart Transplantation and Mechanical Circulatory Support in the Pediatric and Congenital Heart Patient
	History of Heart Transplantation and Pediatric Heart Transplantation
	Current Pediatric Heart Transplant Landscape
	Indications for Heart Transplantation and Recipient Characteristics
	Heart Transplant Evaluation
	Sensitization
	Waitlist Mortality
	Ischemic Times
	Donor Management and Donor Heart Procurement
	Adult Congenital Heart Disease and Heart Transplant
		The ACHD Patient with Systemic Right Ventricle (SRV), Single Ventricular Physiology
			Eisenmenger’s Syndrome and Combined Heart–Lung Transplant
		Anatomic Variability and Heart Transplantation in the Failing Fontan Patient
	Fontan Heart Failure Classification
	Implantation
	Special Circumstances
	Postoperative Management
	Outcomes
	Mechanical Circulatory Support in the Pediatric Population
		Indications for VAD
		Operative Management of Ventricular Assist Devices
		Summary
	References
Anesthetic Considerations for Pediatric Cardiac Hybrid Procedures
	Preoperative Assessment
		Patient Considerations
		Preprocedural Considerations
		Procedure Considerations
	Intraoperative Management
		Induction
		Monitors
		Medications
		Anesthetic Maintenance
		Special Considerations
	Postoperative Considerations
	Summary
	References
Transcatheter Interventional Techniques in the Adult Congenital Heart Disease Patient
	Introduction
	Shunt-Related Interventions
		Atrial-Level Shunts
			Ostium Secundum ASD Closure
			Patent Foramen Ovale Closure
			Superior Sinus Venosus Defect Stenting
		Ventricular Septal Defect Closure
		Patent Ductus Arteriosus Closure
	Valve Interventions
		Balloon Angioplasty of Native Pulmonary Valve Stenosis
		Pulmonary Valve Replacement
		Percutaneous Repair of Native Atrioventricular Valve Regurgitation
		Prosthetic Atrioventricular Valve Replacement
			Tricuspid Valve
			Mitral Valve
		Aortic Valve Replacement
	Aortic Interventions
		Balloon Angioplasty and Stenting of Coarctation of the Aorta
	Pulmonary Artery Interventions
		Balloon Angioplasty and Stenting of Pulmonary Artery Stenosis
		Closure of Paravalvular Leaks
	Special Populations
		Single Ventricle Patients (Fontan Procedure)
		Pregnant Patients
	Hybrid Procedures
	Conclusion
	References
Postoperative Cardiovascular and Hemodynamic Management in Congenital Cardiac Surgery
	Introduction
	Transferring Patient from Operating Room
	Monitoring Techniques
	Hemodynamic Complications
		Low Cardiac Output
			Residual Defects
			Surgical-Related Factors
			Preload
			Afterload
			Contractility Dysfunction
			Dysrhythmia
	Postoperative Bleeding and Cardiac Tamponade
	Pulmonary Hypertension
	Conclusion
	References
Postoperative Arrhythmias and Their Management
	Postoperative Tachyarrhythmias
		Supraventricular Tachycardias
			Junctional Ectopic Tachycardia
			Atrial Tachycardia
			AV Nodal Reentrant Tachycardia and AV Reciprocating Tachycardia
		Ventricular Tachycardias
	Postoperative Bradyarrhythmias
		Sinus Node Dysfunction
		Atrioventricular Block
	Common Surgical Procedures Associated with Postoperative Arrhythmias
		Atrial Septal Defect Closure
		Ventricular Septal Defect Closure
		Atrioventricular Septal Defect Repair
		Tetralogy of Fallot Correction
		Surgical Repair of Univentricular Heart
		Surgical Correction of Transposition of the Great Arteries
	References
Postoperative Respiratory Management in Pediatric Cardiac Surgical Patients
	How Does the Pediatric Patient Differ from the Adult Patient?
