Post-transplant survival in adult congenital heart disease patients as compared to dilated and ischemic cardiomyopathy patients; an analysis of the thoracic ISHLT registry

Previous studies have shown that adult congenital heart disease (ACHD) is associated with high early post-transplant mortality but improved long-term survival in comparison to the overall heart transplant population. We aimed to evaluate survival outcomes of ACHD in adult transplant recipient patients as specifically compared to ischemic (ICM) and dilated cardiomyopathy (DCM) groups. Adult heart transplant recipients between 2004 and 2014 were identified from the ISHLT registry. We used Kaplan-Meier analysis to evaluate overall survival, 1-year survival, and 1-year conditional survival among etiology groups and multivariable Cox proportional hazard (PH) models to assess the association between etiology of cardiomyopathy and 1-year and long-term all-cause mortality and cause-specific mortality. We included 30 130 heart transplant recipients. One-year survival was 78.3% in ACHD, 84.3% in ICM, and 86.2% in DCM patients (P <.001). By multivariable analysis, during first post-transplant year, ACHD and ICM patients were at significantly higher mortality risk than DCM. Adjusted post-transplant mortality risk, conditional on 1-year survival, was not statistically different in ACHD and DCM while ICM patients had 17% higher long-term mortality risk than DCM patients leading to overall worse outcomes in ICM patients. Therefore, ICM patients have poorer outcomes in comparison to both DCM and ACHD patients.