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The Fontan operation: the long-term outlook
1Albert Einstein College of Medicine and The Children's Hospital at Montefiore, Bronx, New York, USA.
Current Opinion in Pediatrics
|August 18, 2015
Summary
Long-term survival after the Fontan procedure is improving, but late complications like arrhythmias and heart failure remain significant concerns for single-ventricle patients. Research now focuses on identifying at-risk individuals to improve outcomes.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease
- Adult Congenital Heart Disease
Background:
- The Fontan procedure offers physiologic palliation for single-ventricle defects.
- Improved perioperative survival highlights the need to address long-term morbidities.
Purpose of the Study:
- To characterize late morbidities in Fontan survivors.
- To develop risk-prediction models for adverse outcomes in long-term Fontan patients.
Main Methods:
- Review of retrospective and prospective observational studies.
- Analysis of data from long-term survivors of the Fontan procedure.
Main Results:
- Ten-year survival is high (94-98%), but conditional survival at age 40 for those >18 is 60%.
- Atrial arrhythmias and heart failure are primary causes of mortality.
- Hypoplastic left heart syndrome, hepatic dysfunction, and reduced exercise tolerance are associated with worse outcomes.
Conclusions:
- The Fontan procedure has transformed single-ventricle care over decades.
- Ongoing research identifies patients at risk for late complications, guiding future management.
