TY - JOUR
T1 - Subvalvular aortic stenosis in adults
T2 - clinical course and long-term outcomes
AU - de Keijzer, Adine R.
AU - Meccanici, Frederike
AU - Keuning, Zoe A.
AU - Esser, Fee A. J.
AU - de Bruijn, Johannes W. C.
AU - Egorova, Anastasia D.
AU - Bouma, Berto J.
AU - van Kimmenade, Roland R. J.
AU - Veen, Kevin M.
AU - Kluin, Jolanda
AU - Voskuil, Michiel
AU - van Melle, Joost P.
AU - Roos-Hesselink, Jolien W.
AU - van den Bosch, Annemien E.
N1 - Publisher Copyright:
© The Author(s) 2026. Published by Oxford University Press on behalf of the European Society of Cardiology. This is an Open Access article distributed under the terms of the Creative Commons Attribution-NonCommercial License (https://creativecommons.org/licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact [email protected] for reprints and translation rights for reprints. All other permissions can be obtained through our RightsLink service via the Permissions link on the article page on our site—for further information please contact [email protected].
PY - 2026/8/1
Y1 - 2026/8/1
N2 - Background and Aims: This study investigates the clinical course and long-term outcomes of adults with subvalvular aortic stenosis (SAS). Methods: Adults with SAS, prospectively registered in the Dutch Congenital Cor Vitia (CONCOR) registry between 2001–2019, were included. All-cause mortality, SAS (re-) operation, and cardiovascular events, including arrhythmias, heart failure, (re-)operation for aortic regurgitation (AR), were assessed. Longitudinal changes in echocardiographic peak velocity, interventricular septal thickness (IVST), and left ventricular posterior wall thickness (LVPW) were analysed using linear mixed-effects models. Differences in the history of SAS repair (operated/unoperated patients), isolated/non-isolated SAS, and sex were explored. Results: Overall, 312 patients were included [age: 26.0 (interquartile range, IQR: 20.0–35.3) years, 68.3% history of SAS repair] with a median follow-up of 16 (IQR: 10–20) years (4423 patient-years). Unadjusted survival at 15 years was lower in the operated group compared to the unoperated group (P = .009) and no significant differences were observed between sexes (P = .083) or isolated/non-isolated SAS (P = .810). The cumulative incidence of (re-)operation for AR at 15 years was 7.6% (95% CI 4.7%–11.0%). The hazard of SAS repair during follow-up was higher in the unoperated group compared to the operated group [HR 0.2 (95% CI 0.1–0.5), P < .001], after correction for covariates. Peak velocity progression was 0.1 m/s (P = .357) during the first period and 0.3 m/s (P = .032) during the second (after ±10 years). No patient showed fast progression (≥0.3 m/s/year) in peak velocity. At baseline no evidence of left ventricular hypertrophy was observed, following IVST/LVPW criteria. Conclusions: Survival of adult SAS patients with a history of SAS repair was substantially lower compared to the unoperated group, reflecting a potentially more severe SAS phenotype. Nevertheless, long-term clinical event rates were considerable. SAS remained stable, suggesting less echocardiographic follow-up may suffice, particularly in mild phenotypes without AR. Additionally, follow-up should focus on the clinical sequelae of SAS.
AB - Background and Aims: This study investigates the clinical course and long-term outcomes of adults with subvalvular aortic stenosis (SAS). Methods: Adults with SAS, prospectively registered in the Dutch Congenital Cor Vitia (CONCOR) registry between 2001–2019, were included. All-cause mortality, SAS (re-) operation, and cardiovascular events, including arrhythmias, heart failure, (re-)operation for aortic regurgitation (AR), were assessed. Longitudinal changes in echocardiographic peak velocity, interventricular septal thickness (IVST), and left ventricular posterior wall thickness (LVPW) were analysed using linear mixed-effects models. Differences in the history of SAS repair (operated/unoperated patients), isolated/non-isolated SAS, and sex were explored. Results: Overall, 312 patients were included [age: 26.0 (interquartile range, IQR: 20.0–35.3) years, 68.3% history of SAS repair] with a median follow-up of 16 (IQR: 10–20) years (4423 patient-years). Unadjusted survival at 15 years was lower in the operated group compared to the unoperated group (P = .009) and no significant differences were observed between sexes (P = .083) or isolated/non-isolated SAS (P = .810). The cumulative incidence of (re-)operation for AR at 15 years was 7.6% (95% CI 4.7%–11.0%). The hazard of SAS repair during follow-up was higher in the unoperated group compared to the operated group [HR 0.2 (95% CI 0.1–0.5), P < .001], after correction for covariates. Peak velocity progression was 0.1 m/s (P = .357) during the first period and 0.3 m/s (P = .032) during the second (after ±10 years). No patient showed fast progression (≥0.3 m/s/year) in peak velocity. At baseline no evidence of left ventricular hypertrophy was observed, following IVST/LVPW criteria. Conclusions: Survival of adult SAS patients with a history of SAS repair was substantially lower compared to the unoperated group, reflecting a potentially more severe SAS phenotype. Nevertheless, long-term clinical event rates were considerable. SAS remained stable, suggesting less echocardiographic follow-up may suffice, particularly in mild phenotypes without AR. Additionally, follow-up should focus on the clinical sequelae of SAS.
KW - Adults
KW - Clinical course
KW - Clinical outcomes
KW - Male-female differences
KW - Subvalvular aortic stenosis
UR - https://www.scopus.com/pages/publications/105046423197
U2 - 10.1093/eurheartj/ehaf1078
DO - 10.1093/eurheartj/ehaf1078
M3 - Article
C2 - 41635281
SN - 0195-668X
VL - 47
SP - 3920
EP - 3934
JO - European heart journal
JF - European heart journal
IS - 29
ER -