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Up to this date, fewer than 200 SQTS cases have been reported in the literature worldwide. Patients with SQTS may experience a cardiac arrest as early as in the neonatal period or as late as 80 years old. The cumulative probability of experiencing a cardiac arrest by the fifth decade of life approaches 40%, highlighting the importance of early recognition and management. SQTS is an autosomal dominant disease with five identified causative genes, including three that encode for potassium channels (<jats:italic>KCNH2<\/jats:italic>, <jats:italic>KCNQ1<\/jats:italic>, and <jats:italic>KCNJ2<\/jats:italic>) and two that encode for subunits of the L\u2010type calcium channels (<jats:italic>CACNA1C<\/jats:italic> and <jats:italic>CACNB2<\/jats:italic>). The term \u201cearly repolarization\u201d (ER) has long been used to refer to a heterogeneous group of specific QRS\u2010T junction patterns that are commonly found on the electrocardiograms of young healthy subjects. In the last decade, it has been suggested that in some individuals, the presence of ER may be associated with an increased risk of sudden cardiac death, and thus the term \u201cearly repolarization syndrome\u201d (ERS) has progressively entered into use. Up to this point, however, whether ER constitutes a true primary arrhythmic disorder or whether it is simply a predisposing substrate that facilitates arrhythmias in the presence of other triggers remains an unresolved issue. In this review paper, we aim to integrate the current literature on SQTS and ERS. For each, we will describe the key steps that first led to the identification of the syndrome before moving into a discussion of our current understanding of each entity, including the epidemiology, genetics, diagnosis, clinical manifestations, and management.<\/jats:p>","DOI":"10.1111\/jce.13265","type":"journal-article","created":{"date-parts":[[2017,6,1]],"date-time":"2017-06-01T10:12:44Z","timestamp":1496311964000},"page":"1226-1236","source":"Crossref","is-referenced-by-count":32,"title":["The new kids on the block of arrhythmogenic disorders: Short QT syndrome and early repolarization"],"prefix":"10.1111","volume":"28","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-0208-2172","authenticated-orcid":false,"given":"Andrea","family":"Mazzanti","sequence":"first","affiliation":[{"name":"Molecular Cardiology IRCCS ICS Maugeri  Pavia Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Katherine","family":"Underwood","sequence":"additional","affiliation":[{"name":"Molecular Cardiology IRCCS ICS Maugeri  Pavia Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dmitriy","family":"Nevelev","sequence":"additional","affiliation":[{"name":"Molecular Cardiology IRCCS ICS Maugeri  Pavia Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shanna","family":"Kofman","sequence":"additional","affiliation":[{"name":"Molecular Cardiology IRCCS ICS Maugeri  Pavia Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Silvia G.","family":"Priori","sequence":"additional","affiliation":[{"name":"Molecular Cardiology IRCCS ICS Maugeri  Pavia Italy"},{"name":"Department of Molecular Medicine University of Pavia  Pavia Italy"},{"name":"Fundaci\u00f3n Centro Nacional de Investigaciones Cardiovasculares  Madrid Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2017,6,27]]},"reference":[{"key":"e_1_2_5_2_1","doi-asserted-by":"publisher","DOI":"10.1093\/cvr\/20.6.398"},{"key":"e_1_2_5_3_1","doi-asserted-by":"publisher","DOI":"10.1161\/01.CIR.0000085071.28695.C4"},{"key":"e_1_2_5_4_1","doi-asserted-by":"publisher","DOI":"10.1136\/hrt.70.1.43"},{"key":"e_1_2_5_5_1","doi-asserted-by":"publisher","DOI":"10.1159\/000047299"},{"key":"e_1_2_5_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2011.03.038"},{"key":"e_1_2_5_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2013.09.078"},{"key":"e_1_2_5_8_1","first-page":"30119","article-title":"Prevalence of Electrocardiographic Patterns Associated With Sudden Cardiac Death in the Spanish Population Aged 40 Years or Older","volume":"1885","author":"Awamleh Garc\u00eda P","year":"2017","journal-title":"Results of the OFRECE Study. 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