SURGICAL REPAIR FOR SUPRAVALVULAR AORTIC-STENOSIS - INTERMEDIATE TO LONG-TERM FOLLOW-UP

Background and aim of the study: Supravalvular aortic stenosis (SAS) h as been surgically treated by either single-sinus or bisinus patch rep air. This article reviews intermediate to long-term results of repair in patients with SAS at our institute. Methods: Between 1974 and 1995, eight patients underwent surgical repair for SAS. The age range was 1 .3 to 14 years (mean 7.3 years). The morphology was the discrete type in seven patients and the diffuse type in one patient. The pressure gr adient across the obstruction was 20 to 105 mmHg (mean 68 mmHg). Two p atients in the early period of the series underwent single-sinus patch repair, and six underwent bisinus patch repair (pantaloon-shaped patc h, extended aortoplasty). Results: There were no operative deaths, and the mean pressure gradient was reduced to 23 mmHg (p < 0.001) in the early postoperative period. The median follow-up period was 8.0 years (1.8-17.3 years). One patient with diffuse type SAS required reoperati on 12.8 years later due to progression of the stenosis, and died after the operation. The remaining seven patients were alive and well at th e last follow-up, when the pressure gradient ranged from 6 to 31 mmHg (reoperation excluded). Conclusion: Surgical repair for discrete type SAS provides satisfactory results in the intermediate to long-term fol low-up.