Pediatric Cardiac Surgery

✍ Scribed by Carl L. Backer (editor), Constantine Mavroudis (editor)

• Publisher: Wiley-Blackwell
• Year: 2023
• Tongue: English
• Leaves: 1123
• Edition: 5
• Category: Library

✦ Synopsis

PEDIATRIC CARDIAC SURGERY

Explore the leading textbook of pediatric cardiac surgery in this easy-to-read and beautifully illustrated volume

The field of pediatric cardiovascular surgery includes not only heart surgery for infants and children, but also adult heart surgery to correct congenital heart defects which have been present since birth. As infant mortality from heart defects declines and patients with congenital heart defects live increasingly long and healthy lives, new challenges emerge and as such, the need for introductions to this life-saving field has never been greater.

For more than thirty years, Pediatric Cardiac Surgery has met this need with an accessible, comprehensive textbook used by professionals at every level of the field. Featuring full color illustrations, this book offers an introduction to the science of cardiovascular development before moving to detailed analysis of defects in different areas of the heart and their corrective procedures. This updated fifth edition incorporates the latest science to remain the indispensable work on this subject.

In Pediatric Cardiac Surgery, 5^th edition readers will also find:

• Contributions from over 75 national and international experts in the field
• Stunning anatomical illustrations by Rachid Idriss
• New chapters reflecting cutting-edge research, as well as new material throughout pertaining to cardiac imaging and medical education

Pediatric Cardiac Surgery is essential for pediatric and adult cardiac surgeons, cardiologists, and all medical professionals working with pediatric or adult cardiovascular patients.

✦ Table of Contents

Cover
Title Page
Copyright
Table of Contents
Contributors
Preface to the Fifth Edition
A Note on Nomenclature
Chapter 1 Development of the Heart and Great Vessels
Origin of Cardiac Precursor Cells
Formation of the Heart Tube and Early Segmentation
Looping
Development of the Inflow (Venous) Pole of the Heart
Systemic Venous Inflow
Pulmonary Venous Inflow
Atrial Septation
Atrioventricular Valvar Formation
Ventricular Chamber Formation and Septation
Outflow Tract
Outflow Tract Septation
Development of the Arterial Valves
Aortopulmonary Septation
Development of the Aortic Arch
Coronary Arterial Development
Conduction
Conclusion
References
Chapter 2 Genetics of Congenital Heart Disease
Common Variants
Common Variants as Gene Modifiers
Rare de novo Variants and Whole‐Exome Sequencing
Copy Number Variants
Conclusion
References
Chapter 3 Fetal Cardiac Physiology and Fetal Cardiac Intervention
Physiology of the Fetal Circulation
History of Fetal Circulation Research
Fetal Circulation
Fetal Intra‐ and Extracardiac Shunts
Venous Return
Cardiac Output, Oxygenation, and Distribution
Intracardiac and Vascular Pressures
Circulatory Changes at Birth
The Transition
Closure of the Arterial Duct
Pulmonary Circulation
Myocardial Performance
Fetal Cardiac Intervention
Rationale for Fetal Cardiac Intervention
Limitations for Success of Fetal Cardiac Surgery
Clinical Outcomes of Fetal Cardiac Intervention
Fetal Aortic Valvuloplasty
Fetal Atrial Septoplasty/Stenting
Fetal Pulmonary Valvuloplasty
Future Directions
References
Chapter 4 Preoperative Diagnostic Evaluation
Extracardiac and Genetic Evaluation
Electrocardiogram and Rhythm Evaluation
Noninvasive Imaging of Congenital Heart Disease
Echocardiography
Cardiac Magnetic Resonance Imaging
Cardiac Computed Tomography
Predischarge Imaging or Catheterization
Cardiac Catheterization
Catheter‐Based Interventions
Neonatal Cardiac Surgery
d‐Transposition of the Great Arteries
Coarctation of the Aorta
Tetralogy of Fallot
Pulmonary Atresia with Intact Ventricular Septum
Hypoplastic Left Heart Syndrome and Functionally Univentricular Heart Disease
Total Anomalous Pulmonary Venous Connection
Common Arterial Trunk
Cardiac Surgery in Older Infants and Children
Atrial Septal Defects
Ventricular Septal Defects
Atrioventricular Septal Defect
Ebstein Anomaly
Anomalous Origin of the Coronary Arteries
Cardiac Reoperations in Adolescents and Adults with Congenital Heart Disease
Conduit Replacements or Insertions
References
Chapter 5 Hybrid Procedures for Congenital Heart Disease
Hybrid Approach for Hypoplastic Left Heart Syndrome with the Objective of Generating a Fontan Circulation
Technical Considerations
Results
Hybrid Approach for Hypoplastic Left Heart Complex with the Objective of Biventricular Repair
Hybrid Procedures in Congenital Heart Defects beyond Hypoplastic Left Heart Syndrome and Hypoplastic Left Heart Complex
Hybrid Procedure Lessons Learned: Extended Therapeutic Options for “End‐Stage” Heart Failure
Case 1
Case 2
Case 3
Summary
References
Chapter 6 Anesthesia for the Patient with Congenital Heart Disease
Demographics
Anesthetic Assessment
Anesthetic Management
Fast Tracking and Early Extubation
Regional Anesthesia
