Outcomes of Branch Pulmonary Artery Stenosis in Williams Syndrome and Non-Williams Supravalvar Aortic Stenosis

Abstract

Background The cardiovascular findings noted in both Williams syndrome (WS) and non-Williams (NW) supravalvar aortic stenosis (SVAS) are most often mediated by abnormal or deficient elastin (ELN). Branch pulmonary artery stenosis (BPAS) is the next most common lesion after SVAS. The prevalence, interventions for, and outcomes of BPAS in WS and NW SVAS has not been well-described. Aim Describe and compare BPAS intervention and outcomes in patients with WS and NW SVAS. Subjects and Methods We included all patients at our institution with either WS or SVAS with negative testing for WS from 2003-2020.Patients with Alagille syndrome, Turner …