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Risk of sudden cardiac death is elevated in ACM patients and can be the presenting symptom particularly in younger individuals and athletes. This review describes current understanding of the genetic architecture of ACM and molecular mechanisms of ACM pathogenesis. We consider an emerging threshold model for ACM inheritance in which multiple factors including pathogenic variants in known ACM genes, genetic modifiers, and environmental exposures, particularly exercise, are required to reach a threshold for disease expression. We also review best practices for integrating genetics\u2014including recent discoveries\u2014in caring for ACM families and emphasize the utility of genotype for both management of affected individuals and predictive testing in family members.<\/jats:p>\n               <jats:p\/>","DOI":"10.1093\/eurheartj\/ehaa141","type":"journal-article","created":{"date-parts":[[2020,2,25]],"date-time":"2020-02-25T20:13:20Z","timestamp":1582661600000},"page":"1393-1400","source":"Crossref","is-referenced-by-count":87,"title":["The role of genetics in cardiovascular disease: arrhythmogenic cardiomyopathy"],"prefix":"10.1093","volume":"41","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-8040-4600","authenticated-orcid":false,"given":"Cynthia A","family":"James","sequence":"first","affiliation":[{"name":"Division of Cardiology, Department of Medicine, Johns Hopkins University , Carnegie 568D, 600 N. 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