		Anatomical Features
		Physiological Features
	Principles and Goals of Mechanical Ventilation
	Ventilation Considerations and Unique Challenges in Congenital Heart Disease Patients
		Mixed Lesions and Cardiac Shunts
		Perioperative Care of Single Ventricle Physiology (Hypoplastic Left Heart Syndrome (HLHS) and Fontan Procedure)
		Right or Left Ventricular Failure
			Fluid Management
	Strategies for Ventilation and Ventilator Modes
	Weaning, Readiness for Extubation, and Extubation Failure
		Weaning Ventilator Support and Spontaneous Breathing Trials (SBT)
		Prediction Tests for Extubation Success
		“Ready for Departure”: Preparing for Extubation
			The “Extubation Procedure”
		Predictors of Extubation Failure
	Noninvasive Ventilation
		Nasal Cannula
		Neonatal Continuous Positive Airway Pressure (nCPAP)
		High Flow Nasal Cannula (HFNC)
	Miscellaneous Issues
		Extra-Corporeal CO2 Removal (ECCO2R)
		High-Frequency Ventilation or High-Frequency Oscillatory Ventilation (HFV or HFOV)
		Inhalational Routes of Drug Delivery
		Chest Physiotherapy
		Chronic Respiratory Failure and Tracheostomy
	References
Cardiac Anesthesia in Infants and Children: Postoperative Bleeding and Coagulation Management
	Introduction
	Risk Factors for Postoperative Bleeding
		Preoperative Risk Factors
		Intraoperative Risk Factors
	Pathophysiology of Postoperative Bleeding
	Point-of-Care Testing and Algorithms in Postoperative Bleeding
	Prophylaxis
		Preoperative Optimization
		Intraoperative
			General Measures
			Prophylactic Agents
				Antifibrinolytics (Table 3)
	Treatment
		Abnormal Clot Generation
			Prothrombin Complex Concentrates (PCCs)
			Activated Factor VII
		Abnormal Clot Stability
			Antifibrinolytics
		Clot Firmness
			Fibrinogen Concentrate
			Cryoprecipitate
			Platelets
			Fresh Frozen Plasma
			Packed Red Blood Cells
	Adverse Effects (Table 6)
	Outcome
	References
Postoperative Central Nervous System Management in Patients with Congenital Heart Disease
	The Impact of CNS Outcome in Pediatric Cardiac Surgery
	Risk Factors for CNS Injury in Neonates and Infants Undergoing Cardiac Surgery
		Preoperative Risk Factors for CNS Injuries in Neonates and Children
		Intraoperative Risk Factors for CNS Injuries in Neonates and Children
		Postoperative Risk Factors for CNS Injuries in Neonates and Children
	Classification of CNS Deficits
		Neurologic Deficits
		Neonatal Seizure
			Etiology
			Risk Factors
			Diagnosis
			Treatment
		Neurocognitive and Neurodevelopmental Disorders
		White Matter Injuries (WMI) Including Periventricular Leukomalacia (PVL)
			Periventricular Leukomalacia (PVL)
			Risk Factors
			Mechanism of Injury
			Prevalence
			Clinical Presentation of PVL
			Diagnosis
			Treatment
			Postoperative Delirium in Pediatric Patients
			Clinical Assessment and Monitoring of Delirium in Pediatric Cardiac Surgery
			Treatment of Postoperative Delirium
	References
Perioperative Management of Endocrine Problems in Pediatric Cardiac Surgery Patients
	Perioperative Management of Patients Receiving Steroids
	When to Suspect an Impaired HPA Axis?
