Sinus-sparing aortoplasty for diffuse nonsyndromic supravalvular aortic stenosis with arch-branch involvement

Diffuse supravalvular aortic stenosis (SVAS) is rare, especially in nonsyndromic patients and becomes more complex when the aortic arch branches are involved. Surgical planning must consider sinus dimensions to minimize the postoperative complications. We describe a 7-year-old boy without Williams-Beuren syndrome who was found to have diffuse SVAS extending from the sinotubular junction to the aortic isthmus, with additional narrowing of the brachiocephalic and left common carotid arteries. Because the aortic sinuses were normal in size, a sinus-sparing aortoplasty with patch augmentation of the stenotic aortic and arch-branch segments was performed. Sinus-sparing aortoplasty is a valuable surgical option in diffuse SVAS when the aortic sinuses are of normal size, allowing the effective relief of obstruction while minimizing the risk of postoperative aortic regurgitation and coronary ischemia.