Cardiac Imaging and Intervention
Pulmonary Hypertension
Cardiac Pharmacology
Postanesthesia Process
Advanced Anesthesia Fellowships
Conclusion
References
Chapter 7 Perioperative Care
Preoperative Care
Specific Considerations for the Neonate
Operative Care
Electrocardiogram Leads
Temperature Monitoring
Oxygen Saturation Probe
Arterial Catheter
Percutaneous Venous Catheters
Atrial Catheters
Pulmonary Artery Catheter
Near‐Infrared Spectroscopy
Pacemaker Wires
Chest Tubes
Nasogastric Tube
Urinary Catheter
Empiric Corticosteroids
Postoperative Care
Transport to the Intensive Care Unit and Handoff
Airway and Breathing
Cardiovascular System
Fluid and Electrolyte Management
Hyperglycemia
Renal Function
Nutrition
Infection Prophylaxis
Complications
Low Cardiac Output
Cardiac Arrhythmias
Bleeding
Cardiac Tamponade
Post‐Pericardiotomy Syndrome
Pulmonary Hypertensive Crisis
Cardiac Arrest
Pulmonary Dysfunction
Renal Failure
Infectious Complications
Central Nervous System Injury
Conclusion
References
Chapter 8 Palliative Operations
Pulmonary Artery Band
Aortopulmonary Shunts
Classic Blalock–Taussig–Thomas Shunt
Modified Blalock–Taussig–Thomas Shunt
Central Aortopulmonary Shunt
Waterston/Cooley Shunt
Potts Shunt
Miscellaneous Palliative Procedures
Conclusion
Data from the Society of Thoracic Surgeons Congenital Heart Surgery Database about Palliative Operations
References
Chapter 9 Management of Pediatric Cardiopulmonary Bypass
History
Principles of Pediatric Cardiopulmonary Bypass
Special Considerations for Pediatric Patients
Systemic Inflammatory Response to Cardiopulmonary Bypass
Monitoring
Anticoagulation and Reversal
Hemodilution and Hematocrit
Blood Gas Management
Temperature
Regional Perfusion Strategies
Ultrafiltration
Management of Post‐Bypass Coagulopathy
Initiation and Termination of Cardiopulmonary Bypass
Circuit Considerations for Pediatric Patients
Circuit Miniaturization
Surface Modifications
Oxygenators
Augmented Venous Return
Heater–Cooler Units and Infection Concerns
End‐Organ Injury and Protection
Myocardium
Neurologic
Pulmonary
Renal
Endocrine
Conclusion
References
Chapter 10 Pediatric Myocardial Protection
Historical Aspects
Uniqueness of the Infant Myocardium
Structural Differences
Metabolic Differences
Calcium Metabolism
Enzymatic Activity
Catecholamine Sensitivity
Functional and Physiological Differences
Ischemic Preconditioning
Principles of Myocardial Protection
Hypothermia
Cardioplegia
Membrane Stabilizers
Substrates
Osmolar Agents
Ions
Buffers
Special Additives
Venous Drainage and Venting
Precise Surgical Correction
Types of Cardioplegia
Crystalloid Cardioplegia
Blood Cardioplegia
Cardioplegia Protocols
Tepid Blood Cardioplegia
Routes of Cardioplegia Administration
Single‐Dose versus Multiple‐Dose Cardioplegia
Intermittent/Continuous Infusion
Surgical Strategies for Effective Cardioplegia
Induction of Cardioplegia
Maintenance Phase
Reperfusion
Cardioplegia Administration Pressure
Integrated Myocardial Protection
Special Circumstances
Cardioplegia: The Future?
Insulin Cardioplegia
Cardioplegia Enrichment with L‐Arginine
Polarizing Cardioplegia
Conclusion
References
Chapter 11 Patent Arterial Duct
Anatomy and Pathophysiology
Natural History
Clinical Features and Diagnosis
Treatment – Premature Infants
Medical
Transcatheter
Surgical
Treatment – Older Infants and Children
Surgical
Video‐Assisted Thoracoscopy
Transcatheter
Data from the Society of Thoracic Surgeons Congenital Heart Surgery Database (STS CHSD) about Patent Arterial Duct
References
Chapter 12 Vascular Rings and Pulmonary Artery Sling
Embryology and Pathology
Double Aortic Arch
Right Aortic Arch
Pulmonary Artery Sling
Innominate Artery Compression
Aberrant Right Subclavian Artery
Rare Vascular Rings
Clinical Presentation and Diagnosis
Chest Radiograph
Barium Esophagogram
Computed Tomography
Magnetic Resonance Imaging
Bronchoscopy
Tracheograms
Cardiac Catheterization
Echocardiography
Surgical Management
Double Aortic Arch
Right Aortic Arch with Left Ligament
Innominate Artery Compression Syndrome
Pulmonary Artery Sling
Tracheal Stenosis
Video‐Assisted Thoracoscopic Surgery
Adults with Vascular Rings
Postoperative Care
Conclusion
Data from the Society of Thoracic Surgeons Congenital Heart Surgery Database (STS CHSD) about Rings and Slings
References
Chapter 13 Coarctation of the Aorta
Embryology and Anatomy
Embryology
Anatomy
Natural History and Pathophysiology
Diagnosis
Surgical Techniques
General Considerations
Resection and End‐to‐End Anastomosis
Prosthetic Patch Aortoplasty
Prosthetic Interposition Graft
Subclavian Flap Aortoplasty
Resection with Extended End‐to‐End Anastomosis
Balloon Dilation Angioplasty
Complications
Conclusion
Data from the Society of Thoracic Surgeons Congenital Heart Surgery Database (STS CHSD) about Coarctation and Aortic Arch Hypoplasia
References
Chapter 14 Interrupted Aortic Arch
Developmental Biology
Genetics
Chromosome 22q11.2 Deletion
Other Chromosomal Deletions
Anatomy and Pathology
Associated Anomalies
Pathophysiology and Clinical Features
Prenatal Diagnosis
Management
