Heart transplantation outcomes in patients with Fontan circulation: Updated systematic review and meta-analysis
In brief
Around 17% of Fontan patients die around transplant, 70% survive five years
In a meta-analysis of 1,186 Fontan patients who underwent heart transplantation, roughly one in six died perioperatively, while about seven out of ten were alive at five years and six out of ten at ten years. Survival varied by transplant era and center experience, highlighting the need for careful patient selection and expertise.
- Journal
- JTCVS open (Q1)
- Published
- 7 May 2026
- Study design
- Systematic review / meta-analysis of RCTs
- Evidence level
- Level 1, High (CEBM 1a)
- Authors
- Andrey Semyashkin, Alina Sandica, Stephan Schubert, Stephan Molatta, Leonid Gherta, Lotfi Ben Mime
- PMID
- 42604301
- DOI
- 10.1016/j.xjon.2026.101855
Why clinicians should know about it
- Picked for Epidemiology (top studies of the week, 23 August 2026).
- Picked for Pediatrics and Child Health (top studies of the week, 23 August 2026): Fontan patients’ transplant survival informs long‑term care
- Picked for Cardiology and Cardiovascular Medicine (paper of the day, 20 August 2026): Heart transplantation outcomes in Fontan patients
Abstract
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%; I 2 = 62.8%; 95% prediction interval ≈6%-33%). Pooled 1-year posttransplant survival was 81.1% (95% CI, 74.9-85.9%; I 2 = 73.5%; prediction interval ≈67%-94%). Pooled survival was 70.1% (95% CI, 64.3-75.3%) at 5 years and 60.1% (95% CI, 54.1-65.7%) at 10 years. Moderate heterogeneity was observed and was attributable to differences in era, center experience, and patient selection. 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.
Abstract as published, via PubMed.
For healthcare professionals. The summary is generated by AI from the published abstract, and the evidence level is assigned automatically from the study design on the Oxford CEBM hierarchy. Neither is medical advice. Read the full paper before changing practice.