Outcomes of Fontan patients undergoing combined heart-liver transplantation in pediatric hospitals across the United States

Infographic · Dec 2025 · 1 min read

In brief

Multi-institutional study of 34 Fontan patients undergoing combined heart-liver transplantation shows 85% one-year survival, with most procedures performed after 2019. Three-year outcomes matched heart-only transplant recipients, demonstrating successful management of this complex population with Fontan-associated liver disease.

82% of Fontan CHLT cases occurred after 2019, reflecting rapid growth in this transplant strategy for Fontan-associated liver disease
One-year survival was 85% overall; all deaths occurred within 6 months, suggesting early post-op period is highest risk
Patients with MELD/PELD ≤10 had 91% hospital survival vs 82% for scores >10, indicating lower scores predict better outcomes
Three-year survival identical between CHLT and heart-only transplant (85% vs 84%), validating CHLT as viable option
Conditional on hospital discharge, long-term survival is excellent, supporting CHLT for select Fontan patients with liver disease

Written by the GCMD Library team from the infographic.

The infographic uses a teal and white color scheme with medical illustrations including a heart with ECG line, an anatomical heart diagram, a liver illustration, and healthcare workers attending to a patient. Data is presented in sections with large statistics highlighted in colored text boxes. The layout flows from top to bottom with key findings emphasized through size and color contrast.

Grant Chappell, Amir Mehdizadeh-Shrifi, Darren Turner, Alexander Bondoc, Suzanne Evans, Alexander G Miethke, Gregory Tiao, Meghan M Chlebowski, Alexander R Opotowsky, David Lehenbauer, Marco Ricci, Awais Ashfaq, David L S Morales

Background: With 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.

Methods: Patients 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.

Results: A 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).

Conclusions: This 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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