Historical Perspective
Diagnosis of Interrupted Aortic Arch
Management of Interrupted Aortic Arch
Preoperative Management
Surgical Approach
Standard Surgical Technique: IAA/VSD
Circulatory Arrest versus Selective Cerebral Perfusion
Interrupted Aortic Arch with Ventricular Septal Defect and Left Ventricular Outflow Tract Obstruction
Interrupted Aortic Arch in Adults
Interrupted Aortic Arch with Other Anomalies
Postoperative Management
Results of Surgery
Complications of Surgery
Early
Late
Special Considerations
Bicuspid Aortic Valve
Unstable Child: Staged Repair of IAA/VSD
Yasui or Left Ventricular Outflow Tract Bypass Procedure for IAA/VSD
Chromosome 22q11.2 Deletion (DiGeorge Syndrome)
Quality of Life
Conclusions
References
Chapter 15 Atrial Septal Defect, Partial Anomalous Pulmonary Venous Connection, and Scimitar Syndrome
Anatomy and Pathology
Ostium Secundum Atrial Septal Defect
Vestibular Atrial Septal Defect
Sinus Venosus Interatrial Communication
Coronary Sinus Defect
Atrioventricular Septal Defect with Exclusive Atrial Shunting (Ostium Primum Defect)
Common Atrium
Pathophysiology and Natural History
Diagnosis
Transcatheter Closure
Surgical Technique
Postoperative Care
Technical Pitfalls
Results of Operation
Results of Transcatheter Closure
Results in Older Patients
Scimitar Syndrome
Conclusion
References
Chapter 16 Ventricular Septal Defect
Definition and Prevalence
Historical Perspectives
Embryology and Pathologic Anatomy
Pathophysiology
Natural History
Diagnosis
Medical Management
Patient Selection
Patients with Large Ventricular Septal Defects
Patients with Small Defects
Patients with Defects Opening Directly beneath the Pulmonary Valve (Doubly Committed and Juxtaarterial Ventricular Septal Defects)
Surgical Considerations
Right Atrial Approach
Transatrial Closure of Perimembranous or Central Ventricular Septal Defect: Exposure by Tricuspid Valve Retraction
Transatrial Closure of Perimembranous or Central Ventricular Septal Defect: Exposure by Tricuspid Valve Incision
Transpulmonary Arterial Approach
Transaortic Approach
Right Ventricular Approach
Left Ventricular Approach
Relationship of the Conduction Pathways to Different Types of Ventricular Septal Defects
Avoiding Conduction Injury during Closure of Different Types of Ventricular Septal Defects Having a Muscular Posteroinferior Rim
Central or Perimembranous Ventricular Septal Defects
Perimembranous Inlet Ventricular Septal Defects
Muscular Ventricular Septal Defects
Gerbode Ventricular Septal Defects
The Conduction System and the Transaortic Approach
Surgical Management of Ventricular Septal Defects with Associated Lesions – Patent Arterial Duct
Ventricular Septal Defect with Aortic Insufficiency
Ventricular Septal Defects with Coarctation of the Aorta
Ventricular Septal Defect with Elevated Pulmonary Artery Pressure
Residual Ventricular Septal Defects in Anatomically Challenging Locations
Results
Pulmonary Artery Bands
Complications
Transcatheter/Transventricular Device Closure
Conclusion
Data from the Society of Thoracic Surgeons Congenital Heart Surgery Database about Ventral Septal Defect
References
Chapter 17 Atrioventricular Septal Defects
Pathology and Anatomy
Hemodynamics/Natural History
Diagnosis
Indications and Timing of Operation
Operative Management and Results
Partial Atrioventricular Septal Defect
Intermediate Atrioventricular Septal Defect
Complete Atrioventricular Septal Defect
Atrioventricular Septal Defect with Tetralogy of Fallot
Results
Risk Factors
Age at Operation
Preoperative Atrioventricular Valve Incompetence
Double‐Orifice Left Atrioventricular Valve
Zone of Apposition
Coarctation of the Aorta
Trisomy 21
Postoperative Management
Conclusion
Data from the Society of Thoracic Surgeons Congenital Heart Surgery Database about Atrioventricular Septal Defect
References
Chapter 18 Common Arterial Trunk
Embryologic and Anatomic Features
Historical Perspectives
Physiology and Clinical Findings
Natural History
Operative Technique
Surgical Repair of Common Arterial Trunk
Surgical Repair of Anatomic Variants
Interrupted Aortic Arch
Truncal Valve Repair for Truncal Regurgitation
Truncal Valve Repair in the Presence of Coronary Artery Anatomy Concerns
Recent Results of Total Correction
Results for Repair of Common Arterial Trunk and Interrupted Aortic Arch
Results for Repair of Common Arterial Trunk and Truncal Regurgitation
Right Ventricular to Pulmonary Artery Continuity
Conclusion
Data from the Society of Thoracic Surgeons Congenital Heart Surgery Database (STS CHSD) about Common Arterial Trunk (Truncus Arteriosus)
References
Chapter 19 Aortopulmonary Window and Aortic Origin of a Pulmonary Artery
Historical Aspects
Embryology and Anatomy
Clinical Features and Natural History
Diagnosis
Surgical Treatment
Postoperative Course
Outcome after Surgery
References
Chapter 20 Isolated Right Ventricular Outflow Tract Obstruction
Pulmonary Atresia with Intact Ventricular Septum
Definition, Etiology
Morphology
Clinical Presentation and Initial Management
Interventional/Surgical Management
Initial Palliation
