title: [Supravalvular aortic stenosis. Apropos of 10 cases].
abstract: We present 10 patients diagnosed of supravalvular aortic stenosis (5 dumb bell shaped, 4 membranous and 1 undifferentiated), 7 in association with Williams-Beuren syndrome, 5 were male and 5 were female. None showed alterations of calcium metabolism and in 8 cases we found other associated cardiac anomalies. In 9, the diagnosis was confirmed with Eco-Doppler. The gradient calculated with the Eco-Doppler had a medium correlation (75%) with the catheterism (Doppler 46 +/- 23 (13-89)-Catheterism 27 +/- 15 (10-50)). 50% had the sinus of Valsalva dilated. Six patients underwent surgery, 5 with an enlargement parch and 1 with a butterfly parch. In 3 cases, associated anomalies were repaired, 2 during supravalvular surgery (one valvular aortic stenosis and one fixed subaortic stenosis). No patient had postoperatory complications.
authors.affiliation: Cardiología Pediátrica, Hospital Infantil de Cruces, Vizcaya.
authors.forename: N
authors.initials: N
authors.lastname: Clerigue Arrieta
authors.forename: A
authors.initials: A
authors.lastname: Cabrera Duro
authors.forename: E
authors.initials: E
authors.lastname: Hernaez Ortega
authors.forename: A
authors.initials: A
authors.lastname: Zubía Aguillo
authors.forename: S
authors.initials: S
authors.lastname: Estébanez Ruiz
authors.forename: S
authors.initials: S
authors.lastname: Mintegui Raso
authors.forename: J
authors.initials: J
authors.lastname: Sánchez Echaniz
authors.forename: J V
authors.initials: JV
authors.lastname: Velasco Vicente
mesh_terms: D001024:Aortic Valve Stenosis / Q000150:complications / Q000000981:diagnostic imaging*; D002648:Child; D015150:Echocardiography, Doppler; D006330:Heart Defects, Congenital / Q000150:complications / Q000000981:diagnostic imaging*; D006801:Humans; D008297:Male; D013577:Syndrome
vernacular_title: Estenosis aórtica supravalvular. A propósito de 10 casos.