Purpose: The population of Adult Congenital Heart Disease (ACHD) patients requiring heart transplantation is expanding but there are few published results for outcomes. Controversy over organ allocation to ACHD exists as they may have higher perioperative risk, particularly single ventricle/Fontan patients. Therefore we examined the UK NHSBT Registry data to assess ACHD outcomes. Methods: Adults (aged >16 years) undergoing a first heart transplant between 1995-2014 were identified. Registration-, operative-and post-transplantation-related clinical data were compared between adults with congenital heart disease (ACHD) and other adult de-novo heart transplant recipients. Posttransplant survival was compared between the groups using the Kaplan-Meier method. The ACHD sub-group registered between 2005-2014 were analysed to assess the impact of complex (single ventricle) morphology on survival using the log rank test. Results: ACHD patients were younger and less likely to be male, waiting times were longer and mechanical circulatory support less common. Although early survival was worse the 1 year creatinine was better and the long term outcomes were similar. In the recent era 2005-2014 there were 74 transplants for ACHD 25 of which were single ventricles. Comparing single ventricles with bi-ventricles, one-year post-transplant survival was similar (95.9% single ventricle vs. 93.5%), (p= 0.4). Fontan survival was 89.7% at 4-years post-transplantation. Note that these analyses are not risk-adjusted. Conclusion: Despite ACHD having worse 90-day survival, long-term posttransplantation survival was comparable between ACHD de-novo heart transplant recipients and those with other diagnoses. In the recent era single main etiology was idiopathic dilated cardiomyopathy (36%), also followed by ischemic cardiomyopathy (22.7%) and by Chagas cardiomyopathy (22.7%). Almost all patients were hospitalized at the time of transplantation (96 %) and in use of intravenous inotrope, of these 33.3% were also with IABP and only 6.7 % used some mechanical circulatory support, such as ECMO and centrifugal pump (CENTRIMAG). The comparison with the data from the ISHLT is below and all showed statistically significant difference (p < 0.001). Conclusion: Our population differs from the registry of ISHLT, since the vast majority of patients are hospitalized at the time of transplant, in use of intravenous inotrope and / or intra-aortic balloon, rarely in use of circulatory assist device. These data suggest that in our population patients are transplanted in advanced stages of heart failure, rarely with mechanical circulatory support other than IABP as a bridge to transplant.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.