[Surgical treatment of supravalvular aortic stenosis]. / Tratamiento quirúrgico de la estenosis aórtica supravalvular.

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.

Tratamiento quirúrgico de la estenosis aórtica supravalvular / Supravalvular aortic stenosis surgical treatment

OBJETIVO: Corrección desde 1998 de 10 pacientes afectados de una estenosis aórtica supravalvular. MATERIAL Y MÉTODOS: El diagnóstico se realizó con ecocardiografía y resonancia magnética. Siete tenían un síndrome de Williams-Beuren, confirmándose en 5 una deleción del cromosoma 7. Cinco tenían obstrucción de tipo membranosa y otros 5 "en reloj de arena". Para el primer grupo se aplicó una resección y aortoplastia con parche en lágrima, y en el segundo grupo aortoplastia con parche en Y invertida con liberación de ostium coronario en 2 casos. RESULTADOS: No existió mortalidad en ningún grupo, reduciéndose el gradiente de 60 ñ 8 a 5 ñ 1 mmHg (rango 0-14). La estancia no fue superior a 7 ñ 1 días. El seguimiento medio fue de 58 ñ 8 meses (2-120). CONCLUSIÓN: Todos los pacientes se encuentran en grado funcional I-II de la NYHA. En los controles ecocardiográficos ningún paciente superó los 15 mmHg, no precisando reintervención (AU)

[Aortic valve stenosis. Surgical treatment in children]. / Estenosis aórtica valvular. Tratamiento quirúrgico en la infancia.

OBJECTIVES: The results of aortic commissurotomy, as the first step in the treatment of aortic valve stenosis in children, have been estimated after a medium-term follow-up. MATERIAL AND METHODS: Twenty-two patients were operated at a mean age of 7.3 +/- 3.6 years of age. Surgery was performed under cardiopulmonary bypass and moderate hypothermia. Twenty patients underwent cardiac catheterization (the systolic gradient pressure was 67.9 +/- 24.7 mmHg and the end diastolic pressure was 17 +/- 7.1 mmHg). We evaluated 10 patients with Doppler flow echocardiography (the valvular area was 0.8 +/- 0.2 cm2/m2). After surgery, we made a follow-up of 8.6 +/- 5.4 years. RESULTS: The aortic valve was bicuspid in 13 cases and tricuspid in 9 cases. There was no mortality. Before the age of 7, 7 patients developed restenosis. The mean pressure gradient was 92.5 +/- 16.6 mmHg, so six of them underwent valvuloplasty and the other one was operated with an aortic valvulated homograft. The pressure gradient, which was estimated in 20 patients, was lowered to 43.5 +/- 17.5 mmHg and the aortic area was increased to 2.4 cm2/m2. Six patients had a pressure gradient above 40 mmHg. Six patients had previous aortic regurgitation and this reappeared in 3 patients after valvulotomy. At this time, 12 patients have aortic regurgitation, being mild in 9 patients, mild to moderate in 1 and moderate in 2 patients. CONCLUSION: Valvulotomy is a palliative therapeutic method. We had no mortality. Restenosis appeared in 7 patients, before the age of seven years, and it can be easily corrected. The aortic regurgitation is usually mild. Valve replacement can be avoided in childhood.