Heart failure and re-interventions in adult patients with residual right-sided congenital heart disease.

BACKGROUND

Heart failure (HF) is the leading cause of death among adults with congenital heart disease. However, specific data on right-sided adult congenital heart disease (RACHD) remain scarce. This study assessed (1) the prevalence and risk factors of HF; (2) its prognostic impact; and (3) the impact of re-intervention in adults with right-sided residual congenital heart disease and HF (RACHD-HF).

METHODS

Data from RACHD patients since 2010 were retrospectively analysed. HF was defined as symptoms requiring therapy with objective cardiac dysfunction. Cox models identified predictors of the primary composite outcome (death, ventricular assist device [VAD], or heart transplant), with Kaplan-Meier curves for event-free survival. (S61354).

RESULTS

980 RACHD patients (mean age 39.8 ± 12.8 years) were evaluated. HF was diagnosed in 73 patients (7.4%) and was associated with older age, arrhythmias, prior endocarditis, coronary artery disease, and electrophysiological interventions. Double outlet right ventricle and Ebstein's anomaly were more common in the HF group. Among HF patients, 34% underwent re-intervention, which was associated with lower right ventricular systolic pressure (p = 0.034) but did not alter medical therapy. Over a median 3.9-year follow-up (IQR 3.4-4.9), 49 (5.0%) reached the composite outcome. Event-free survival was 99.1% and 98.3% at 1 and 3 years, respectively, in non-HF patients compared to 77.8% and 62.3% in HF patients (p < 0.001). Re-intervention was not associated with higher survival.

CONCLUSIONS

HF represents a significant prognostic marker for death, VAD, or transplant in RACHD, underscoring the need for enhanced surveillance and optimised treatment. Re-intervention offers structural benefit without fully preventing adverse outcomes.