Abstract

Background: The prevalence of adult congenital heart disease (ACHD) patients with heart failure is increasing. No guidelines exist for evaluation and listing for heart transplant (HT) in ACHD. We sought to review the outcomes of HT committee decisions [accepted and transplanted (T), accepted awaiting transplant (A), deferred (D), ineligible (I)]. Methods: This was a retrospective analysis performed on ACHD (≥ 18 yrs) referred for HT between 2000-2014. Data on demographics, CHD severity, NYHA class, imaging, exercise testing, catheterization and HT committee decision were recorded. Primary outcome was all-cause mortality. Analysis was completed to identify variables that were statistically different amongst groups. Results: Sixty-two ACHD were included in analysis, A (n= 17) I (n=14) T (n=22) D (n=9), NYHA class III-IV (98%), CHD of great complexity (74%). Median time to HT, after listing, was 3 mo. (mean 5.5). Survival was significantly lower in the group awaiting transplant compared to all other groups (p<0.001): A (18.6%) I (65.3%) T (78.3%) D (100%) ( Fig ). Among those who had not been transplanted, several variables were significantly different (p<0.05) including: systolic BP, alb, BNP, Hct, right atrial pressure and peak oxygen consumption. For those awaiting transplant with pulmonary hypertension (PH) mortality was 100% but 40% for those without PH (p<0.1). Conclusion: Waitlist mortality in the ACHD population is high. Given poor outcomes while on the waitlist, earlier listing may improve survival. Specific variables were statically different among patients not transplanted and there was a high mortality in those with PH. These may have contributed to the difference in survival among groups and may impact HT committees in the evaluation and listing for HT.

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