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We report an instance of aborted sudden cardiac arrest in a 7\u2010year\u2010old male with a confirmed <jats:italic>SOS1<\/jats:italic> variant and a lack of evidence to support a structural cardiac, metabolic, or infectious etiology. This is the second reported instance of sudden cardiac arrest related to ventricular fibrillation in a child with <jats:italic>SOS1<\/jats:italic>\u2010related NS in the absence of any structural cardiac defects. Although no definitive correlation can be ascertained from a limited existing body of knowledge surrounding <jats:italic>SOS1<\/jats:italic> and ventricular fibrillation unrelated to structural heart defects, it provokes the idea of an arrhythmia phenotype and future research is warranted to guide proper clinical treatment, monitoring, and management of such individuals.<\/jats:p>","DOI":"10.1002\/ajmg.a.63912","type":"journal-article","created":{"date-parts":[[2024,10,24]],"date-time":"2024-10-24T04:30:05Z","timestamp":1729744205000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["<scp><i>SOS1<\/i><\/scp>\u2010Related Noonan Syndrome and Sudden Cardiac Arrest in the Absence of Cardiomyopathy\u2014An Arrhythmia Phenotype?"],"prefix":"10.1002","volume":"197","author":[{"ORCID":"https:\/\/orcid.org\/0009-0007-4561-8049","authenticated-orcid":false,"suffix":"Jr","given":"Michael A.","family":"Cirelli","sequence":"first","affiliation":[{"name":"Mayo Clinic Alix School of Medicine Mayo Clinic  Scottsdale Arizona USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Philip","family":"Wackel","sequence":"additional","affiliation":[{"name":"Division of Pediatric Cardiology, Department of Pediatric and Adolescent Medicine Mayo Clinic  Rochester Minnesota USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Rabia","family":"Javed","sequence":"additional","affiliation":[{"name":"Division of Pediatric Hospital Medicine, Department of Pediatric and Adolescent Medicine  Rochester Minnesota USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ralitza","family":"Gavrilova","sequence":"additional","affiliation":[{"name":"Department of Clinical Genomics Mayo Clinic  Rochester Minnesota USA"},{"name":"Department of Neurology Mayo Clinic  Rochester Minnesota USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M. 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