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.
