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1 April 2000 Tratamiento quirúrgico de la estenosis aórtica supravalvular

Anales de Pediatría (English Edition)

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Vol. 52. Issue 4.

Pages 309-313 (1 April 2000)

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Vol. 52. Issue 4.

Pages 309-313 (1 April 2000)

DOI: 10.1016/S1695-4033(00)77349-8

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Tratamiento quirúrgico de la estenosis aórtica supravalvular

Supravalvular aortic stenosis surgical treatment

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G. Aldamiz Echevarríaa, P. Martínez Corralesa, J. Aramendi Gallardoa, A. Llorente Urcullua, D. Rodrigo Carbonerob, A. Cabrera Durob,*

a Servicios de Cirugía Cardíaca

b Servicios de Cardiología Pediátrica. Hospital Infantil Cruces. Baracaldo. Vizcaya

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Objetivos

Corrección desde 1998 de 10 pacientes afectados de una estenosis aórtica supravalvular.

Material y método

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 ± 8 a 5 ± 1 mmHg (rango 0 14). La estancia no fue superior a 7 ± 1 días. El seguimiento medio fue de 58 ± 8 meses (2-120).

Conclusiones

Todos los pacientes se encuentran en grado functional I-II de la NYHA. En los controles ecocardiográficos ningún paciente superó los 15 mmHg, no precisando reintervención.

Palabras clave:

Estenosis aórtica supravalvular

Resonancia magnética

Cirugía

Objectives

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.

Key words:

Supravalvular aortic stenosis

Magnetic Resonance Imaging

Surgery

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