"FUNCTIONALLY" SINGLE VENTRICLE : the most complex congenital heart defect.
"Functionally" Single Ventricle: The Most Complex Congenital Heart Defect covers the most recent knowledge, methodologies, and techniques for diagnosing, managing, and treating pediatric patients with the "functionally" single ventricle congenital heart defect.
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| Format: | eBook |
| Language: | English |
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[S.l.] :
Academic Press,
2025.
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| Subjects: | |
| Online Access: | Connect to the full text of this electronic book |
Table of Contents:
- Intro
- "Functionally" Single Ventricle: The Most Complex Congenital Heart Defect
- Copyright
- Dedication
- Contents
- Contributors
- Foreword by David J. Barron
- Foreword by Roberta G. Williams
- Preface
- Acknowledgments
- Chapter 1: Definition and terminology
- Problems with terminology
- Definition of various morphologies in ``functionally´´ single ventricle
- References
- Chapter 2: Epidemiology
- Incidence
- Risk factors
- Survival
- References
- Chapter 3: Genetics of single ventricle congenital heart disease
- Introduction
- Developmental etiology of single ventricle
- Evidence for heritability of single ventricle congenital heart disease
- Genetic contributions to single ventricle heart disease
- Aneuploidy
- Structural variants
- Rare single gene variants
- Noncoding variants
- Genomics and clinical outcomes
- Future directions: Functional genetic models
- References
- Chapter 4: Morphology
- Introduction
- Philosophical considerations relative to nomenclature
- The anatomy of the functionally univentricular heart
- Hypoplastic left heart syndrome
- Left ventricular hypoplasia with deficient ventricular or atrioventricular septation
- Pulmonary atresia with intact ventricular septum
- Atrioventricular valvar atresia
- Double inlet ventricle
- Straddling atrioventricular valves
- Complex double outlet ventricle
- Morphogenesis
- References
- Chapter 5: Associated cardiac and noncardiac malformations
- Introduction
- Associated cardiac malformations
- General assessment
- Associated cardiac malformations in specific morphologies of single ventricle
- Double inlet single ventricle
- Tricuspid atresia
- Hypoplastic left heart syndrome
- Pulmonary atresia with intact ventricular septum
- Atrioventricular septal defect
- Double outlet right ventricle.
- Congenitally corrected transposition of the great arteries
- Straddling a-v valve
- Associated noncardiac malformations
- Extra-cardiac anomalies
- Genetic anomalies
- Gene testing
- Outcomes
- Adult congenital heart disease
- Socioeconomic impact on outcomes
- References
- Chapter 6: Fetal diagnosis
- Prenatal detection
- Counseling
- References
- Chapter 7: Ethical implications of fetal diagnosis
- Conclusions
- References
- Chapter 8: Fetal interventions
- Catheter-based fetal cardiac intervention for left-sided obstructive heart disease
- Background
- Fetal aortic balloon valvuloplasty
- Patient selection
- Procedural technique
- Procedural outcomes
- Postnatal outcomes
- Hypoplastic left heart syndrome with highly restrictive or intact atrial septum
- Fetal atrial septal stent placement
- Patient selection
- Procedural technique
- Procedural outcomes
- Postnatal outcomes
- Medical interventions for left-sided congenital heart disease
- Maternal hyperoxygenation
- References
- Chapter 9: Differences in fetal brain development
- Introduction
- Foundation of fetal brain and cerebrovascular development
- Foundation of fetal circulation
- Fetal brain differences in single ventricle pathophysiology
- Fetal cerebrovascular changes
- Fetal autonomic nervous system changes
- Fetoplacental changes
- Macrostructural fetal brain differences
- Microstructural brain differences in single ventricle pathophysiology
- Implications on postnatal years
- Conclusions
- References
- Chapter 10: Postnatal diagnosis
- Introduction
- Clinical presentation
- Diagnostic tools
- Critical congenital heart disease screening
- Chest X-ray
- Electrocardiogram
- Echocardiography
- Anatomical assessment using segmental anatomy approach
- Essential anatomic features needed for decision-making.
