Long-term Surgical Prognosis of Primary Supravalvular Aortic Stenosis Repair

Surgical repair of coronary artery ostial stenosis in patients with Williams and elastin arteriopathy syndromes

“…Other surgical groups have reported the presence of PPAS in 20% to 40% of patients with SVAS associated with Williams syndrome. 6,7 However, in the absence of surgical techniques for PPAS repair, this hemodynamic burden is left unaddressed and deferred to interventional catheterization techniques. It is certainly a matter of debate whether the right-sided lesions must always be addressed concomitantly or whether there are situations in which the physiologic tolerance and operative load might dictate "prudence" in deferring the PPAS repair.…”

“…It is not surprising that the presence of PPAS has been found to be a risk factor for postoperative mortality following SVAS repair. 6,7 There are 3 recent series that have been published on SVAS repair. In the Boston series, 22 of 87 patients underwent coronary artery procedures, with 9 patch augmentations of the coronary ostium.…”

“…In the Boston series, 22 of 87 patients underwent coronary artery procedures, with 9 patch augmentations of the coronary ostium. 6 Two of the 3 late deaths occurred in patients with coronary artery involvement. In the Toronto series, 18 27 patients of 53 were identified with coronary artery lesions.…”

“…5 A smaller subset of patients with SVAS will have more extensive arch involvement, including the transverse arch, isthmus, and proximal descending aorta. 6,7 Therefore, it is imperative to evaluate the entire aortic arch in patients with Williams and elastin arteriopathy.…”

Commentary: An opportunity to better characterize coronary ostial stenosis in patients with Williams syndrome and other elastin arteriopathies

“…This techniques and results are similar to those reported by a group from Boston Children's Hospital. 1,5 The indication for coronary artery intervention in this report are manifold: preoperative evidence of myocardial ischemia, preoperative imaging with evidence of obstruction, intraoperative evidence of ostial obstruction, and finally evidence of coronary artery insufficiency after surgical repair of supravalvular aortic or pulmonary stenosis. There is a clear opportunity here to help this complex patient population.…”