ObjectiveTo explore the experience of surgical repair for congenital aortic stenosis (AS) in our center.MethodsWe retrospectively reviewed the clinical data of 145 children diagnosed with AS, who underwent aortic repair from 2008 to 2019, with or without aortic insufficiency (AI), including 104 males and 41 females with a median age of 2.9 (0.6, 7.8) years. The preoperative and intraoperative data, early and long-term valvular function, long-term survival rate and freedom from reoperation and aortic valve replacment (AVR) were analyzed.ResultsThere were 120 patients receiving commissurotomy, 15 valvuloplasty with extra pericardium patch, and 25 AVR. The mean follow-up time was 0.25-11.20 (4.2±2.8) years. Survival rate at 10 years was 92.3%. Freedom from reoperation and AVR was 63.2% and 60.4% respectively. Multivariate analysis revealed that using a patch during surgery (P=0.036) was an independent risk factor for reoperation. A history of preoperative balloon dilation (P=0.029) and significant preoperative AI (P=0.001) contributed to AVR.ConclusionSurgical treatment of congenital aortic valve stenosis in children is a safe and effective method that provides enough time to achieve a more definitive solution. Using a patch during surgery increases reoperation hazard. A history of previous balloon dilation and significant preoperative AI may result in AVR during the follow-up.