Long-term Outcomes after Fontan Single Ventricle Palliation: A Systematic Review and Meta-Analysis of Regional Registry Data.

BACKGROUND

While several Fontan patient registries have been established, a systematic analysis of these collective data does not exist to provide insight into long-term outcomes. This meta-analysis aims to provide an updated assessment of published Fontan patient outcomes.

METHODS

A systematic search identified registry studies reporting long-term outcomes following the Fontan procedure. A reconstructed individual patient data-based meta-analysis assessed survival and failure rates. Subgroup analyses evaluated outcomes across different Fontan techniques.

RESULTS

Six registry studies, including 7,854 Fontan patients, were included. The mean age at Fontan completion was 5.8 years, with a mean follow-up age of 21.4 years. 58.7% of patients were male. An extracardiac conduit (ECC) was used in 62.1% of patients, a lateral tunnel (LT) in 23.1%, and the atriopulmonary (AP) technique in 14.8%. 5.1% of patients underwent early surgical revision. Cumulative survival rates were 93.6% at 10 years, 87.2% at 20 years, and 80.2% at 30 years. Upon comparison, both ECC and LT were associated with significantly improved survival compared to the AP technique (p<0.001). Finally, freedom-from-Fontan-failure rates at 10, 20, and 30 years of follow-up were 76.6%, 74.9%, and 70.2%, respectively.

CONCLUSIONS

These data demonstrate that Fontan palliation yields better long-term survival than previously reported. ECCs are most common, and both ECC and LT techniques outperform the AP technique. These data provide important context for patient counseling regarding expected long-term outcomes after single-ventricle palliation and underscore the need for lifelong specialized surveillance in this population.