Outcomes of Fontan Patients Undergoing Combined Heart-Liver Transplantation in Pediatric Hospitals Across the United States.

With increasing patients successfully surviving Fontan palliation, there is a rise in Fontan associated liver disease (FALD) and transplant strategies inclusive of combined heart-liver transplantation (CHLT). Therefore, we reviewed a combined dataset assessing outcomes of pediatric patients undergoing CHLT. Patients undergoing Fontan CHLT from 2010-2024 at pediatric hospitals were queried from a merged dataset of the Pediatric Health Information System and United Network for Organ Sharing. 2:1 matching was completed with Fontan heart-only transplant recipients. In total, 34 patients underwent Fontan CHLT at 9 pediatric hospitals from 2010-2024, with 82% (28/34) after 2019. Almost half (47%, 16/34) were 18 or younger at the time of CHLT, with a median age of 19 years [15-24]. Over two-thirds (68%) had HLHS, 38% DILV, 26% DORV, and 18% tricuspid atresia. One-third (11/34) of patients had a MELD/PELD score of ≤10 at CHLT, with a 91% hospital survival, and those with a score >10 had an 82% survival. Overall, one-year survival was 85%, 94% for those >18 years old, and 75% for those ≤18 years. All deaths occurred before hospital discharge and within 6 months of transplant, with mean follow-up of 4 years. Compared to HT, three-year survival was similar (CHLT: 85% vs. HT: 84%, log-rank p-value: 0.96). 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 the transplant management for this complex population.