- Essential functional assessment needed for decision-making
- Cardiac catheterization and angiography
- Hemodynamic assessment
- Role in pulmonary atresia with intact ventricular septum
- Advanced cross-sectional imaging
- Cardiac computed tomography angiography (CTA)
- Cardiac magnetic resonance imaging (MRI)
- Volume-rendered 3D reconstructions
- Conclusion
- References
- Chapter 11: Pathophysiology
- Type and degree of mixing at the intracardiac level
- Presence and degree of atrioventricular valve malfunction
- Presence and degree of outlet obstruction
- Effective size of the interventricular communication
- Progression of the systemic or pulmonary obstructions
- Progression of the atrioventricular valve(s) regurgitation
- Progression of ventricular hypertrophy
- Progressive deterioration of the systolic function
- Progressive disturbances of atrioventricular conduction
- Progressive left atrial hypertension
- Restrictive interatrial communication
- Obstructed atrioventricular junction
- Progressive development of pulmonary vascular obstructive disease
- Pathophysiology patterns
- Restricted pulmonary blood flow
- Unrestricted pulmonary blood flow, with or without obstruction to the systemic flow
- Balanced circulation
- Pathophysiology: Ratio of pulmonary blood flow to systemic blood flow
- References
- Chapter 12: Neonatal care
- Prenatal diagnosis
- Palliative care
- Delivery planning
- Newborn pulse oximetry screening
- Postnatal diagnosis
- Medical transport
- Transitional physiology and neonatal resuscitation
- Newborn assessment
- Prostaglandin/ductal patency
- Ductal-dependent congenital heart disease
- Special considerations
- Prematurity and congenital heart disease
- References
- Chapter 13: Required diagnostic information for decision-making
- Introduction
- Fetal diagnosis.
- Postnatal evaluation
- Electrocardiogram
- Chest radiography
- Echocardiography
- Advanced imaging
- Cardiac catheterization
- Decision-making in the operating room
- Hybrid palliation
- Putting the picture together and additional considerations
- Following stage 1 palliation
- Determination of bidirectional Glenn candidacy
- Anatomic assessment
- Functional assessment
- Putting the picture together
- Prior to stage 3 palliation
- Further assessment
- Fenestration
- Future directions
- Putting the picture together
- Conclusions
- References
- Chapter 14: Anesthesia
- Introduction
- Perioperative management
- Preoperative management
- Intraoperative management
- Anesthetic drugs
- Airway management
- Central access
- Arterial access
- Cardiopulmonary bypass
- Systemic inflammatory response
- Anticoagulation
- Cardiopulmonary bypass management
- Neuro injury and neuromonitoring
- Separation from cardiopulmonary bypass
- Coagulation and bleeding post bypass
- Physiological changes of staged repair
- Stage I
- Stages II and III
- Long term
- Extracorporeal membrane oxygenation
- Enhanced recovery after surgery
- Noncardiac surgery
- Cardiac transplantation
- Future
- References
- Chapter 15: Single ventricular interventional procedures
- Stage I interventions
- Ductal stenting
- Balloon valvuloplasty
- Right-ventricular outflow tract stenting
- Internal flow restrictors
- Hybrid procedures
- Balloon atrial septostomy
- Radiofrequency perforation of the atrial septum
- Atrial septal stenting
- Stenting of Sano or Blalock-Taussig shunts
- Aortopulmonary collaterals
- Stage II interventions
- Stenoses of the superior cavopulmonary connection
- Elimination of accessory blood flow in pulsatile bidirectional Glenn
- Veno-venous collaterals
- Aortopulmonary collaterals.
- Porto-systemic venous connections
- Transcatheter bidirectional Glenn procedure
- Stage III interventions
- Stenoses of the total cavopulmonary connection pathway
- Stenosis of the Damus-Kaye-Stansel and arch
- Lymphatic interventions
- Veno-venous collaterals
- Aortopulmonary collaterals
- Pulmonary vein interventions
- Percutaneous Fontan completion
- Fontan fenestration creation
- Fontan fenestration titration
- Fontan fenestration closure
- References
- Chapter 16: Surgical procedures
- Introduction
- First-stage palliation
- Goals of surgery
- Unobstructed systemic and pulmonary blood flow
- Unobstructed systemic and moderately restrictive pulmonary blood flow
- Unobstructed systemic and highly restricted pulmonary blood flow
- Unobstructed pulmonary and restrictive systemic blood flow
- Superior cavo-pulmonary anastomosis
- Fontan completion
- Biventricular repair
- Fetal development of small left heart structures
- Endocardial fibroelastosis
- Left-ventricular recruitment
- Biventricular conversion
- Atrioventricular valve regurgitation
- References
- Chapter 17: Perioperative management
- Introduction
- Pathophysiology
- Preoperative management
- Stage 1
- Stage 2
- Stage 3
- Intraoperative management
- Interventions and postoperative management
- Stage 1
- Stage 2
- Stage 3
- Teamwork
- References
- Chapter 18: Interstage monitoring
- Introduction
- Interstage pathophysiology
- Interstage monitoring strategies
- How does home monitoring work?
- Drawbacks of interstage monitoring
- The influence of interstage monitoring on stage 2 palliation
- Summary
- References
- Chapter 19: Required postoperative diagnosticinformation
- Postoperative diagnostic evaluation after stage 1 palliation
- Postoperative diagnostic evaluation after stage 2 palliation.