Modified Simple Sliding Aortoplasty for Supra Valvular Aortic Stenosis in Two Siblings Without William’s Syndrome

Abstract

Two male siblings (13 and 11 years old) without Williamʼs syndrome were referred for surgical enlargement for severe supra valvular aortic stenosis (SVAS). The stenosis was most prominent at the sinotubular junction (STJ) and severe aortic wall thickness with elastic hard spread widely from the STJ into the ascending aorta in both cases. Since both coronary artery orifices located next to the STJ and the commissure between the two orifices, the non-coronary sinus of Valsalva was the only to be excised. To avoid use of artificial maerial, modified simple sliding aortoplasty was performed. The procedure consisted of transection of the ascending aorta at the stenotic STJ, a vertical incision made into the non-coronary sinus of Valsalva perpendicular to aortic valve annulus, another vertical incision made into the minor curvature of the distal stamp of the ascending aorta, and direct approximation of the two aortic ends. After this simple repair, the pressure gradient across the ST was significantly reduced in both cases. The two boys returned to daily life without any restrictions. Long term follow-up is necessary to warrant benefits of this procedure.