ArticleThe Journal of thoracic and cardiovascular surgery2026
Outcomes of Fontan patients undergoing combined heart-liver transplantation in pediatric hospitals across the United States.
Article in The Journal of thoracic and cardiovascular surgery, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 3 papers.
What it found
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Who cites it
3 citing papers in PubMed.
- Fontan Failure: Current State of the Art in Mechanical Circulatory Support and Transplantation.Children (Basel, Switzerland) · 2026Review
- Article
- Prognostic biomarkers in Fontan associated liver disease.Translational pediatrics · 2026Review
Corrections and comments
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Authors and funding
13 authors.
Funding
Abstract
backgroundWith increasing numbers of patients surviving Fontan palliation, there is a rise in Fontan-associated liver disease and transplantation strategies inclusive of combined heart-liver transplantation (CHLT). Therefore, we reviewed a combined dataset assessing outcomes of pediatric patients undergoing CHLT.
methodsPatients undergoing Fontan CHLT between 2010 and 2024 at pediatric hospitals were queried from a merged dataset of the Pediatric Health Information System and United Network for Organ Sharing. Matching (2:1) was completed with Fontan heart-only transplant recipients.
resultsA total of 34 patients underwent Fontan CHLT at 9 pediatric hospitals between 2010 and 2024, 82% (n = 28) after 2019. Almost one-half (47%; n = 16) were age ≤18 years at the time of CHLT, with a median age of 19 years (interquartile range, 15-24 years). More than two-thirds (68%) had hypoplastic left heart syndrome, 38% had double-inlet left ventricle, 26% had double-outlet right ventricle, and 18% had tricuspid atresia. One-third of the patients (n = 11) had a Model for End-Stage Liver Disease/Pediatric End-Stage Liver Disease score of ≤10 at CHLT, with 91% hospital survival, and those with a score >10 had 82% survival. One-year survival was 85% overall, 94% for those age >18 years, and 75% for those age ≤18 years. All deaths occurred before hospital discharge and within 6 months of transplantation, with a mean follow-up of 4 years. Three-year survival was similar in the CHLT and heart transplant -only (HT) groups (85% vs 84%; P = .96, log-rank test).
conclusionsThis multi-institutional analysis demonstrates the rapidly increasing numbers of Fontan palliated patients successfully undergoing CHLT in pediatric hospitals. Conditional on surviving to discharge, long-term survival has been excellent, demonstrating a success story in transplantation management for this complex population.
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