Key result
Congenital aortic stenosis progresses slowly in adults, with peak velocity increasing ~0.06 m/s per year.
Why the study?
Congenital aortic stenosis is a heterogeneous disease, but repeated data describing disease progression is limited, particularly in adults.
Observational (n=402)
Yes
Overall disease progression in adult congenital aortic stenosis patients is slow and independent of baseline peak velocity, but older age and concentric LV remodeling are associated with faster progression.
Answers are grounded in this paper and cite their sources.
Supports tailored surveillance by age and LV remodeling in adult congenital AS; leaves open optimal intervals pending prospective validation.
Background: Congenital aortic stenosis (AS) is a heterogeneous disease. However, repeated data describing disease progression is limited, especially in adults. Therefore, the objective of this study is to investigate progression of peak velocity and aortic dimensions in adult congenital AS patients, identifying markers for fast progression. Methods: Adult patients (aged 18-55 years) with a native aortic valve and at least mild AS at baseline registered in the Dutch CONCOR registry between 2001 and 2019 from all six tertiary expert centers for congenital heart disease were included. Patients with severe aortic regurgitation at baseline or no available echocardiograms during follow-up were excluded. Data on ascending aortic dimensions and peak velocity changes over time were analyzed using mixed models until death, aortic valve replacement or March 1, 2023. Results: 402 patients (63 % male) were included with a median age of 26 [IQR 19-41] years and peak velocity of 3.1 [IQR 2.7-3.6] m/s. Median follow-up time was 6.8 [IQR 3.8-11.6] years. Peak velocity significantly progressed over time with 0.06 ± 0.10 m/s/year (p < 0.001), independent of baseline velocity. Older age and concentric left ventricular (LV) remodeling were associated with faster progression. Mean progression of ascending aortic dimension was 0.4 ± 0.5 mm/year (p < 0.001), with faster progression in younger patients (p = 0.002). No correlation between aortic growth rate and stenosis severity progression was seen (r = 0.001, p = 0.98). Conclusion: Overall disease progression was slow in adult congenital AS patients, and independent of baseline peak velocity. Progression of stenosis should be monitored more carefully in older patients and patients with signs of concentric LV remodeling.
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Keuning et al. (2025) conducted an observational in Congenital aortic stenosis (n=402). Congenital aortic stenosis was evaluated on Progression of aortic valve peak velocity (0.06 m/s/year, p=<0.001). In adult patients with congenital aortic stenosis, overall disease progression was slow, with aortic valve peak velocity increasing by 0.06 m/s/year and ascending aortic dimension by 0.4 mm/year.
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