Patch aortoplasty for supravalvular aortic stenosis with bicuspid aortic valve associated with Williams syndrome

We experienced a case in which a 7-year-old boy with a characteristic elfin face, heart murmur, and mental retardation, underwent extended patch aortoplasty using diamond shaped dacron patch for congenital supravalvular aortic stenosis. The aortography performed before operation demonstrated diffuse stenosis just above the aortic valve, which was a typical hourglass type. The preoperative peak systolic pressure gradient between the left ventricle and ascending aorta was 86 mmHg, and was improved postoperatively. In this procedure no cusp was incised, resulting in no deformity of the aortic valve and no obstruction of coronary arteries. In conclusion this method was excellent for the diffuse type of supravalvular aortic stenosis.