Case report

Successful repair of supravalvular aortic stenosis in patient with Williams – Beuren syndrome

B.M. Todurov

Heart Institute Healthcare Ministry of Ukraine, Kyiv, Ukraine

This article describes a case of female patient with genetic Williams – Beuren syndrome who underwent successful correction of rare congenital heart disease – supravalvular aortic stenosis. Local narrowing of aorta of «sand clock» type at the level of sinotubular connection with pressure gradient 100 mm Hg and moderate local stenoses of the pulmonary artery have been detected in 37-year old patient. Aortoplasty with autopericardium technique described by McGoon was performed with good immediate outcome. This is a method of choice in all cases of this pathology.

Кey words: supravalvular aortic stenosis, Williams – Beuren syndrome, autopericardium.