[Surgical treatment of diffuse supravalvular aortic stenosis with Williams syndrome]

A five-year-old boy was admitted to our hospital because of a cardiac murmur and an abnormal electrocardiogram. He had a distinct pattern of facial features and mild mental and developmental retardation. An aortogram revealed that the aorta was hypoplastic from just above the Valsalva sinuses to the aortic arch. Moreover, the basal portions of the arch vessels were also hypoplastic. A diagnosis of diffuse supravalvular aortic stenosis was made. The pressure gradient between the left ventricle and the descending aorta was 74 mmHg on catheter examination. Surgical therapy was therefore indicated. The hypoplastic lesions of the aorta and arch vessels were enlarged with a Dacron patch under cardiopulmonary bypass and deep hypothermic circulatory arrest. The postoperative course was uneventful. The pressure gradient decreased to 7 mmHg on catheter examination. This type of supravalvular aortic stenosis is quite rare. Further follow-up is required to evaluate long-term outcome.