Congenital aortic stenosis in adults: Rate of progression and predictors of clinical outcome

Background Little data are available on the natural history of young adults with congenital valvular aortic stenosis (AS). The aim of the present study was to determine the progression rate of AS in young adults, and to identify predictors of stenosis progression and outcome. Methods Retrospective study of all patients seen at a single centre diagnosed with congenital AS (≥ 2.5 m/s) between 1992 and 2005, excluding patients with severe aortic regurgitation. The slope of the regression of the aortic jet velocity on the time elapsed since the baseline study was used to define the rate of progression of stenosis. Results A total of 84 adults (mean age, 23.5 ± 7.9 years) were studied who had at least two echocardiograms > 1 year (5.6 ± 2.6 years) apart. The annual progression of aortic jet velocity was 0.09 ± 0.15 m/s per year. Multivariable linear regression analysis identified older age (p < 0.001) as an independent predictor of faster haemodynamic progression. During the follow-up period of 7.7 ± 2.7 years, no patient died and 35 patients (42%) underwent aortic valve intervention. By multivariable Cox regression analysis, severe AS (≥ 4.0 m/s) and rapid progression of aortic jet velocity (≥ 0.2 m/s/year) were independent predictors of intervention. Cumulative intervention-free survival for patients with severe AS was 78 ± 8% at 3 years and 48 ± 10% at 5 years versus respectively 98 ± 2% and 96 ± 3% for patients with mild-to-moderate AS (log-rank: p < 0.001). Conclusions Progression of congenital AS was relatively low in young adults compared to elderly with degenerative AS. Older age was associated with more rapid progression.