Anales espanoles de pediatria · 2000
[Surgical treatment of supravalvular aortic stenosis].
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Aldamiz Echevarría G, Martínez Corrales P, Aramendi Gallardo J, Llorente Urcullu A, Rodrigo Carbonero D, Cabrera Duro A
Аннотация
OBJECTIVE: Surgical correction for 10 supravalvular aortic stenosis since 1988.
MATERIALS AND METHODS: 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).
CONCLUSIONS: Postoperatively, all the patients were in NYHA class I-II. The residual gradient was less than 15 mmHg in the echocardiography study during the follow-up and none of them required an additional operation.
MATERIALS AND METHODS: 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).
CONCLUSIONS: Postoperatively, all the patients were in NYHA class I-II. The residual gradient was less than 15 mmHg in the echocardiography study during the follow-up and none of them required an additional operation.