{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,7]],"date-time":"2026-10-07T18:19:37Z","timestamp":1791397177370,"version":"4.3.3"},"reference-count":148,"publisher":"MDPI AG","issue":"3","license":[{"start":{"date-parts":[[2025,3,8]],"date-time":"2025-03-08T00:00:00Z","timestamp":1741392000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JPM"],"abstract":"<jats:p>Background: Short QT syndrome (SQTS) is a rare inheritable channelopathy characterized by a shortened corrected QT interval on an electrocardiogram and a significant risk of atrial and ventricular arrhythmias, potentially leading to sudden cardiac death. Despite advancements in our understanding of SQTS, knowledge gaps persist due to its extreme rarity. This scoping review aims to summarize the available knowledge on its clinical presentations, genetic mutations, and management strategies, while identifying areas for further investigation. Methods: This scoping review was conducted across the PubMed, Scopus, and Cochrane databases and identified relevant case reports, case series, and available studies on SQTS. We focused on articles that reported clinical outcomes, genetic mutations, diagnostic criteria, and management strategies, while excluding studies on the secondary causes of short QT intervals. Results: SQTS is present across a wide age range, from asymptomatic individuals to those experiencing syncope, palpitations, or sudden cardiac arrest. Common genetic mutations include KCNQ1, KCNH2, and KCNJ2. Management strategies vary, with some patients receiving implantable cardioverter defibrillators for secondary prevention and others treated pharmacologically, primarily with hydroquinidine. Our findings highlight the rarity and clinical variability of SQTS, underscoring the need for optimized diagnostic criteria and individualized management strategies. Conclusions: This review emphasizes the need for continued research to better understand the genetic basis of SQTS, optimize diagnostic tools, and improve treatment approaches. Large-scale studies and the integration of genetic and clinical data are critical to addressing the gaps in SQTS management and improving outcomes for patients with this potentially life-threatening arrhythmic disorder.<\/jats:p>","DOI":"10.3390\/jpm15030105","type":"journal-article","created":{"date-parts":[[2025,3,10]],"date-time":"2025-03-10T05:46:52Z","timestamp":1741585612000},"page":"105","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":9,"title":["The Uncommon Phenomenon of Short QT Syndrome: A Scoping Review of the Literature"],"prefix":"10.3390","volume":"15","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5008-114X","authenticated-orcid":false,"given":"Aristi","family":"Boulmpou","sequence":"first","affiliation":[{"name":"Third Department of Cardiology, Ippokratio General Hospital of Thessaloniki, Aristotle University of Thessaloniki, 54642 Thessaloniki, Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andreas","family":"Giannopoulos","sequence":"additional","affiliation":[{"name":"Department of Pediatric Cardiology, Second Department of Pediatrics, AHEPA University Hospital, Aristotle University of Thessaloniki, 54636 Thessaloniki, Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9643-9066","authenticated-orcid":false,"given":"Christodoulos","family":"Papadopoulos","sequence":"additional","affiliation":[{"name":"Third Department of Cardiology, Ippokratio General Hospital of Thessaloniki, Aristotle University of Thessaloniki, 54642 Thessaloniki, Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7287-3850","authenticated-orcid":false,"given":"Georgios","family":"Giannopoulos","sequence":"additional","affiliation":[{"name":"Third Department of Cardiology, Ippokratio General Hospital of Thessaloniki, Aristotle University of Thessaloniki, 54642 Thessaloniki, Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ioannis","family":"Papagiannis","sequence":"additional","affiliation":[{"name":"Department of Pediatric Cardiology and Adult Congenital Heart Disease, Onassis Cardiac Surgery Centre, 17674 Athens, Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Georgios","family":"Zormpas","sequence":"additional","affiliation":[{"name":"Second Department of Cardiology, Ippokratio General Hospital of Thessaloniki, Aristotle University of Thessaloniki, 54642 Thessaloniki, Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anastasia","family":"Keivanidou","sequence":"additional","affiliation":[{"name":"Department of Pediatric Cardiology, Second Department of Pediatrics, AHEPA University Hospital, Aristotle University of Thessaloniki, 54636 Thessaloniki, Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Liana","family":"Fidani","sequence":"additional","affiliation":[{"name":"Second Department of Pediatrics, AHEPA University Hospital, Aristotle University of Thessaloniki, 54636 Thessaloniki, Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0885-9170","authenticated-orcid":false,"given":"Vassilios","family":"Vassilikos","sequence":"additional","affiliation":[{"name":"Third Department of Cardiology, Ippokratio General Hospital of Thessaloniki, Aristotle University of Thessaloniki, 54642 Thessaloniki, Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2025,3,8]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"99","DOI":"10.1159\/000047299","article-title":"Idiopathic short QT interval: A new clinical syndrome?","volume":"94","author":"Gussak","year":"2000","journal-title":"Cardiology"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"782","DOI":"10.1161\/01.CIR.88.2.782","article-title":"Diagnostic criteria for the long QT syndrome. 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