The Challenging Pathway Toward Heart Transplant Listing for Adult Congenital Heart Disease Patients

Adult congenital heart disease (ACHD) patients are at risk for end-stage heart failure; heart transplantation (Htx) represents the only definitive therapy available although not easily achievable for all patients. The study aims to assess the pathway difficulties and outcomes of ACHD patients with end-stage heart failure referred for Htx evaluation. This is a single center retrospective study on ACHD patients with end-stage heart failure referred to Htx evaluation from 2004 to 2015. Demographic data, medical history, failure modality, and follow-up were obtained from patient charts. End-points were Htx list enrollment, transplant, and survival. Statistical analysis was performed comparing patients listed and not listed. There were 21 ACHD patients with end-stage heart failure referred to Htx evaluation. Transplant listing was declined for 12 (57%) meanwhile 9 patients were listed. Htx was successfully achieved in 3 patients after 24 and 36 months, respectively. Three patients are still on the wait list and three died while waiting, with a listed group mortality of 33.3% (3/9). Mortality occurred in first 18 months after Htx list enrollment. Not listed group mortality was 50% (6/12) and occurred after a median time of 17.5 months (IQR: 9-23 months). There was no difference in survival (P = 0.574) between listed and not listed (89, 63, and 63% vs. 83, 56, and 47% at 12-24-48 months). Follow-up median duration was 27 months (IQR: 14-56 months). Heart transplant listing for ACHD patients with end-stage heart failure is hard to obtain. Almost 2/3 of the patients were declined. Survival for these patients is reduced severely either in waiting list for transplant or excluded indicating the potential need of mechanical circulatory support as bridge to transplant or as destination therapy to improve survival likelihood.