We report herein the results of a retrospective study conducted on ten consecutive Japanese patients who underwent successful surgical relief of fixed subaortic stenosis between 1972 and 1994 at ages ranging from 8 months to 21 years, and followed for 3.6 years and 26 years. Associated cardiovascular defects were present in six patients, two had a history of infective endocarditis, a discrete fibrous ring was found in nine patients, and a redundant abnormal sheet was found in one. A stenotic structure was removed in nine patients and incised in one, while myotomy was additionally performed in one. There were no early complications or deaths. Cardiac catheterization revealed a significant decrease in the peak systolic pressure gradient from 84+/-22 mm Hg preoperatively to 32+/-22 mm Hg postoperatively (P = 0.0017). Reoperation of an aortic valve replacement with or without valvular annulus enlargement was required in four patients because of a small annulus with aortic insufficiency or infective endocarditis. Infective endocarditis was a major cause of late mortality (n = 1) and morbidity (n = 1), but the remaining eight patients have been asymptomatic. Thus, although this lesion is relatively rare in Japan, the typical discrete type may be more common than previously believed. While a relief operation is associated with low early mortality, the palliative aspect regarding pathology of the aortic valve should not be underestimated, including poor growth of the valve annulus, deterioration of aortic insufficiency, and infective endocarditis. The most appropriate operative procedure for reoperation remains to be evolved.