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SVAS outside of WS may also occur sporadically or in a familial form, often with identifiable mutations in the elastin (<jats:italic>ELN<\/jats:italic>) gene. While risk of sudden cardiac death in patients with SVAS has been extensively described in the context of WS, less is known about risk in patients with isolated SVAS. We report a case of a nonsyndromic two-year-old boy with evolving manifestations of SVAS who developed sudden cardiac arrest and death during a sedated cardiac magnetic resonance imaging study. A strong family history of SVAS was present and targeted genetic testing identified an <jats:italic>ELN<\/jats:italic> gene mutation in the boy\u2019s affected father and other paternal relatives. We review risk factors found in the literature for SCA in SVAS patients and utilize this case to raise awareness of the risk of cardiac events in these individuals even in the absence of WS or severe disease. This case also underscores the importance of genetic testing, including targeted panels specifically looking for <jats:italic>ELN<\/jats:italic> gene mutations, in all patients with SVAS even in the absence of phenotypic concerns for WS or other genetic syndromes.<\/jats:p>","DOI":"10.1007\/s00246-022-03089-3","type":"journal-article","created":{"date-parts":[[2023,2,17]],"date-time":"2023-02-17T07:53:51Z","timestamp":1676620431000},"page":"946-950","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Sudden Cardiac Arrest During a Sedated Cardiac Magnetic Resonance Study in a Nonsyndromic Child with Evolving Supravalvar Aortic Stenosis Due to Familial ELN Mutation"],"prefix":"10.1007","volume":"44","author":[{"given":"Dor","family":"Markush","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pedro A.","family":"Sanchez-Lara","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Katheryn","family":"Grand","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Robert","family":"Wong","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ruchira","family":"Garg","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2023,2,15]]},"reference":[{"issue":"6","key":"3089_CR1","doi-asserted-by":"publisher","first-page":"588","DOI":"10.1136\/hrt.71.6.588","volume":"71","author":"D Kitchiner","year":"1994","unstructured":"Kitchiner D, Jackson M, Malaiya N, Walsh K, Peart I, Arnold R (1994) Incidence and prognosis of obstruction of the left ventricular outflow tract in Liverpool (1960\u201391): a study of 313 patients. 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