Long-term survival and complications of Fontan patients: where do we stand?

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.