TY - JOUR
T1 - Long-term clinical outcomes in adult congenital aortic stenosis
T2 - results from a national study
AU - Keuning, Zoë A
AU - Meccanici, Frederike
AU - Schreurs, Bibi A
AU - Veen, Kevin
AU - Bouma, Berto J
AU - Voskuil, Michiel
AU - Jongbloed, Monique R M
AU - van Melle, Joost P
AU - van Kimmenade, Roland R J
AU - Kapoor, Rowina
AU - Bozkurt, Irem
AU - Hirsch, Alexander
AU - Roos-Hesselink, Jolien W
AU - van den Bosch, Annemien E
N1 - Publisher Copyright:
© Author(s) (or their employer(s)) 2025. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ Group.
PY - 2025/8/27
Y1 - 2025/8/27
N2 - 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.
AB - 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.
U2 - 10.1136/openhrt-2025-003534
DO - 10.1136/openhrt-2025-003534
M3 - Article
C2 - 40866083
SN - 2053-3624
VL - 12
JO - Open Heart
JF - Open Heart
IS - 2
M1 - e003534
ER -