Survival Outcomes in Heart Transplant Patients; Specific Focus on ACHD Patients and Their Outcomes as Compared to Dilated and Ischemic Cardiomyopathy Patients

Purpose Congenital heart disease (CHD) 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. This was a retrospective analysis of the ISHLT Registry data. Methods Adult heart transplant recipients between 2004 and 2014 were identified from the ISHLT registry. We used Kaplan-Meier analysis test to evaluate overall survival, 1-year survival and 1-year conditional survival among etiology groups and multivariable Cox proportional hazard (PH) model to assess the association between etiology of cardiomyopathy and 1-year, 1-year conditional all-cause mortality and cause-specific mortality. Results We included 30,130 heart transplant recipients: 1,839 (6.1%) CHD, 16,044 (53.2%) DCM and 12,247 (40.6%) ICM patients. By multivariable analysis CHD or ICM patients were at significantly higher mortality risk than DCM during the first year post-transplant. One-year survival was 78.3% in CHD, 84.3% in ICM and 86.2 in DCM patients. One-year conditional post-transplant survival was similar in CHD and DCM (HR 0.97, 95% CI 0.83-1.13) while ICM patients had higher long-term mortality risk than DCM patients (HR 1.17, 95% CI 1.09-1.26). Cause-specific mortality analyses showed that CHD patients were at higher risk for infection and graft failure related mortality than DCM patients within the first year post-transplant. Conclusion Patients with CHD or ICM are at significantly higher early all-cause mortality than DCM patients. CHD and DCM patients had comparable long-term survival and significantly improved long-term survival compared to ICM patients. This has implications for CHD patient selection for transplant and perioperative management.