Definitive Repair, Outcomes, Late Survival
Pulmonary Stenosis with Intact Ventricular Septum
Definition, Epidemiology
Morphology
Pathophysiology and Natural History
Clinical Presentation and Initial Management
Interventional/Surgical Management
Outcomes, Late Survival
References
Chapter 21 Tetralogy of Fallot
Definition, Morphology, and Nomenclature
History of Surgical Management
Presentation and Diagnosis
Medical Management
Surgical Repair
Palliative Procedures
Complete Repair
Results
Mortality
Morbidity
Early Reoperation and Reinterventions
Arrhythmias and Sudden Death
Long‐Term Complications Following Repair of Tetralogy of Fallot
Pulmonary Valve Restoration
Pulmonary Valve Replacement
Bioprosthetic Pulmonary Valve Insertion for Severe Pulmonary Regurgitation without Pulmonary Stenosis
Bioprosthetic Pulmonary Valve Insertion for Combined Pulmonary Regurgitation and Pulmonary Stenosis
Neurologic Outcomes
Aortic Valve and Root Problems
Special Circumstances
Tetralogy of Fallot with Absent Pulmonary Valve
Tetralogy of Fallot and Atrioventricular Septal Defect
Conclusion
Data from the Society of Thoracic Surgeons Congenital Heart Surgery Database about Tetralogy of Fallot
References
Chapter 22 Tetralogy of Fallot with Pulmonary Atresia and Major Aortopulmonary Collaterals
Clinical Features
Diagnostic Evaluation
In the Neonatal Period
Prior to Final Repair
Suitability for Repair
Surgical Management
Tetralogy of Fallot with Pulmonary Atresia and Ductus‐Dependent Lung Circulation
Tetralogy of Fallot with Pulmonary Atresia with Major Aortopulmonary Collateral Arteries
Indication for Surgery and Surgical Strategy
Surgical Techniques
Primary Rehabilitation Strategy
Primary Unifocalization Strategy
Postoperative Management
Primary Rehabilitation
Primary Unifocalization
Outcomes
Tetralogy of Fallot with Pulmonary Atresia and Major Aortopulmonary Collateral Arteries
Conclusion
Data from the Society of Thoracic Surgeons Congenital Heart Surgery Database (STS CHSD) about Tetralogy of Fallot with MAPCAs
REFERENCES
Chapter 23 Ventricular to Pulmonary Artery Conduits
Polytetrafluoroethylene Ventricular Outflow Tract Reconstruction
Allograft Conduits
Pulmonary Allografts in Ross Aortic Valve Replacement Patients
Bovine Jugular Venous Valved Conduit
Percutaneous Pulmonary Valves
Nonvalved Right Ventricular to Pulmonary Artery Conduits
Stentless and Stented Xenograft Valves in the Right Ventricular to Pulmonary Artery Position
Mechanical Valves in the Pulmonary Position
Bioengineered Valved Conduits for Right Ventricular Outflow Tract Reconstruction
When to Operate for Right Ventricular Outflow Tract Dysfunction
Conclusion
Data from the Society of Thoracic Surgeons Congenital Heart Surgery Database about Conduit Operations
References
Chapter 24 Double‐Outlet Right Ventricle
Controversies
History
Embryology
Morphology
Atrioventricular and Ventriculoarterial Connections
Subaortic Defects
Double‐Outlet Right Ventricle with Left‐Sided Aorta
Doubly Committed Defect
Subpulmonary Defect (Taussig–Bing)
Noncommitted (Remote) Defects
Right Ventricular Outflow Tract Obstruction
Subaortic Stenosis
Conduction System
Coronary Arterial Anatomy
Associated Cardiac Abnormalities
Classification
Pathophysiology
Congestive Heart Failure
Cyanosis
Diagnosis
Physical Examination, Electrocardiogram, and Chest Radiograph
Echocardiogram
Cardiac Catheterization and Cineangiography
Magnetic Resonance Imaging/Computed Tomographic Imaging
Three‐Dimensional Imaging
Natural History
Treatment
Impediments to Complete Anatomic Repair
General Aspects of Surgical Repair
Double‐Outlet Right Ventricle, Interventricular Defect Type (Subaortic or Doubly Committed Defects without Pulmonary Stenosis)
Double‐Outlet Right Ventricle, Tetralogy of Fallot Type (Subaortic or Doubly Committed Defect with Pulmonary Stenosis)
Double‐Outlet Right Ventricle, Transposition of the Great Arteries Type (Subpulmonary Defect)
Double‐Outlet Right Ventricle with Subpulmonary Defect and Pulmonary Stenosis
Double‐Outlet Right Ventricle, Remote Defect Type
Double‐Outlet Right Ventricle with Atrioventricular Septal Defect
Double‐Outlet Left Ventricle
History
Embryology
Morphology
Pathophysiology
Diagnosis
Natural History
Treatment
Double‐Outlet Left Ventricle with Pulmonary Stenosis
Complete Intraventricular Repair
Pulmonary Artery Translocation
REV Procedure
Rastelli Procedure
Double‐Outlet Left Ventricle without Pulmonary Stenosis
Double‐Outlet Left Ventricle with Tricuspid and Right Ventricular Hypoplasia
References
Chapter 25 Transposition of the Great Arteries
Embryologic and Anatomic Considerations
Physiology
Clinical Presentation
Medical Management of the Neonate
Surgical Management
Arterial Switch Operation for Transposition of the Great Arteries with Intact Ventricular Septum
Technique
Pericardial Harvest, Evaluation, and Dissection
Cannulation and Cardiopulmonary Bypass
Conduct of Cardiopulmonary Bypass
Myocardial Protection
Neoaortic Reconstruction and Coronary Artery Transfer
Special Coronary Problems
Neopulmonary Artery Reconstruction
