The First Case of Successful Stenting of the Dissection of the Ascending Aorta and the Aortic Arch that Occurred During Surgical Correction of the Supralvular Aortic Stenosis in a Child with the Williams Syndrome

Abstract

Congenital supravalvular aortic stenosis (SVAS), in vast majority of cases is a manifestation of Williams syndrome. Surgical correction of this pathology is a "gold standard" for treatment in these patients. One of the most dangerouse potential complications in surgical repair of this disease is acute dissection of the ascending aorta, arising in 0.7% of cases. This complication can be attributed to both surgical errors and or due to the anatomical features of the aortic wall. We report on a pediatric patient with Williams syndrome, 2.3 yr, 11.9 kg, who underwent surgical repair for supravalvar aortic stenosis using the Doty technique. Aortic dissection was diagnosed using aortography. This was managed by implanting two Valeo stents.