Outcomes of Adults with Congenital Heart Disease Undergoing Heart and Heart-Liver Transplantation: A Single-Center Experience

OBJECTIVE: The number of adults with congenital heart disease (ACHD) continues to rise, often presenting with multiorgan dysfunction. This is the largest single-center series reporting outcomes of ACHD who underwent either heart transplantation (HT) or combined heart-liver transplantation (CHLT). METHODS: Adults (age ≥ 18) with congenital heart disease were identified in retrospective review. All-cause mortality was compared in a Cox-proportional hazards model. Mann-Whitney and Chi-squared tests were used to analyze differences between groups. RESULTS: Between 1987 and 2024, 81 ACHD underwent HT (N=67, 83%) or CHLT (N=14, 17.3%) at our institution. There has been an increasing number of HTs and CHLTs performed for ACHD at our institution, especially over the past decade. Patients who underwent CHLT were more likely to have single-ventricle physiology (57% vs 20% for CHLT vs HT, respectively, p=.007). Amongst patients palliated with prior Fontan, the median time between Fontan and transplant was 19 years for HT and 26 years for CHLT. The en bloc technique was used for 13 of 14 patients who underwent CHLT (92.9%). Median cross-clamp time and total heart ischemic time were similar between HT and CHLT (p=.20, p=.19, respectively). Short- and mid-term survival were comparable for patients who underwent CHLT and HT (Figure 2). CHLT recipients exhibited a higher degree of freedom from acute cellular rejection, antibody-mediated rejection, and chronic rejection, though this did not reach statistical significance when compared to HT recipients (Figure 3). CONCLUSION: Our experience demonstrates that both HR and CHLT can be performed safely with excellent outcomes.