Separation from Cardiopulmonary Bypass and Postoperative Care
Results and Analysis
Arterial Switch and Ventricular Septal Defect Closure for Transposition of the Great Arteries and Ventricular Septal Defect
Current Approach
Staged Repair of Transposition of the Great Arteries with Intact Ventricular Septum
Arterial Switch Operation for Double‐Outlet Right Ventricle and Subpulmonary Ventricular Septal Defect (Taussig–Bing Anomaly)
Staged Arterial Switch Operation for Right Ventricular Failure after the Atrial Baffle Procedures
Late Complications and Reoperations Following the Arterial Switch Operation
Acquired Supravalvar Pulmonary Stenosis
Postoperative Arrhythmias
Neoaortic Insufficiency and Neoaortic Anastomotic Stenosis
Coronary Artery Problems
Damus–Stansel–Kaye Procedure
Transposition of the Great Arteries, Ventricular Septal Defect, and Pulmonary Stenosis
Transposition of the Great Arteries–Ventricular Septal Defect–Coarctation/Interrupted Aortic Arch
Functional Status and Quality of Life
Future Considerations
References
Chapter 26 Congenitally Corrected Transposition of the Great Arteries
Important Historical Aspects
Diagnosis
Natural History and Clinical Presentation
Surgical Indications and Options
Interim (Palliative or Nondefinitive) Operations for ccTGA
Definitive Operations for ccTGA
Management of Systemic Right Ventricular Failure in ccTGA
Specific Surgical Techniques for ccTGA
Physiologic Repair for ccTGA with Ventricular Septal Defect with or without Left Ventricular Outflow Tract Obstruction
Anatomic Correction of ccTGA via Double Switch
Anatomic Correction of ccTGA via Senning or Mustard plus Rastelli Operation
Anatomic Correction using Intra‐atrial Baffle plus Translocation of the Great Vessels
Cavopulmonary Connection Technique in ccTGA
Pacemakers in ccTGA
Cardiac Transplantation in ccTGA
Analysis of Outcome for ccTGA: Physiologic Repair
Anatomic Repair
Univentricular Repair
Conclusion
References
Chapter 27 The Functionally Univentricular Heart
Background
Definition
Classification
Cardiac Lesions
Double‐Inlet Ventricles
Mitral Atresia
Tricuspid Atresia
Unbalanced Atrioventricular Septal Defect
“Single Ventricle with Heterotaxy Syndrome”
“Single Ventricle with Total Anomalous Pulmonary Venous Connection” and Other Lesions
Clinical Presentation
Diagnosis
Indications for Surgical Intervention
Historical Surgical Management
Current Surgical Management
First‐Stage Palliation
Second‐Stage Palliation
Third‐Stage Fontan Circulation
Other Considerations
Fenestration
Atrioventricular Valvar Repair or Replacement
Severe Cyanosis Following Second‐Stage Palliation
Fontan Takedown
Mechanical Circulatory Support
Transplantation
Results
Late Considerations
Protein‐Losing Enteropathy
Plastic Bronchitis
Hepatic Dysfunction
Arrhythmias and Fontan Conversion
Exercise Capacity
Thromboembolism and Stroke
Collaterals
Pulmonary Arteriovenous Malformations
Conclusion
Data from the Society of Thoracic Surgeons Congenital Heart Surgery Database about Glenn and Fontan
References
Chapter 28 Fontan Conversion
Evolution of Chronic Fontan Physiology
Early Catheter Ablation Techniques
Introduction of Fontan Revision
Introduction of Fontan Conversion
Fontan Conversion with Associated Procedures
Conduct of Fontan Conversion
Pacemaker Implantation
Anatomic and Electrophysiologic Variations of Fontan Conversion
Takedown of Right Atrial–Right Ventricular Bjork Modification
Takedown of Atrioventricular Valve Isolation Patch for Right‐Sided Maze Procedure
Right Atrial Cannulation in the Presence of a Right Atrial Clot
Distended Left Superior Caval Vein Causing Left Pulmonary Vein Stenosis
Discontinuous Pulmonary Arteries
Right Ventricular Hypertension and Tricuspid Regurgitation after Atriopulmonary Fontan for Pulmonary Atresia and Intact Ventricular Septum
Right Atrial Reduction in the Setting of a Systemic Right Ventricle Leading to Pulmonary Vein Stenosis
Unwanted Inferior Caval Vein Retraction during the Extracardiac Connection
Surgical Translocation of Atrial Septal Alignment
The Modified Right‐Sided Maze Procedure for Various Single‐Ventricle Pathology
Fontan Conversion as a Reproducible Procedure
Conclusion
Data from the Society of Thoracic Surgeons Congenital Heart Surgery Database Fontan conversion
References
Chapter 29 Ebstein Malformation
Overview
Anatomy
Physiology
The neonate
Presentation and Diagnosis
Indications for Surgery
Surgical Strategies
Children and Adults
Presentation and Diagnosis
Indications for Surgery
Surgical Strategies
Tricuspid Valve Repair
Tricuspid Valve Replacement
One‐and‐a‐half ventricle repair
Plication of the Atrialized Right Ventricle
Right Reduction Atrioplasty and Surgical Arrhythmia Management
Heart Transplantation
Postoperative Management
Neonates
Children and Adults
Risk Factors and Outcome
References
Chapter 30 Left Ventricular Outflow Tract Obstruction
Valvar Aortic Stenosis
Critical Aortic Stenosis
Anatomy
Diagnosis
Pathophysiology
Management
Surgical Valvotomy
Balloon Valvotomy
Results of Valvotomy
Which Initial Palliation Is Better?
