A Williams-Beuren Syndrome with Critical Supravalvular Aortic Stenosis

A 4.5-year-old boy with the typical clinical aspects of Williams-Beuren syndrome who had severe supravalvular aortic stenosis was reported. Cardiac catheterization revealed 320 mmHg left ventricular systolic pressure and 180 mmHg supravalvular gradient. The stenosis was enlarged successfully with an oval-shaped patch by cardiopulmonary bypass technique. He was followed up for six months without any complication.