Outcomes Following Heart Transplantation in Adults with Congenital Heart Disease

Abstract

<h3>Purpose</h3> The number of adults with congenital heart disease (ACHD) requiring treatment with heart transplant (HT) is growing. Determinants for survival after HT in this challenging population remain unknown. The aim of this study is to investigate the factors associated with outcomes after HT in ACHD. <h3>Methods</h3> We reviewed the UNOS-STAR database and analyzed 35,952 HT from 2000 to 2018. Recipient and donor characteristics, outcomes for patients undergoing HT for ischemic cardiomyopathy (ICM) (n=14,236), nonischemic cardiomyopathy (NICM) (n=20,676), and ACHD (n=1,040) were compared. The mean follow-up for the total study population was 6.20±4.84 years. Kaplan-Meier survival curves with Log-rank statistics as well as Cox-proportional hazards analysis with univariate analysis as well as multivariable regression were used to analyze survival data. Univariable and multivariable forward and reverse binary logistic regression was used to evaluate determinants of hospital mortality. <h3>Results</h3> When compared to the other groups, ACHD recipients had a higher incidence of postoperative stroke, dialysis, and death from acute rejection. Multivariable analysis confirmed that ACHD was associated greater in-hospital death compared to ICM (HR=0.54, P<0.001) and NICM (HR=0.46, P<0.001). Factors associated with increased mortality were history of cerebrovascular disease (HR=1.11, P=0.026), prior history of malignancy (HR=1.12, P=0.006), pre-transplant biventricular support (HR=1.12, P=0.069), postoperative stroke (HR=1.47, P<0.001) and postoperative dialysis (HR=1.71, P<0.001). O blood group donor hearts were also associated with increased in-hospital death compared to blood groups A (HR=0.824, P=0.001), B (HR=0.818, P=0.029), and AB (HR=0.659, P=0.051). In-hospital deaths were less likely with donors who were male (HR=0.851, P=0.011) and with a history of cocaine use (HR=0.826, P=0.012). ACHD patients more likely to receive a subsequent re-transplant. The 5-, 10-, and 15-year survival were 72.3%, 63.4%, and 53.0% for ACHD; 75.8%, 56.1%, and 35.6% for ICM and 79.2%, 64.9%, and 49.0% for NICM. Survival, conditional upon discharge from the hospital was significantly higher in ACHD. <h3>Conclusion</h3> HT in ACHD is associated with a higher early post-operative mortality, better long-term conditional survival, and a higher rate of retransplantation when compared to patients with ICM and NICM.