Critical Aortic Stenosis Associated with Severe Annular Hypoplasia
Results of Infant Ross/Ross–Konno
Fetal Aortic Valvuloplasty
Aortic Valve Stenosis beyond the Newborn Period
Embryology
Anatomy
Pathophysiology
Clinical Features
Diagnosis
Future Directions in Diagnosis and Evaluation
Indications for Intervention
Treatment
Surgical History
Surgical Techniques
Results
Subvalvar Aortic Stenosis
Embryology and Anatomy
Pathophysiology
Clinical Features
Diagnosis and Indication for Intervention
Surgical Treatment
Results
Supravalvular Aortic Stenosis
Embryology: Systemic Elastin Arteriopathy and Williams–Beuren Syndrome
Anatomy
Pathophysiology
Clinical Features
Diagnosis
Medical and Interventional Management
Indication for Surgical Intervention
Surgical Management
Diffuse Supravalvular Aortic Stenosis
Results
Data from the Society of Thoracic Surgeons Congenital Heart Surgery Database about Left Ventricular Outflow Tract Obstruction and Left Heart Lesions
References
Chapter 31 Hypertrophic Cardiomyopathy
Definition, Classification, Etiology, and Diagnosis
Natural History
Treatment
Outcome
Hypertrophic Cardiomyopathy with Left Ventricular Outflow Tract Obstruction
Operative Management of Hypertrophic Cardiomyopathy with Left Ventricular Outflow Tract Obstruction
Preoperative Echocardiography
Surgical Procedure
Implantable Cardioverter‐Defibrillator Placement
Complications
Outcomes of Surgery for Hypertrophic Cardiomyopathy with Left Ventricular Outflow Tract Obstruction in Children
Conclusions
Data from the Society of Thoracic Surgeons Congenital Heart Surgery Database (STS CHSD) about Cardiomyopathy
References
Chapter 32 Hypoplastic Left Heart Syndrome
Epidemiology
Fetal Development
Anatomy
Common Anatomic Features
Additional Anatomic Features
Clinical Features and Diagnosis
Management of the Neonate with HLHS
Initial Stabilization
Staged Reconstruction
Stage 1 Palliation: Norwood Procedure
Postoperative Management
Mechanical Circulatory Support
Interstage Management
Medical Therapy
Home Monitoring
Stage 2 Palliation: Cavopulmonary Connection
Stage 3 Palliation: The Fontan Procedure
Outcomes of Staged Reconstruction
Heart Transplantation
Neurodevelopmental Outcome
Conclusion
Data from the Society of Thoracic Surgeons Congenital Heart Surgery Database about Hypoplastic Left Heart Syndrome
References
Chapter 33 Aorto‐Left Ventricular Tunnel
Pathophysiology and Anatomy
Pathogenesis
Presentation and Diagnosis
Treatment
Conclusion
References
Chapter 34 Congenital Anomalies of the Mitral Valve
Historical Note
Normal Mitral Valve Anatomy
Mitral Valve Pathology, Classification, and Analysis
Supravalvar Region
Annulus
Valve Leaflets
Subvalvar Apparatus
Congenital Mitral Stenosis
Congenital Mitral Regurgitation
Ischemic Mitral Regurgitation
Pathophysiology and Clinical Presentation
Imaging and Studies
Treatment
Medical Management
Indications for Surgical Management
Transcatheter Therapy
Surgical Repair Techniques
Supravalvar Repair
Annular Remodeling
Leaflet Repair
Subvalvar Reconstruction
Mitral Valve Replacement
Postoperative Management
Outcomes
Conclusion
Data from the Society of Thoracic Surgeons Congenital Heart Surgery Database (STS CHSD) about Mitral Valve Anomalies
References
Chapter 35 Total Anomalous Pulmonary Venous Connection
Embryology
TAPVC and Genetics
Classification
Cardiac Anatomy, Associated Lesions
Natural History
Diagnosis
Operative Management and Anesthetic Considerations
Surgical Repair of Supracardiac Type
Repair of the Cardiac Type
Repair of the Infracardiac Type
Repair of the Mixed Type
Ligation of the Vertical Vein
Results
Postoperative Pulmonary Vein Stenosis
Surgical Management for Postoperative Pulmonary Vein Stenosis
Sutureless Technique for Primary Repair of TAPVC
Data from the Society of Thoracic Surgeons Congenital Heart Surgery Database (STS CHSD) about Pulmonary Venous Anomalies
References
Chapter 36 Cor Triatriatum, Pulmonary Vein Stenosis, and Atresia of the Common Pulmonary Vein
Embryology of the Pulmonary Venous System
Pathologic Anatomy
Cor Triatriatum (Divided Left Atrium)
Anatomy
Pathophysiology
Clinical Presentation
Treatment
Atresia of the Common Pulmonary Vein
Pulmonary Vein Stenosis
Cor Triatriatum Dexter (Divided Right Atrium)
Data from the Society of Thoracic Surgeons Congenital Heart Surgery Database (STS CHSD) about Cor Triatriatum
References
Chapter 37 Anomalous Systemic Venous Connections
History
Incidence
Anatomy
Left Superior Caval Vein to Coronary Sinus without Coronary Sinus Ostial Atresia
Left Superior Caval Vein with Coronary Sinus Ostial Atresia
Unroofed Coronary Sinus Syndrome
Complete Unroofing with Left Superior Caval Vein
Complete Unroofing without Left Superior Caval Vein
Partially Unroofed Coronary Sinus with or without Left Superior Caval Vein
Right Superior Caval Vein to the Left Atrium
Direct Inferior Caval Vein Connections
Indirect Inferior Caval Vein Connections
Atrial (Appendage) Isomerism
Diagnosis
Pathophysiology and Natural History
Surgical Management of Systemic Venous Route Abnormalities
Left Superior Caval Vein with Coronary Sinus Ostial Atresia
