The operative outcome in children with supravalvular aortic stenosis.

The authors present their experience in surgical treatment of children with supravalvular aortic stenosis (SAS). A retrospective analysis was carried out on 20 patients operated in the years 1980-1999. The group included 12 boys and 8 girls aged 0.9 to 14.5 years (mean 7.8 +/- 3.4 years). The diagnosis of Williams syndrome in 6 children (30%) was based on the typical face. The mean systolic pressure gradient across the SAS was 84.3 +/- 34.5 mmHg (range from 41 to 168 mmHg). Intraoperative studies allowed to determine that 16 (80%) children had the hourglass type, 2 (10%) tubular type and 2 (10%)--fibromuscular membrane type of stenosis. Sixteen (80%) children had anomalies of the aortic valve and/or coronary arteries, and in 13 (56%) children associated heart or great vessels anomalies were noted. All the patients were operated on using cardiopulmonary bypass and hypothermia. The associated anomalies were corrected simultaneously. There were no early and late deaths. Because of restenosis one child was reoperated. In the follow up the mean systolic pressure gradient between the left ventricle and ascending aorta revealed by echocardiography in 19 (95%) children to be 8 +/- 2 mmHg (range from 0 to 28 mmHg). Eighteen children are now in NYHA class I (New York Heart Association) and develop normally. Supravalvular aortic stenosis is a rare complex anomaly, which, because of life-threatening complications and low operative risk, should be fully repaired as soon as possible.