Acquired Supravalvular Aortic Stenosis: Review of a Rare Conundrum with Diverse Etiologies

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Background Supravalvular aortic stenosis is a rare form of left ventricular outflow tract obstruction. Classically, supravalvular aortic stenosis occurs secondary to elastin arteriopathy, in association with Williams–Beuren syndrome, or as non-syndromic supravalvular aortic stenosis. In contrast to congenital supravalvular aortic stenosis, acquired supravalvular aortic stenosis refers to supravalvar aortic obstruction resulting from local pathological processes. It is exceedingly rare and occurs secondary to diverse etiologies. Methods A comprehensive search was conducted using PubMed and Google Scholar (1950-2024) to identify relevant cases. We also include a case series of four patients with acquired supravalvular aortic stenosis, unrelated to elastin mutations, identified between 2020 and 2025. Results Diverse etiologies, including familial hypercholesterolemia, aortic dissection, post-surgical complications, tumor-metastasis-related obstruction, fungal aspergilloma, Takayasu arteritis, and others, have been described to cause acquired supravalvular aortic stenosis in the past. Familial hypercholesterolemia is the most frequently reported cause of acquired supravalvular aortic stenosis. We also describe our experience in managing four cases of acquired supravalvular aortic stenosis. Conclusions This series highlights the rarity and diverse etiologies of acquired supravalvular aortic stenosis, emphasizing the need for targeted echocardiographic assessment in at-risk patients.

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