Heart transplant evaluation in adults with congenital heart disease presenting with advanced heart failure

Abstract

Background: Adults with congenital heart disease (CHD) with stage D heart failure (HF) have 1-year mortality risk of almost 40% without heart transplantation, and as a result, heart transplant evaluation is the logical next step in the management of these patients. The purpose of this study was to describe the clinical characteristics and outcome of adults with CHD referred for heart transplant evaluation.

Method: Adults with CHD referred for heart transplant evaluation were divided into 3 treatment pathways after multidisciplinary transplant evaluation. (i) Transplant-listed group (patients with stage D HF and eligible for transplant); (ii) Transplant-declined group (patient with stage D HF but had contraindications for transplant); (iii) Transplant-deferred group (patients without stage D HF).

Results: There were 416 patients who underwent transplant evaluation (transplant-listed group [N = 159,38%]; transplant-declined group [N = 101,24%]; transplant-deferred group [N = 156;38%]). Of 159 patients in the transplant-listed group, 110 underwent heart transplant. The patients who received heart transplant had similar survival compared to patients without stage D HF. Among patients with stage D HF (N = 260), heart transplantation was associated with a 9-fold reduction in all-cause mortality. End-organ dysfunction and congestion were common among patients who were declined for transplant listing or died while waiting for transplant and were associated with all-cause mortality.

Conclusions: Heart transplantation was associated with improved survival in adults with CHD presenting with stage D HF. End-organ dysfunction and congestion were associated with greater odds of being declined for transplant and waitlist mortality, suggesting need for earlier referral for transplant evaluation to improve outcomes.

Condensed abstract: Among 416 patients referred for heart transplant evaluation, 38% (N = 159) were listed for transplant because of stage D HF of which 110 underwent transplant, 24% (N = 101) were declined for transplant listing because of contraindications to transplant, and 38% (N = 156) were deferred for transplant listing because they did not have stage D HF. Among patients with stage D HF (N = 260), heart transplantation was associated with a 9-fold reduction in all-cause mortality. End-organ dysfunction and congestion were associated with greater odds of being declined for transplant and waitlist mortality, suggesting need for earlier referral for transplant evaluation.

Keywords: Heart failure; Heart transplant evaluation; Prognostication.