Surgical aortic valvotomy has a long history of providing excellent palliation for aortic stenosis in infancy and childhood. The fate of aortic valve repairs for dominant aortic regurgitation in this same age group is considerably less clear.From 1990 to 2000, a total of 21 patients underwent aortic valve repair for aortic regurgitation at our institution. Seventeen patients were younger than 17 years at the time of repair (3-17 years, mean 8.1 +/- 3.7 years). Of these 17 children, 6 (35%) had bicuspid valves and 11 (65%) had tricuspid valves. Type of repair varied with valve type, but repair generally consisted of commissure resuspension, partial commissure closure, triangular resection of redundant leaflets, or some combination.There were no deaths. Follow-up ranged from 1 to 11 years (mean 5.3 +/- 2.4 years). At present 3 of 17 (17.6%) have mild aortic regurgitation according to echocardiography and 6 (35.2%) have moderate aortic regurgitation. In 8 of 17 cases (47.1%) the repair clearly failed, requiring reoperation from 0.5 to 73 months after the original operation (mean 18.9 months). Reoperation consisted of 6 Ross procedures and 2 mechanical aortic valve replacements. There were no deaths at the secondary operation.Aortic valve repair in children with a dominant feature of aortic insufficiency tended to fail progressively and at a high rate. Leaflet thickening was associated with higher risk of repair failure in this series. The threshold for aortic valve replacement should remain low.