Tratamiento quirúrgico de la estenosis aórtica supravalvular

  1. G. Aldamiz Echevarría
  2. P. Martínez Corrales
  3. J. Aramendi Gallardo
  4. A. Llorente Urcullu
  5. D. Rodrigo Carbonero
  6. A. Cabrera Duro
Journal:
Anales de Pediatría: Publicación Oficial de la Asociación Española de Pediatría ( AEP )

Year of publication: 2000

Volume: 52

Issue: 4

Pages: 309-313

Type: Article

DOI: 10.1016/S1695-4033(00)77349-8 DIALNET GOOGLE SCHOLAR lock_openOpen access editor

Sustainable development goals

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.