Long-term outcomes of primary aortic valve repair for isolated congenital aortic stenosis in children.

Objective: We aimed to assess the long-term outcomes of children with isolated congenital aortic stenosis who underwent primary aortic valve repair.
Methods: Records of all children (n = 111) with isolated congenital aortic stenosis who underwent primary aortic valve repair between 1980 and 2016 were reviewed. An optimal operative outcome consisted of a residual left ventricular outflow tract peak systolic gradient <35 mm Hg and trivial or less aortic insufficiency.
Results: Median age at surgery was 0.4 years (interquartile range, 1 month-7.9 years) and median weight at surgery was 7.0 kg (interquartile range, 3.7-25.0 kg). Fifty-two patients (46.8%; 52/111) underwent aortic valve repair with the use of patch material. Early mortality was 0.9% (1/111). Late mortality was 0.9% (1/110). Freedom from aortic valve reoperation was 52.1% (95% CI, 38.7-63.8) at 10 years. Freedom from aortic valve replacement was 67.9% (95% CI, 55.4-77.5) at 10 years. An optimal outcome was achieved in 48 patients (43.2%; 48/111). At 10 years, freedom from aortic valve reoperation was 78.2% (95% CI, 63.1-87.8) in patients with an optimal outcome, compared with 39.4% (95% CI, 22.8-55.6) in those with a suboptimal outcome (P = .03). Tricuspid aortic valve was associated with a suboptimal outcome (P = .01).
Conclusions: Aortic valve repair achieves relief of congenital aortic stenosis with very low early mortality and excellent long-term survival, even in neonates. Although nearly half of the patients required aortic valve reoperation by 10 years, two-thirds of the patients remain free from aortic valve replacement. An optimal outcome was more commonly achieved with bicuspid aortic valves compared with tricuspid aortic valves.
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