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Discrete subaortic stenosis in adults

1978; Elsevier BV; Volume: 42; Issue: 2 Linguagem: Inglês

10.1016/0002-9149(78)90912-8

1879-1913

Chung-Shin Sung, Everett C. Price, Denton A. Cooley,

Congenital Heart Disease Studies

Discrete subaortic stenosis tends to be a progressive, severe defect that occurs most often in children. Of 138 patients who underwent operation for this defect at the Texas Heart Institute and affiliated hospitals from 1956 to 1976, only 36 patients (26 percent) were adults. The clinical, hemodynamic and surgical findings and follow-up data of these 36 patients, aged 18 to 65 years, are reviewed. The high incidence rate of associated cardiac defects and bacterial endocarditis is emphasized. Follow-up information, available for 22 of the original 36 patients, revealed three perioperative and two late deaths. One patient died 6 months after operation of unknown cause, and another died 4 years later of myocardial infarction. Fifteen of the 17 known survivors are asymptomatic or in improved condition. The symptoms and electrocardiographic findings of discrete subaortic stenosis are similar to those of aortic valve stenosis. The presence of an aortic ejection click or calcification of the valve on chest roentgenography or fluoroscopy indicates a diagnosis of valve stenosis. The usual criteria for diagnosis, localization of the gradient below the aortic valve at cardlac catheterizatlon or anglographic demonstration of discrete subaortic stenosis, are not always evident. Echocardiography may aid in determining the diagnosis by demonstrating early systolic closure of the aortic valve leaflets. An acceptable low surgical mortality rate can be achieved; however, some patients require aortic valve replacement or multiple operations for adequate repair of their defect. Patients with a thin membrane generally have a more favorable result than those with a flbro-muscular collar or secondary muscular hypertrophy. Aortic regurgitation is not always predictable and may recur or progress postoperatively.