Abstract
Supravalvular aortic stenosis, which is a rare congenital cardiac anomaly, is associated with several lesions and has a progressive nature. Herein, we report a five-year-old girl with bicuspid aorta who underwent initial Doty operation at the age of nine months. A combined redo Doty operation and an aortic valve commissurotomy were performed two years later. Due to the rapidly progressing aortic regurgitation and both valvular and supravalvular gradient, a repeated surgery was required at the age of five years and an aortic homograft was successfully inserted with an annulus enlargement and the patient was discharged uneventfully. In conclusion, although Doty repair yields satisfactory results in most patients, certain cases with identified risk factors may require reoperations due to the progressive nature of the disease. Therefore, these patients should be kept under a close follow-up lifelong.