[Staged operation for supravalvular aortic stenosis syndrome associated with severe multiple peripheral pulmonary...

Successful surgical correction of supravalvular aortic stenosis (SAS) syndrome associated with severe peripheral pulmonary stenosis by a staged approach in a 6-year-old boy is reported. Right ventriculogram demonstrated bilateral severe peripheral pulmonary stenoses and cardiac catheterization revealed severe pressure gradient of 94 mmHg between the trunk of pulmonary artery and right and left peripheral pulmonary arteries, respectively. Bilateral severe peripheral pulmonary stenoses were relieved successfully by staged operations. Initially, a stenotic portion of the right pulmonary artery and its bifurcation was removed and a DeBakey Knitted Dacron graft was interposed between the right pulmonary artery and the peripheral pulmonary artery which was enlarged by arterioplasty. Nine days later, stenosis of the left peripheral pulmonary artery was relieved by arterioplasty using a Rygg patch. Finally, 8 months after the initial operation, the patient underwent definitive repair of SAS by extended aortoplasty. The postoperative course of the patient was uneventful. Our experience indicates that in the surgical treatment of the patient with SAS and associated severe multiple peripheral pulmonary stenoses, a staged approach, that is initial staged repair of the bilateral pulmonary stenoses by arterioplasty through thoracotomy followed by definitive intracardiac repair of SAS, is the procedure of choice.