Eighty-three patients aged 11 months to 25 years were followed up a median of 6.1 years (range 8 days to 24 years) after diagnosis of fixed subaortic stenosis (SAS). Fourteen (17%) had significant noncardiac defects and 47 (57%) had additional cardiac malformations. The left ventricular (LV) outflow gradient increased in 25 of 26 patients catheterized more than once before surgery. Of 15 patients less than 12 years old with gradients less than or equal to 40 mm Hg, 10 ultimately underwent operation after developing severe obstruction; another has progressed to a gradient of 45 mm Hg at 6 years of age. Before surgery (at a median age of 12 years), 55% had aortic regurgitation (AR), which was usually mild. Infective endocarditis occurred in 12% of the group, with a frequency of 14.3 cases per 1,000 patient-years. Seventy-four patients were operated on, with 6 early (8%) and 7 late (9%) deaths. Twelve underwent reoperation to relieve residual obstruction. Surgery reduced gradients in patients with discrete SAS from 83 +/- 33 to 29 +/- 30 mm Hg, but in 6 patients with tunnel SAS the reduction was less satisfactory. AR was absent or mild in most patients postoperatively. When the gradient was reduced to less than 80 mm Hg, infective endocarditis did not occur unless there were other residual lesions. These data suggest that it is reasonable to resect discrete SAS in children less than 10 to 12 years old with LV outflow gradients greater than or equal to 30 mm Hg.