[Supravalvular aortic stenosis. Apropos of 10 cases].
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.
Clerigue Arrieta
N
N
Cardiología Pediátrica, Hospital Infantil de Cruces, Vizcaya.
Cabrera Duro
A
A
Hernaez Ortega
E
E
Zubía Aguillo
A
A
Estébanez Ruiz
S
S
Mintegui Raso
S
S
Sánchez Echaniz
J
J
Velasco Vicente
J V
JV
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
Estenosis aórtica supravalvular. A propósito de 10 casos.