	Patients with Non-Suppressed HPA Axis
	Patients with the Uncertain Status of the HPA Axis
	Other Groups of Patients Mandating More Sophisticated Care
	How to Manage the Dosage of Glucocorticoids in the Perioperative Period
	Surgical Procedures
	Perioperative Management of the Pituitary Gland
	Central Adrenal Insufficiency
	Central Hypothyroidism
	Central Diabetes Insipidus (DI)
	Perioperative Management of the Diabetes Mellitus Patients
	Perioperative Management of Patients with Metabolic Disorders
		Hypoglycemia
		Phenylketonuria
			Preoperative Management
		Propionic and Methylmalonic Acidemia
			Preoperative Management
			Postoperative Management
		Maple Syrup Urine Disease
			Preoperative Management
		Urea Cycle Defects
			Perioperative Management
		Fatty Acid Oxidation Defects
		Homocystinuria
		Mucopolysaccharidosis
			Preoperative Management
			Postoperative Management
	References
Regional Anesthesia and Perioperative Acute Pain Management in Pediatric and Adult Congenital Heart Surgical Patients
	Introduction
	Innervation of the Thorax
	Mechanisms of Pain After Cardiac Surgery
	Consequences of Pain and Importance of Effective Analgesia After Cardiac Surgery
	Risk Factors for Postoperative Pain
	Assessing Pain After Cardiac Surgery
		Assessing Pain in Adults
		Assessing Pain in Children
	Current Trends in the Treatment of Acute Pain After Cardiac Surgery
		Enhanced Recovery After Surgery (ERAS) and “Fast-Track” Cardiac Surgery
		Minimally Invasive Cardiac Surgery
		Treatment of Pain in Pediatric Cardiac Surgery Patients
	Systemic Therapies
		Acetaminophen
		Non-Steroidal Anti-Inflammatory Drugs
		Gabapentinoids
		Lidocaine
		Corticosteroids
		Alpha-2 Adrenergic Agonists
		N-Methyl-d-Aspartate (NMDA) Receptor Antagonists
			Ketamine
			Magnesium
		Opioids
		Methadone
	Regional Anesthesia for Cardiac Surgery
		Neuraxial Anesthesia/Analgesia
			Thoracic Epidural and Caudal Anesthesia/Analgesia
			Spinal Anesthesia
			Risks of Neuraxial Techniques in Cardiac Surgery
		Paraspinal Regional Anesthesia Techniques
			Paravertebral Block
			Erector Spinae Plane Block
		Chest Wall Blocks
			Continuous Wound Infiltration and Intraoperative Parasternal Intercostal Blocks
			Parasternal Intercostal Plane Block
			Interpectoral Plane and Pectoserratus Plane Block
			Serratus Anterior Plane Block
	Conclusions and Future Directions
	References
Chronic Postoperative Pain in Congenital Heart Disease Patients
	Introduction
	Definition of Chronic Postsurgical Pain
	Incidence, Risk Factors, and Impact of Chronic Pain in Patients with Congenital Heart Disease
		Incidence, Risk Factors, and Impact of Chronic Pain in Children and Young Adults with Congenital Heart Disease
		Incidence, Risk Factors, and Impact of Chronic Pain in Adults with Congenital Heart Disease
	Mechanism of Chronic Pain
	Assessment of Chronic Pain in Patients with Congenital Heart Disease
	Prevention of Chronic Pain in Congenital Heart Disease
		Pharmacologic Intervention
		Local Anesthesia, Regional Anesthesia, and Other Interoperative Interventions
	Treatment of Chronic Pain Associated with Congenital Heart Disease
		Spinal Cord Stimulation
		Radiofrequency Ablation
		Transcutaneous Electrical Nerve Stimulation
		Psychotherapy
	Prognosis
	References
Postoperative Renal Management, Fluid/Electrolyte Management and Acid–Base Disorders
	Introduction
	Physiology: From Birth to Adults
		Fluid Balance and Distribution
		Fluid and Solute Movements
		Electrolyte Balance
			Sodium
			Potassium
			Calcium
			Phosphorus
			Magnesium
			Chloride
			Bicarbonate
		Acid–Base Balance
	Renal Physiology
		Renal Blood Flow
		Glomerular Filtration Rate
	Urine Concentration and Sodium Excretion Fraction
	Congenital Heart Disease Surgery and Acute Kidney Injury
	CPB and Ultrafiltration
	Classification and Diagnosis
		RIFLE Classification System
		AKIN Classification System
		The Multi-societal Database Committee for Paediatric and Congenital Heart Disease Classification
	Lab Findings: New Biomarkers
	Treatment Course
		Fluid Management
			Goal-Directed Fluid Therapy
			Type of Fluid Solution
				Colloids
				Crystalloids
			Concerns Regarding Paediatric Patients
