Abstract
AIMS: While heart failure (HF) is a leading cause of death in adults with congenital heart disease (ACHD), few studies report contemporary outcomes after the first HF hospitalization. We examined outcomes of ACHD patients newly admitted for HF compared with ACHD patients without HF and the general HF population without ACHD. METHODS AND RESULTS: Using population databases from a single-payer health system from 1994 to 2018, ACHD patients newly admitted for HF were matched 1:1 to ACHD patients without HF (n = 4030 matched pairs). Similarly, ACHD patients newly admitted for HF were matched 1:1 to HF patients without ACHD (n = 4336 matched pairs). Patients with ACHD and HF (median age 68 years, 45% women) experienced higher mortality in short-term [30 day adjusted hazard ratio (HR) 4.68, 95% confidence interval (CI) 4.06, 5.43, P < 0.001], near-term (1 year HR 3.87, 95% CI 3.77, 4.92, P < 0.001), and long-term (24 year HR 1.59, 95% CI 1.13, 2.36, P = 0.008) follow-up. Patients with ACHD and HF had fewer baseline cardiovascular comorbidities than non-ACHD HF but demonstrated higher 30 day (HR 1.56, 95% CI 1.41, 1.73, P < 0.001), 1 year (HR 1.30, 95% CI 1.20, 1.40, P < 0.001), and 24 year (HR 2.40, 95% CI 1.73, 3.38, P < 0.001) mortality. Those with ACHD and HF also exhibited higher cardiovascular readmission rates at 30 days with HRs 9.15 (95% CI; 8.00, 10.48, P < 0.001) vs. ACHD without HF, and 1.71 (95% CI; 1.54, 1.85, P < 0.001) vs. HF without ACHD, and the higher readmission risk extended to 10 year follow-up. CONCLUSIONS: Adults with congenital heart disease patients with new HF have high risks of death and cardiovascular hospitalization, and preventative strategies to improve outcomes are urgently needed.