Single-center perioperative management and 1-year heart transplantation outcomes for patients with adult congenital heart disease.

Additional data are needed to define the optimal listing strategy and peri-transplant management for adults with congenital heart disease (ACHD). In this study, we evaluated the perioperative management and 1-year outcomes for 30 patients who underwent orthotopic heart transplantation (OHT) for ACHD. We conducted a single-center retrospective case series of all patients who received an OHT at our institution for ACHD from January 1, 2017 through June 30, 2024. Descriptive statistical analyses were used to illustrate the baseline characteristics, peri-transplant management, and 1-year outcomes of participants. Seventeen (56.7%) patients received a heart-only transplant, 4 (13.3%) had a heart-lung transplant, 8 (26.7%) had a heart-liver transplant, and 1 (3.3%) had a heart-liver-kidney transplant. Embolization of aortopulmonary and venous collateral vessels before transplantation was performed in 12 (80%) Fontan patients, with a median of 2 procedures per patient. Twenty-eight (93.3%, n = 30) patients were alive at 90 days, and 26 (92.9%, n = 28) were alive at 1 year. This cohort of ACHD patients had a 92.9% 1-year survival rate, consistent with other high-volume centers in the United States. In this paper, we discuss our institutional practices that address barriers to transplant for ACHD patients, such as early referral for transplant, indications for listing, pretransplant embolization of collateral vessels, and multiorgan transplant.