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HCM is a global condition, diagnosed in &amp;gt;50 countries and in all continents. HCM affects people of both sexes and various ethnic and racial origins, with similar clinical course and phenotypic expression. The most unpredictable and devastating consequence of HCM is represented by arrhythmic SCD, most commonly caused by sustained ventricular tachycardia or ventricular fibrillation. Indeed, HCM represents one of the main causes of arrhythmic SCD in the young, with a marked preference for children and adults &amp;lt;30\u2009years. SCD is most prevalent in patients with paediatric onset of HCM but may occur at any age. However, risk is substantially lower after 60\u2009years, suggesting that the potential for ventricular tachyarrhythmias is mitigated by ageing. SCD had been linked originally to sports and vigorous activity in HCM patients. However, it is increasingly clear that the majority of events occurs at rest or during routine daily occupations, suggesting that triggers are far from consistent. In general, the pathophysiology of SCD in HCM remains unresolved. While the pathologic and physiologic substrates abound and have been described in detail, specific factors precipitating ventricular tachyarrhythmias are still unknown. SCD is a rare phenomenon in HCM cohorts (&amp;lt;1%\/year) and attempts to identify patients at risk, while generating clinically useful algorithms for primary prevention, remain very inaccurate on an individual basis. One of the reasons for our limited understanding of these phenomena is that limited translational research exists in the field, while most efforts have focused on clinical markers of risk derived from pathology, instrumental patient evaluation, and imaging. Specifically, few studies conducted in animal models and human samples have focused on targeting the cellular mechanisms of arrhythmogenesis in HCM, despite potential implications for therapeutic innovation and SCD prevention. These studies found that altered intracellular Ca2+ homoeostasis and increased late Na+ current, leading to an increased likelihood of early and delayed after-depolarizations, contribute to generate arrhythmic events in diseased cardiomyocytes. As an array of novel experimental opportunities have emerged to investigate these mechanisms, including novel \u2018disease-in-the-dish\u2019 cellular models with patient-specific induced pluripotent stem cell-derived cardiomyocytes, important gaps in knowledge remain. Accordingly, the aim of the present review is to provide a contemporary reappraisal of the cellular basis of SCD-predisposing arrhythmias in patients with HCM and discuss the implications for risk stratification and management.<\/jats:p>","DOI":"10.1093\/cvr\/cvaa124","type":"journal-article","created":{"date-parts":[[2020,4,24]],"date-time":"2020-04-24T19:10:03Z","timestamp":1587755403000},"page":"1585-1599","source":"Crossref","is-referenced-by-count":65,"title":["Abnormalities in sodium current and calcium homoeostasis as drivers of arrhythmogenesis in hypertrophic cardiomyopathy"],"prefix":"10.1093","volume":"116","author":[{"given":"Raffaele","family":"Coppini","sequence":"first","affiliation":[{"name":"Department of Neurosciences, Psychiatry, Drug Research and Child Health (NeuroFarBa), University of Florence, Florence, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lorenzo","family":"Santini","sequence":"first","affiliation":[{"name":"Department of Neurosciences, Psychiatry, Drug Research and Child Health (NeuroFarBa), University of Florence, Florence, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Iacopo","family":"Olivotto","sequence":"first","affiliation":[{"name":"Department of Clinical and Experimental Medicine, University of Florence, Largo Brambilla, 3 - 50134 Florence, Italy"},{"name":"Cardiomyopathy Unit, Careggi University Hospital, Florence, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael J","family":"Ackerman","sequence":"first","affiliation":[{"name":"Division of Heart Rhythm Services, Department of Cardiovascular Medicine, Mayo Clinic, 200 First St. SW, Rochester, MN, USA"},{"name":"Division of Pediatric Cardiology, Department of Pediatric and Adolescent Medicine, Mayo Clinic, 200 First St. SW, Rochester, MN, USA"},{"name":"Windland Smith Rice Sudden Death Genomics Laboratory, Department of Molecular Pharmacology & Experimental Therapeutics, Mayo Clinic, 200 First St. SW, Rochester, MN, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7839-1361","authenticated-orcid":false,"given":"Elisabetta","family":"Cerbai","sequence":"first","affiliation":[{"name":"Department of Neurosciences, Psychiatry, Drug Research and Child Health (NeuroFarBa), University of Florence, Florence, Italy"},{"name":"Laboratory of Non-Linear Spectroscopy (LENS), Via Nello Carrara 1, 50019 Sesto Fiorentino, Florence, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2020,5,4]]},"reference":[{"key":"2020070907341838000_cvaa124-B1","doi-asserted-by":"crossref","first-page":"1308","DOI":"10.1001\/jama.287.10.1308","article-title":"Hypertrophic cardiomyopathy: a systematic review","volume":"287","author":"Maron","year":"2002","journal-title":"JAMA"},{"key":"2020070907341838000_cvaa124-B2","doi-asserted-by":"crossref","first-page":"63","DOI":"10.1016\/j.amjmed.2003.10.012","article-title":"Hypertrophic cardiomyopathy: an important global disease","volume":"116","author":"Maron","year":"2004","journal-title":"Am J Med"},{"key":"2020070907341838000_cvaa124-B3","doi-asserted-by":"crossref","first-page":"785","DOI":"10.1161\/01.CIR.92.4.785","article-title":"Prevalence of hypertrophic cardiomyopathy in a general population of young adults. 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