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Long-term survival and complications of Fontan patients: where do we stand?
Adam Najm1, Fayez Yassine1, Assyl Amhaz1
1Faculty of Medicine, American University of Beirut Medical Center Beirut, Lebanon.
Insights
Single ventricle disease (SVD) requires the Fontan procedure for palliation. While survival has improved, lifelong complications like Fontan-associated liver disease (FALD) necessitate systematic monitoring and risk stratification for better patient outcomes.
Area of Science:
- Cardiology
- Pediatric Cardiology
- Congenital Heart Disease
Background:
- Single ventricle disease (SVD) is a severe congenital heart defect (CHD).
- The Fontan procedure is the final stage of surgical palliation for SVD, following the Norwood and Glenn procedures.
- Modern surgical advances have shifted focus from mortality to improving long-term survival and quality of life for Fontan patients.
Purpose of the Study:
- To review recent cohort studies on Fontan patients.
- To identify long-term outcomes and associated risk factors after the Fontan procedure.
- To emphasize the need for risk stratification and systematic monitoring of lifelong complications.
Main Methods:
- A narrative review of literature.
- Searched PubMed, Scopus, and Google Scholar up to December 2025.
- Focused on recent cohort studies concerning Fontan patients.
Main Results:
- The Fontan procedure is not curative and leads to numerous morbidities, including arrhythmia, heart failure, and Fontan-associated liver disease (FALD).
- FALD can affect over half of patients within 35 years, with risks of cirrhosis and hepatocellular carcinoma.
- Preoperative factors (demographic, surgical, biomarker) predict postoperative complications.
- Fontan patients demonstrate excellent short- and long-term survival rates compared to previous eras.
Conclusions:
- Improved survival in Fontan patients shifts focus to managing lifelong complications.
- Fontan-associated liver disease (FALD) requires prioritized, early, and systematic monitoring.
- Healthcare systems must adapt to provide lifelong follow-up and early detection strategies for Fontan survivors.
Abstract:
Single ventricle disease (SVD) is a rare but severe form of congenital heart disease (CHD) which requires surgical palliation through the Fontan procedure. This operation, which was pioneered in 1971, has become the final part of a surgical pathway after the Norwood and Glenn procedures. The pathway aims to reduce the load on the functional ventricle whilst improving systemic blood oxygenation. Advances in surgical technique and the modern era have shifted the approach from addressing mortality concerns to offering a lifeline to patients in need. With improved survival, the Fontan population grows which requires an emphasis on the lifelong complications that these individuals face along with specific risk factors that predispose them to these issues allowing for risk stratification and systematic monitoring. This narrative review aims to summarize the recent cohort studies on Fontan patients to identify long-term outcomes of the procedure along with their associated risk factors. The literature review was conducted till December 2025 using PubMed, Scopus, and Google Scholar, the procedure itself is not curative. I has numerous morbidities including arrhythmia, heart failure, neurocognitive delays, protein-losing enteropathy, renal dysfunction, and Fontan-associated liver disease (FALD). FALD specifically may affect over half of Fontan patients within 35 years and the seriousness of FALD sequelae including cirrhosis and hepatocellular carcinoma underscores the need to prioritize early and systematic monitoring. Preoperatively, demographic, surgical, and biomarker risk factors have been shown to be predictors of postoperative complications/mortality. Overall, Fontan patients tend to have excellent survival rates over both the short and long terms compared to prior surgical eras. As postoperative concerns now shift from early mortality to long-term complications, our healthcare system must adapt to ensure lifelong follow-up and a systematic approach for early detection.
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