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In the era of next\u2010generation sequencing, multiple genes including <jats:italic>COL3A1<\/jats:italic> can be simultaneously analyzed, and among patients suffering from aortopathy even without any other clinical features suggestive of vEDS, pathogenic <jats:italic>COL3A1<\/jats:italic> variants have been increasingly identified. Here, we briefly summarize the characteristics of 12 Japanese patients from 11 families with arteriopathy and pathogenic or likely pathogenic <jats:italic>COL3A1<\/jats:italic> variants in our hospital. Five patients did not have any extra\u2010arterial clinical features, however, the multigene panel testing for hereditary thoracic aortic aneurysm and dissection unexpectedly revealed that two had glycine substitutions in the triple\u2010helical region and three had haploinsufficient type variants in the <jats:italic>COL3A1<\/jats:italic> gene, whose pathogenicities were all classified as pathogenic or likely pathogenic. Further genetic screening and identification of pathogenic variants in patients with nonsyndromic arteriopathy and aortopathy will enable us to develop risk\u2010stratification and management based on the genetic diagnosis.<\/jats:p>","DOI":"10.1002\/ajmg.a.62774","type":"journal-article","created":{"date-parts":[[2022,5,11]],"date-time":"2022-05-11T09:45:25Z","timestamp":1652262325000},"page":"2777-2782","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":8,"title":["Nonsyndromic arteriopathy and aortopathy and vascular Ehlers\u2013Danlos syndrome <scp>causing <i>COL3A1<\/i><\/scp> variants"],"prefix":"10.1002","volume":"188","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-6615-6196","authenticated-orcid":false,"given":"Hiroki","family":"Yagi","sequence":"first","affiliation":[{"name":"Department of Cardiovascular Medicine The University of Tokyo Hospital  Tokyo Japan"},{"name":"Marfan Syndrome Center The University of Tokyo 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