Heart transplantation outcomes in patients with Fontan circulation: Updated systematic review and meta-analysis.

Objective: A subset of patients with Fontan circulation develops progressive circulatory failure requiring advanced therapies such as heart transplantation. We performed an updated systematic review and meta-analysis to evaluate survival and complication rates after the Fontan operation and subsequent transplantation. Methods: MEDLINE, Embase, Web of Science, and Cochrane CENTRAL were searched from inception to January 1, 2026, for randomized trials, cohort studies, and registry analyses reporting survival, complications, or interventions after Fontan completion. Primary end points were peritransplant mortality, transplant-free survival, and posttransplant survival at 1, 5, and 10 years. Secondary end points included protein-losing enteropathy, Fontan-associated liver disease, arrhythmia, and transplantation-related outcomes. Random-effects meta-analyses of logit-transformed proportions were performed using Hartung-Knapp adjustment. Results: Fifteen transplant cohorts comprising 1186 patients with Fontan circulation undergoing heart transplantation were included. Early mortality after Fontan completion remained low (∼1%) and was not pooled. Peritransplant mortality ranged from 8% to 31%, with a pooled estimate of 17.3% (95% CI, 12.7-22.9%; Conclusions: This updated meta-analysis demonstrates that peritransplant mortality in patients with Fontan circulation remains substantial (∼17%), whereas long-term survival after transplantation is acceptable, approximating 70% at 5 years and 60% at 10 years.