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Outcomes after the Fontan procedure
1Department of Pediatrics, Children's Hospital of Philadelphia and University of Pennsylvania School of Medicine, Philadelphia, 19104, USA. marino@email.chop.edu
Insights
Advances in pediatric cardiology and surgery have improved survival for infants with single-ventricle heart defects undergoing the Fontan procedure. However, significant long-term health issues persist despite surgical innovations.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Surgery
- Intensive Care Medicine
Background:
- Significant advancements in pediatric cardiac care have improved survival rates for infants with critical congenital heart disease.
- The single-ventricle population has particularly benefited from staged palliation, including the Fontan procedure, leading to increased survival.
- Recent studies highlight progressive improvements in outcomes following the Fontan procedure due to surgical and technological innovations.
Purpose of the Study:
- To review recent outcomes and persistent morbidities in single-ventricle patients after Fontan completion.
- To assess the impact of surgical innovations and technological advancements on Fontan procedure outcomes.
Main Methods:
- Review of recent studies focusing on outcomes after the Fontan procedure.
- Analysis of surgical innovations (lateral tunnel, extracardiac modifications, fenestration) and technological improvements (modified ultrafiltration).
Main Results:
- Improved early postoperative survival and intermediate/late outcomes reported in recent studies.
- Surgical and technological advancements have contributed to better results after Fontan completion.
- Despite improvements, significant morbidities remain, impacting long-term patient health.
Conclusions:
- While the Fontan procedure has seen survival improvements, persistent morbidities necessitate ongoing research and management strategies.
- Key long-term complications include cardiac dysfunction, hemodynamic abnormalities, reduced exercise capacity, arrhythmias, protein-losing enteropathy, growth issues, and cognitive deficits.
Abstract:
Over the past two decades, advances in congenital heart surgery, pediatric cardiology, and intensive care medicine have dramatically increased the survival of infants with critical congenital heart disease. The group of patients that has perhaps benefited the most from this progress has been the single-ventricle population. Staged palliation culminating in the Fontan procedure has resulted in a decreasing mortality rate and an increase in the number of single-ventricle survivors. Over the past 18 months, many studies have focused on outcomes after the Fontan procedure. These reports demonstrate progressive improvement in early postoperative survival and intermediate and late postoperative outcomes due to surgical innovations, such as the lateral tunnel and extracardiac Fontan modifications, and fenestration, as well as technological improvements, such as modified ultrafiltration. Despite these improvements, significant morbidity remains after the Fontan completion, including myocardial systolic and diastolic dysfunction, systemic arterial and venous hemodynamic abnormalities, diminished exercise capacity, arrhythmias, protein-losing enteropathy, somatic growth retardation, neo-aortic valve root dilation and insufficiency, thromboembolic complications, and below-average cognitive development.