Left Superior Caval Vein to the Coronary Sinus without Coronary Sinus Ostial Atresia
Azygos or Hemiazygos Continuation of the Inferior Caval Vein
Separate Connection of Hepatic Veins into the Right Atrium
Surgical Management of Systemic Venous Destination Abnormalities
Left Superior Caval Vein to the Left Atrium without a Coronary Sinus
Repositioning the Atrial Septum
Superior Atrial Approach
Left Superior Caval Vein Translocation with Interatrial Septal Repositioning
Bidirectional Cavopulmonary Connection
Roofing the Coronary Sinus
Translocation of the Left Superior Caval Vein to the Right Atrium
Partially Unroofed Midportion of the Coronary Sinus
Inferior Caval Vein to the Left Atrium
Total Anomalous Systemic Venous Drainage
Right Superior Caval Vein to the Left Atrium
Results
Special Situations
Heart Transplantation in Patients with Anomalies of Systemic Venous Connection
Cavopulmonary Connections in Patients with Complex Functionally Single Ventricle
References
Chapter 38 Connective Tissue Disorders
Diagnostic Syndromes and Associated Heart Disease
Marfan Syndrome
Loeys–Dietz Syndrome
Ehlers–Danlos Syndrome
Osteogenesis Imperfecta
Aortic Surgery in Connective Tissue Diseases
Aortic Root Aneurysms
Aortic Root Replacement: Surgical Techniques
Bicuspid Aortic Valves and Valve Repair
Aortic Arch Repair
Simultaneous Repair of Pectus Excavatum
Outcomes Following Valve‐Sparing Aortic Root Replacement
Mitral Valve Surgery in Connective Tissue Diseases
Techniques for Mitral Valve Surgery
Conclusion
Data from the Society of Thoracic Surgeons Congenital Heart Surgery Database (STS CHSD) about Aortic Aneurysms
References
Chapter 39 Coronary Artery Anomalies
Anomalous Left Coronary Artery from Pulmonary Artery
Pathophysiology
Clinical Features
Surgical Management
Results
Discussion
Anomalous Pulmonary Origin of Right Coronary Artery
Anomalous Aortic Origins of Coronary Arteries
Left Main Coronary Artery from Right Aortic Sinus of Valsalva
Right Coronary Artery from Left Aortic Sinus of Valsalva
Anomalous Circumflex Coronary Artery from Right Aortic Sinus of Valsalva or Right Coronary Artery
Surgical Management
Single Coronary Artery
Left Main Coronary Artery Atresia
Surgical Management
Coronary Arteriovenous Fistulas
Intramyocardial Course of Coronary Arteries (Bridging)
Therapeutic considerations
Coronary Aneurysms
Coronary Aneurysms in Kawasaki Disease
Surgical Management
References
Chapter 40 Cardiac Tumors
Historical Background
Nomenclature and Classification
Incidence
Primary Cardiac Tumors
Secondary Cardiac Tumors
Tumor Histotypes
Primary Cardiac Tumors
Genetic Predisposition
Clinical Appearance
Symptoms Related to Intracavitary Obstruction and Infiltration
Arrhythmias
Diagnostic Modalities
Chest Roentgenography
Electrocardiography
Echocardiography
Magnetic Resonance Imaging
Computed Tomography
Angiography
Biopsy
General Principles of Surgical Resection
General Considerations of Treatment Strategies
Primary Cardiac Tumors
Rhabdomyomas
Fibromas
Myxomas
Intrapericardial Teratomas
Angiomas
Hamartomas
Other Primary Benign Cardiac Tumors
Primary Malignant Cardiac Tumors
Secondary Cardiac Tumors
Data from the Society of Thoracic Surgeons Congenital Heart Surgery Database (STS CHSD) about Heart Tumors
References
Chapter 41 Diseases of the Pericardium
Historical Perspectives
Embryology and Anatomy
Diseases of the Pericardium
Effusive Pericarditis
Constrictive Pericarditis
Cardiac Tamponade
Postoperative Pericardial Effusion
Postoperative Cardiac Tamponade
Postpericardiotomy Syndrome
Congenital Defects of the Pericardium
Pericardial Neoplasia
Benign Pericardial Mass
Pericardial Cyst
Pneumopericardium
Chylopericardium
References
Chapter 42 Surgical and Transcatheter Management of Arrhythmias
History of Arrhythmia Surgery
Recommendations for Arrhythmia Surgery
Arrhythmia Surgery Techniques
Focal Atrial Tachycardia, Atrioventricular Nodal Reentry Tachycardia, and Supraventricular Tachycardia due to Accessory Connections
Focal or Automatic Atrial Tachycardia
Atrioventricular Nodal Reentry Tachycardia
Accessory Connection‐Mediated Tachycardia
Macroreentrant Atrial Tachycardia
Atrial Fibrillation
Prophylactic Atrial Arrhythmia Surgery
Device Therapy of Congenital Heart Disease
Conclusion
Data from the Society of Thoracic Surgeons Congenital Heart Surgery Database about Arrhythmia Surgery
References
Chapter 43 Pediatric Heart Transplantation
Indications
Cardiomyopathy
Congenital Heart Disease
Retransplantation
Contraindications
Ventricular Assist Device
Pretransplant Evaluation
Donor Evaluation
Immunosuppression and Rejection
Transplant Techniques
Donor Operation
Recipient Operation
Outcomes
Conclusion
Data from the Society of Thoracic Surgeons Congenital Heart Surgery Database about Heart Transplantation
References
Chapter 44 Lung and Heart–Lung Transplantation
Lung Transplantation
Indications
Cystic Fibrosis
Pulmonary Hypertension
Pulmonary Fibrosis
Surfactant Genetic Anomalies
Retransplantation
Heart–Lung Transplantation
Indications
Contraindications
Listing and Donor Evaluation
Surgical Technique
Organ Harvest
Transplant Procedure
Living Donor Lung Transplantation
