Evaluation of Nine Cases with Supravalvular Aortic Stenosis

In this study, nine patients with supravalvular aortic stenosis (SVAS) diagnosed between 1994 and 1999 were reviewed, retrospectively. Six of the cases were male and three female. The age of the patients ranged between 1 and 15 years (mean 6.4 ± 5.3 years) when SVAS was diagnosed. During the diagnosis, mean age of the SVAS patients with Williams syndrome was 4.8 ± 3.6 years, however in non Williams-SVAS patients was 8.5±6.9 years. Five of the male patients had Williams syndrome. The other patients had normal phenotype. Two patients were cousins. On echocardiographic examination, the mean systolic pressure gradient of SVAS was measured as 95±45 mmHg (38-180 mmHg). In seven patients who underwent cardiac catheterization and angiography, the mean systolic pressure gradient was measured as 98±50 mmHg (35-200 mmHg). The type of supraaortic stenosis was an hourglass type in all patients. Five patients with SVAS were operared on . Cardiac catheterization was planned in two patients diagnosed echocardiographically. One patient died postoperatively at the early period. In one patient who underwent operation, systolic pressure gradient increased gradually postoperatively. In this patient reoperation was planned. SVAS is the least common of the three types of fixed aortic stenosis. In the patients with SVAS may be in three forms: Williams syndrome, autosomal dominant familial trait and sporadic cases. The patients with SVAS were evaluated as clinically, age of diagnosis and the reasons of delayed diagnosis.