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Mice with <jats:italic>Lmod2<\/jats:italic> deficiency die around 3\u2009weeks of age due to severe dilated cardiomyopathy (DCM), resulting from decreased heart contractility due to shorter thin filaments. To date, there have been three infants from two families reported with biallelic variants in <jats:italic>LMOD2<\/jats:italic>, presenting with perinatal onset DCM. Here, we describe a third family with a child harboring a previously described homozygous frameshift variant, c.1243_1244delCT (p.L415Vfs*108) with DCM, presenting later in infancy at 9\u2009months of age. Family history was relevant for a sibling who died suddenly at 1\u2009year of age after being diagnosed with cardiomegaly. <jats:italic>LMOD2<\/jats:italic>\u2010related cardiomyopathy is a rare form of inherited cardiomyopathy resulting from thin filament length dysregulation and should be considered in genetic evaluation of newborns and infants with suspected autosomal recessive inheritance or sporadic early onset cardiomyopathy.<\/jats:p>","DOI":"10.1002\/ajmg.a.62699","type":"journal-article","created":{"date-parts":[[2022,2,21]],"date-time":"2022-02-21T12:42:27Z","timestamp":1645447347000},"page":"1858-1862","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":19,"title":["<scp><i>LMOD2<\/i><\/scp>\u2010related dilated cardiomyopathy presenting in late infancy"],"prefix":"10.1002","volume":"188","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-7568-2737","authenticated-orcid":false,"given":"Erica","family":"Lay","sequence":"first","affiliation":[{"name":"Department of Molecular and Human Genetics Baylor College of Medicine  Houston Texas USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mahshid S.","family":"Azamian","sequence":"additional","affiliation":[{"name":"Department of Molecular and Human Genetics Baylor College of Medicine  Houston Texas USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Susan W.","family":"Denfield","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Section of Cardiology, Texas Children's Hospital  Houston Texas USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"William","family":"Dreyer","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Section of Cardiology, Texas Children's Hospital  Houston Texas 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