Abstract 18229: Heart Transplantation for Genetic Cardiomyopathies is Associated With Favorable Long-Term Survival and Graft Outcomes

Abstract

Introduction and hypothesis: Genetic cardiomyopathies (GNCM) are a spectrum of myocardial disorders that can lead to heart failure, and frequently portend the need for heart transplantation. Post-transplant outcomes in this subgroup of patients have not been examined in a large, multicenter transplant cohort. Methods: Patients who underwent first-time heart transplantation in the United States between 1987 and 2012 were retrospectively identified from the United Network for Organ Sharing database. Patients with hypertrophic cardiomyopathy (HOCM), arrhythmogenic right ventricular cardiomyopathy (ARVC), and left ventricular non-compaction (LVNC) constituted the GNCM group. Primary outcome was survival. Secondary outcomes included rejection, cardiac allograft vasculopathy (CAV), and graft failure. Results: Of the 49,417 transplant recipients identified, 997 recipients (mean age 36±20 years; 55% males; 79% Caucasian) had GNCM (HOCM n=836; ARVC n=83; LVNC n=78). Patients transplanted for GNCM had significantly higher 1, 5 and 10 year survival rates compared to those without GNCM (86%, 76%, 66% vs. 82%, 69%, 50%, respectively, log-rank p<0.001) (Figure 1A). After adjusting for age, sex, and race in multivariate Cox regression analysis; GNCM was associated with favorable post-transplant survival, with a hazard ratio of 0.70 (95% confidence interval 0.58-0.86; p=0.001). While the incidence of rejection was similar in GNCM compared to non-GNCM (43% vs. 40%, p=0.11), the incidences of CAV and graft failure were significantly lower compared to non-GNCM (24% vs 32%, p<0.001, and 9% vs 15%, p<0.001, respectively). The survival rates for HOCM, ARVC, and LVNC, were all similar to each other but significantly higher compared to non-GNCM (log-rank p<0.001) (Figure 1B). Conclusions: Patients with GNCM seem to have better post-transplant survival and graft outcomes than patients transplanted for other cardiomyopathies.