%0 Journal Article
%T Williams Syndrome: Supravalvar Aortic, Aortic Arch, Coronary and Pulmonary Arteries: Is Comprehensive Repair Advisable and Achievable?
%A Ma M
%A Martin E
%A Algaze C
%A Collins RT
%A McElhinney D
%A Mainwaring R
%A Hanley F
%J Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu
%V 26
%N 0
%D 2023
%M 36842795
暂无%R 10.1053/j.pcsu.2022.12.003
%X Williams syndrome, and various elastin protein mediated arteriopathies, presents a clinical challenge to pediatric cardiovascular specialists. In the severest phenotypes, multilevel obstruction to the systemic and pulmonic arterial systems result in biventricular dysfunction which can be imminently life-threatening. As a longstanding, quaternary referral center for complex pulmonary arteriopathies and pediatric connective tissue disease, Stanford Medicine Children's Health has developed a sizeable experience managing these patients. This manuscript is a summary of our current strategies, with a focus on our surgical techniques, peri-procedural considerations on timing and staging of various interventions, and long-term results.