Congenital heart disease
Fixed subaortic stenosis in the young: Medical and surgical course in 83 patients

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Abstract

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 < 12 years old with gradients < 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 patientyears. 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 < 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 < 10 to 12 years old with LV outflow gradients ⪢- 30 mm Hg.

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    This study was supported by Training Grant 5 T32 H20 7 193 02 from the U.S. Public Health Service, Bethesda, Maryland.

    This work was done in part during Dr. Wright's tenure as a research fellow of the American Heart Association, Southeast Massachusetts Division (#73-414-801), Boston, Massachusetts.

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