Heart–Lung Transplantation
Posttransplant Immunosuppression
Posttransplant Management and Complications
Late Complications
Survival
Special Considerations
Infant Lung Transplantation
Mechanical Support
Growth
Conclusions and Future Directions
Data from the Society of Thoracic Surgeons Congenital Heart Surgery Database (STS CHSD) about Heart Transplantation
References
Chapter 45 Endocarditis in Patients with Congenital Heart Disease
Definition
Epidemiology
Pathophysiology
Microbiology
Diagnosis
Clinical Manifestations
Laboratory Investigations
Imaging Studies
Antimicrobial Therapy
Indications for Surgery
Congestive Heart Failure
Valvar Dysfunction
Mechanical Intracardiac Complications
Remote Embolic Complications
Failure of Adequate Medical Therapy
Culture‐Negative Endocarditis
Prosthetic Valve/Device Endocarditis
Timing of Surgery
Surgical Procedures
Technique of Cardiopulmonary Bypass
Endocarditis Associated with Specific Lesions in Patients with Congenital Heart Disease
Endocarditis Associated with Small Residual or Persistent Ventricular Septal Defects
Tricuspid Valve Repair/Replacement and Ventricular Septal Defect Closure for Bacterial Endocarditis
Aortic Valve Repair/Replacement and Ventricular Septal Defect Closure for Bacterial Endocarditis
Mitral Valve Repair/Replacement for Bacterial Endocarditis
Hemodynamic Jet Lesions Causing Endocarditis in Patients with Patent Arterial Duct or Coarctation of the Aorta
Endocarditis Associated with Patent Arterial Duct
Endocarditis Associated with Coarctation of the Aorta
Endocarditis Associated with Surgical or Transcatheter Pulmonary Valve Insertion
Results
Infective Endocarditis in Children
Early Surgery
Risk Factors for Death
Conclusions
Data from the Society of Thoracic Surgeons Congenital Heart Surgery Database about Endocarditis
References
Chapter 46 Pediatric Mechanical Circulatory Support
Patient Selection for Ventricular Assist Device Support
Preimplant Patient Status
Intent of Device Therapy
Underlying Diagnosis
Patient Size
Patient Comorbidities
Device Selection
Extracorporeal Membrane Oxygenation
Short‐Term Ventricular Assist Devices
Durable Ventricular Assist Devices
Pulsatile Devices
Continuous‐Flow Devices
Biventricular Support
Operative Care
Postoperative Care
Transition to Outpatient Management
Unique Pediatric Issues
Mechanical Support for Congenital Heart Disease
Mechanical Support of the “Failing Fontan”
Device Fitting
Conclusion
References
Chapter 47 Adult Congenital Heart Disease
General Considerations
Heart Failure
Pulmonary Arterial Hypertension
Arrhythmias
Cyanosis
Hemostatic Dysfunction
Stroke
Renal Dysfunction
Metabolic Complications
Hemoptysis
Pregnancy and Heart Disease
Surgical Considerations
Resternotomy
Collateral Vessels
Blalock–Taussig–Thomas Shunt
Glenn Shunt
Pulmonary Artery Band
Anesthesia Considerations
Monitoring
Anesthesia in Specific Defects
Postoperative Care
Respiratory Management
Cardiovascular Monitoring
Low Cardiac Output
Mechanical Assist Devices
Short‐Term Devices
Long‐Term Devices
Specific Congenital Heart Defects
Atrial Septal Defects
Fossa Ovalis Membrane Aneurysm
Ventricular Septal Defects
Tetralogy of Fallot
Coarctation of the Aorta
Fontan‐Type Repair
Transposition of the Great Arteries
Left Ventricular Outflow Tract Obstruction
Discrete Subaortic Stenosis
Diffuse/Tunnel Subaortic Stenosis
Konno Procedure
References
Chapter 48 Bioethics in Congenital Heart Surgery
Informed Consent
Transparency, Informed Consent, and Nudging In Pediatric Aortic Stenosis and Symptomatic Left Ventricular Endocardial Fibroelastosis
Case Presentation
Commentary
Rhetoric, Persuasion, and Psychology
Surgeon Responsibility and Psychological Techniques of Persuasion
Selective Emphasis
Beneficent Persuasion
Nudging and Informed Consent
Shared Decision‐Making
Competency, Transparency, and Informed Consent
Autonomy and Transparency
Clinical Outcome
Rare and Expensive Medical Conditions
Government‐Level Funding
Utility, Economics, and Resource Allocation
Healthcare Economics
Respect for Patient Autonomy as a Medical Virtue
Centrality of the Principle of Respect for Patient Autonomy
From Principles to Virtues
Respect for Patient Autonomy as a Medical/Professional Virtue: Classical Understanding of “Virtue” (Arête)
Fetal Cardiac Intervention: The Burden of Knowledge
The Problem of Language: Throwing the Proverbial Baby out with the Bathwater?
Aporia and Postmodernism: Philosophic Considerations
Postmodernity and Medicine?
The Future of Fetal Cardiac Surgery: Gazing into the Abyss
Ethical Considerations for Postcardiotomy Extracorporeal Membrane Oxygenation
Evolution of Selection Criteria for Postcardiotomy Extracorporeal Membrane Oxygenation
Ethical Issues
Medical Futility: “When Further Therapy Is Hopeless”
Power
Trust
Money
Hope
Attempts to Define Futility
References
Chapter 49 Education in Congenital Cardiac Surgery
“Boot Camp”
Surgical Training of Congenital Heart Surgery Using Three‐Dimensional Print Models
Biologic Simulation Methods That Recreate Congenital Heart Operations
Conclusion
Reflections by the Editors
References
Index
EULA