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Heart transplantation outcomes in patients with Fontan circulation: Updated systematic review and meta-analysis
Andrey Semyashkin1, Alina Sandica2,3, Stephan Schubert4,3
1Department of Pediatric Cardiac Surgery and Congenital Heart Defects, Klinikum Stuttgart, Stuttgart, Germany.
Insights
Patients with Fontan circulation undergoing heart transplantation face substantial peritransplant mortality around 17%. However, long-term survival post-transplant is acceptable, reaching approximately 70% at 5 years and 60% at 10 years.
Area of Science:
- Cardiology
- Pediatric Cardiology
- Cardiac Surgery
Background:
- Fontan circulation is a palliative surgical procedure for complex single-ventricle congenital heart defects.
- A subset of patients with Fontan circulation develop progressive circulatory failure, necessitating advanced treatments like heart transplantation.
- Evaluating outcomes after heart transplantation in this high-risk population is crucial for guiding clinical management.
Purpose of the Study:
- To conduct an updated systematic review and meta-analysis.
- To evaluate survival rates and complication profiles following heart transplantation in patients with Fontan circulation.
- To provide current evidence on the efficacy and risks of heart transplantation in this specific patient group.
Main Methods:
- Comprehensive search of major biomedical databases (MEDLINE, Embase, Web of Science, Cochrane CENTRAL) up to January 2026.
- Inclusion of randomized trials, cohort studies, and registry analyses reporting outcomes after Fontan completion and subsequent transplantation.
- Application of random-effects meta-analyses with Hartung-Knapp adjustment to pooled data on mortality and complications.
Main Results:
- Fifteen transplant cohorts including 1186 patients with Fontan circulation were analyzed.
- Peritransplant mortality was pooled at 17.3% (95% CI, 12.7-22.9%), with significant heterogeneity observed.
- Pooled posttransplant survival was 81.1% at 1 year, 70.1% at 5 years, and 60.1% at 10 years.
Conclusions:
- Heart transplantation in patients with Fontan circulation is associated with substantial peritransplant mortality.
- Despite the risks, long-term survival after heart transplantation is considered acceptable, with significant proportions surviving up to 10 years.
- Heterogeneity in outcomes may be attributed to variations in surgical era, center experience, and patient selection criteria.
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.