Supravalvar Aortic Stenosis in Infancy

Abstract

Supravalvar aortic stenosis (SVAS) is a rare anomaly of the aortic root caused by a genetically based deficiency in elastin production. Concomitant primary and secondary cardiovascular lesions complicate surgical management and impact early and late outcomes. Because SVAS is a rare lesion, surgical series are relatively small and span lengthy time periods. Consequently, risk factors that influence early and late outcomes are not well defined. Patients who come to surgery during infancy are particularly challenging, but little attention has been directed as to whether or not young age influences outcomes. This review suggests that complicating associated features of elastin arteriopathy are more prevalent in patients who require relief of SVAS during infancy, and that concomitant lesions significantly increase the difficulty and risk of treating younger patients with SVAS.