		Parental Nutrition
		Monitoring Daily Fluid Balance
		Pharmacologic Management of Fluid Overload
			Dopamine
			Loop Diuretics
			Nesiritide
			Fenoldopam
	Renal Replacement Therapy
		Peritoneal Dialysis
		Continuous Renal Replacement Therapy
	Electrolyte Management
		Sodium
		Potassium
		Calcium
		Magnesium
	Basis of Pathophysiology Acid–Base Balance in the Postoperative ICU Setting of Cardiac Surgery
		Metabolic Acidosis
		Metabolic Alkalosis
		Respiratory Acidosis and Alkalosis
	A Practical Approach to…
		Fluid Management
		Renal Management
		Acid–Base Disorders Management
	References
Cardiac Intensive Care and Management of Cardiac Arrest in Pediatric Congenital Heart Disease
	Specialized Staff
	Communication
	Monitoring
	Guidelines and Protocolized Care
	Early Recognition of Deterioration
	Cardiac Arrest and Cardiopulmonary Resuscitation
		Physiology of Cardiac Arrest
		Mechanics of Resuscitation
		Monitoring During Resuscitation
		Cardioversion and Defibrillation
		Airway
		Vascular Access
		Pharmacology
		Mechanical Support
			Mechanical Support in Single Ventricle Patients after Cardiac Arrest
		Special Considerations for Resuscitation in Delivery Room
		Special Considerations for Resuscitation in Immediate Post-Operative Period
		Resuscitation in Patients with Mechanical Circulatory Support (MCS)
	Post-Cardiac Arrest Care
		Stabilization
		Investigation into Etiology
		Prevention of Secondary Injury
		Cognitive Rehabilitation
	Outcomes and the Future
	References
Cardiac Intensive Care and Management of Cardiac Arrest in Adult Congenital Heart Diseases
	Perioperative ICU Management of the ACHD Patient by Organ System
		Cardiac
			Heart Failure
			MCS in the ACHD Patient
			Short-Term Support Options
			Long-Term Support Options
		Arrhythmias
		Cardiac Arrest and ECPR
		Neurologic
		Pulmonary
		Gastrointestinal and Hepatic
		Renal
		Hematologic
		Infectious Disease
	References
Nutritional Supports in Congenital Heart Disease
	Introduction
	Types of Congenital Heart Defects
	Effects, Challenges, and Side Effects of CHD
		Hypermetabolism
	Nutritional Support in Patients with CHD
		Calorie Requirements
	Preoperative Nutritional Support
	Postoperative Nutritional Support
	Management of Chylothorax
	Conclusion
	References
Future Approaches for Anesthesia in Congenital Cardiac Surgery and Interventional Procedures
	Future Approaches in Airway Management
	Future Approaches for Arterial Catheter Placement
	Non-Invasive Measurement of Kidney Perfusion
	Non-Invasive Measurement of Hemoglobin
	Cardiometry and Other Non-Invasive Methods for Cardiac Output Monitoring
	Artificial Intelligence and Machine Learning in Pediatric Cardiac Anesthesiology
	Personalized Diagnostics in Congenital Cardiac Patients
	Personalized Pharmaceutics and Individualized Anesthesia Management: Its Role in the Future
	Summary
	References
Pediatric Cardiac Surgery in Emerging Countries
	The Burden of Congenital Heart Disease in the World and Emerging Nations in Particular
	Shortages and Solutions
		Human Resources
	Developing a Sustainable Congenital Heart Center in an Emerging Country
		Models of Assistance
			Education Abroad
			Experienced Physician Returns to Lead Program
			Visiting Team of Specialists
			One-Year Program
		A Success Story
	Analysis of the Five Main Areas of Program Assistance
		On-Site Surgical and Interventional Activity
		Education
		Biomedical Engineering Support
	A Different Way of Doing Business
		Development of a Teamwork Culture with Nurse Empowerment and a Horizontal Hierarchy
		Data Collection
		Data Monitoring
	Summary
	References
Medical Education for Congenital Heart Disease
	Introduction
	Pediatric Cardiac Anesthesiology Training
	Pediatric Cardiology
	Pediatric Cardiac Critical Care
	ACHD Training
	Specialty Certification
	Considerations for an Effective Medical Education Program for Congenital Heart Disease
		Case Volume
		Types of Cases/Types of Lesions
		Resources: Human and Teams
		Resources: Facility and Equipment
	Educational Strategies
		Be Humble and Know Your Limits
		Understand Your Learner
		Use Technology to Your Advantage
		Medical Simulation
	Conclusion
	References
Index