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