Tratamiento quirúrgico de la estenosis aórtica supravalvular
- G. Aldamiz Echevarría
- P. Martínez Corrales
- J. Aramendi Gallardo
- A. Llorente Urcullu
- D. Rodrigo Carbonero
- A. Cabrera Duro
Year of publication: 2000
Volume: 52
Issue: 4
Pages: 309-313
Type: Article
Abstract
Surgical correction for 10 supravalvular aortic stenosissince 1988. Material and method Diagnosis was carried out by means of echocardiography and magnetic resonance. Seven patients showed features of Williams-Beuren syndrome, 5 patients showed in their genotype a delection of 7-chromosome. 5 showed membranous-localized type obstruction and the other 5 with hourglass type. The patients in the first group underwent circumferential resection of the stenosis ring followed by a diamond- shaped patch and the second group were treated with resection of the stenosing ring associated with an inverted Y- shaped patch with releasing of the coronary ostia in two of them. Results There was no hospital death. The gradient was substantially reduced from 60 ± 8 to 5 ± 1 mm Hg (range 0-14). The average staying was under 7 ± 1 days. The average follow- up was 58 ± 8 months (2-120). Conclusion Postoperatively, all the patients were in NYHA class III. The residual gradient was less than 15 mmHg in the echocardiography study during the follow-up and none of them required an additional operation.