Pediatr Transplant. 2025 Nov;29(7):e70174. doi: 10.1111/petr.70174.
ABSTRACT
BACKGROUND: Fontan-associated liver disease can progress to advanced fibrosis, raising the potential need for combined heart-liver transplantation (CHLT) in selected patients. However, the benefits of CHLT over isolated orthotopic heart transplantation (HT), particularly in terms of mortality, remain uncertain. In this systematic review, we compared mortality outcomes following CHLT versus HT in patients with Fontan circulation, with the aim of supporting clinical decision-making.
METHODS: This systematic review was conducted according to the 2020 PRISMA guidelines and registered in PROSPERO. PubMed, Scopus, and Embase were searched. Studies examining HT or CHLT in patients with Fontan circulation that provided information about total and/or 1-year mortality were included. Bias risks were assessed using the Newcastle-Ottawa Scale. We used random- and fixed-effect models, depending on heterogeneity, to estimate pooled effects.
RESULTS: Sixteen studies were included in this analysis. CHLT was associated with a lower mortality rate per patient-year compared to HT (0.03 vs. 0.09; p < 0.01). However, after excluding studies in which transplantations were performed before the year 2000, the difference between groups was no longer statistically significant. One-year mortality rates were also not significantly different between CHLT and HT (0.09 vs. 0.14; p = 0.28), with similar results observed after excluding pre-2000 studies.
CONCLUSION: Overall, this systematic review suggests that CHLT may result in mortality rates comparable to those of isolated HT. These findings support the consideration of CHLT in patients with concomitant liver disease and reinforce the importance of comprehensive liver evaluation in transplant candidates.
PMID:40916564 | DOI:10.1111/petr.70174
