Long-term clinical outcomes in adult congenital aortic stenosis: results from a national study.

Abstract

Background: Previous research on congenital aortic stenosis (AS) mainly focused on children, while data on long-term outcomes in adults is scarce. Therefore, this study aims to evaluate outcomes in adult patients with congenital AS and identify prognostic markers for aortic valve replacement (AVR).

Methods: In this multicentre study, patients aged 18-55 years with congenital AS (peak velocity ≥2.5 m/s) registered in the Dutch CONCOR (Congential Cor Vitia) registry from expert centres for congenital heart disease between 2001 and 2019 were included. Exclusion criteria were severe aortic regurgitation (AR) or prior AVR. Associations with the composite endpoint of all-cause mortality and AVR were assessed with multivariable Cox regression.

Results: Of the 427 included patients (median age 26 years, 62.5% male) median aortic peak velocity was 3.1 (IQR 2.7-3.6) m/s, 35% had moderate AR and 29% showed signs of left ventricular (LV) remodelling. During a median follow-up period of 7.9 (IQR 4.1-12.6) years, 7 patients died (1.6%) and 177 patients underwent AVR (41.5%). LV systolic dysfunction was the primary indication for AVR in three patients (1.7%). Peak velocity at baseline (HR 3.17, 95% CI 2.29 to 4.39), non-sinus rhythm (HR 3.12, 95% CI 1.62 to 6.02) and concentric LV geometry (HR 1.64, 95% CI 1.04 to 2.58) were associated with the primary endpoint beside age. Significant male-female differences were observed in prognostic factors for the primary endpoint.

Conclusion: AVR was often indicated in adult patients with congenital AS, even if the stenosis at baseline was mild. Moreover, monitoring LV remodelling alongside severity of AS is more important than focusing on systolic LV dysfunction.