{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,24]],"date-time":"2026-07-24T04:21:30Z","timestamp":1784866890191,"version":"3.55.0"},"reference-count":125,"publisher":"Oxford University Press (OUP)","issue":"3","license":[{"start":{"date-parts":[[2021,9,13]],"date-time":"2021-09-13T00:00:00Z","timestamp":1631491200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/journals\/pages\/open_access\/funder_policies\/chorus\/standard_publication_model"}],"funder":[{"DOI":"10.13039\/501100004884","name":"Sichuan Provincial Department of Education","doi-asserted-by":"crossref","award":["17ZB0476"],"award-info":[{"award-number":["17ZB0476"]}],"id":[{"id":"10.13039\/501100004884","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2022,3,2]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:p>Cardiovascular diseases are the main cause of sudden cardiac death (SCD) in developed and developing countries. Inherited cardiac channelopathies are linked to 5\u201310% of SCDs, mainly in the young. Short QT syndrome (SQTS) is a rare inherited channelopathy, which leads to both atrial and ventricular tachyarrhythmias, syncope, and even SCD. International European Society of Cardiology guidelines include as diagnostic criteria: (i) QTc\u2009\u2264\u2009340\u2009ms on electrocardiogram, (ii) QTc\u2009\u2264\u2009360\u2009ms plus one of the follwing, an affected short QT syndrome pathogenic gene mutation, or family history of SQTS, or aborted cardiac arrest, or family history of cardiac arrest in the young. However, further evaluation of the QTc ranges seems to be required, which might be possible by assembling large short QT cohorts and considering genetic screening of the newly described pathogenic mutations. Since the mechanisms underlying the arrhythmogenesis of SQTS is unclear, optimal therapy for SQTS is still lacking. The disease is rare, unclear genotype\u2013phenotype correlations exist in a bevy of cases and the absence of an international short QT registry limit studies on the pathophysiological mechanisms of arrhythmogenesis and therapy of SQTS. This leads to the necessity of experimental models or platforms for studying SQTS. Here, we focus on reviewing preclinical SQTS models and platforms such as animal models, heterologous expression systems, human-induced pluripotent stem cell-derived cardiomyocyte models and computer models as well as three-dimensional engineered heart tissues. We discuss their usefulness for SQTS studies to examine genotype\u2013phenotype associations, uncover disease mechanisms and test drugs. These models might be helpful for providing novel insights into the exact pathophysiological mechanisms of this channelopathy and may offer opportunities to improve the diagnosis and treatment of patients with SQT syndrome.<\/jats:p>","DOI":"10.1093\/europace\/euab214","type":"journal-article","created":{"date-parts":[[2021,9,8]],"date-time":"2021-09-08T11:35:45Z","timestamp":1631100945000},"page":"481-493","source":"Crossref","is-referenced-by-count":18,"title":["Preclinical short QT syndrome models: studying the phenotype and drug-screening"],"prefix":"10.1093","volume":"24","author":[{"given":"Xuehui","family":"Fan","sequence":"first","affiliation":[{"name":"University of Mannheim, University of Heidelberg, Germany"},{"name":"Key Laboratory of Medical Electrophysiology, Ministry of Education and Medical Electrophysiological Key Laboratory of Sichuan Province, Collaborative Innovation Center for Prevention of Cardiovascular Diseases, Institute of Cardiovascular Research, Southwest Medical University, Luzhou, Sichuan, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Guoqiang","family":"Yang","sequence":"additional","affiliation":[{"name":"Department of Acupuncture and Rehabilitation, Hospital (T.CM.) Affiliated to Southwest Medical University, Luzhou, Sichuan, China"},{"name":"Research Unit of Molecular Imaging Probes, Department of Radiologic Technology, Faculty of Associated Medical Sciences, Chiang Mai University, Chiang Mai, Thailand"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jacqueline","family":"Kowitz","sequence":"additional","affiliation":[{"name":"University of Mannheim, University of Heidelberg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4748-0158","authenticated-orcid":false,"given":"Firat","family":"Duru","sequence":"additional","affiliation":[{"name":"Department of Cardiology, University Heart Centre, University Hospital Zurich, Zurich, Switzerland"},{"name":"Center for Integrative Human Physiology, University of Zurich, Zurich, Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ardan M","family":"Saguner","sequence":"additional","affiliation":[{"name":"Department of Cardiology, University Heart Centre, University Hospital Zurich, Zurich, Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ibrahim","family":"Akin","sequence":"additional","affiliation":[{"name":"University of Mannheim, University of Heidelberg, Germany"},{"name":"DZHK (German Center for Cardiovascular Research) Partner Site, Heidelberg-Mannheim, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5895-0127","authenticated-orcid":false,"given":"Xiaobo","family":"Zhou","sequence":"additional","affiliation":[{"name":"University of Mannheim, University of Heidelberg, Germany"},{"name":"Key Laboratory of Medical Electrophysiology, Ministry of Education and Medical Electrophysiological Key Laboratory of Sichuan Province, Collaborative Innovation Center for Prevention of Cardiovascular Diseases, Institute of Cardiovascular Research, Southwest Medical University, Luzhou, Sichuan, China"},{"name":"DZHK (German Center for Cardiovascular Research) Partner Site, Heidelberg-Mannheim, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0139-1045","authenticated-orcid":false,"given":"Ibrahim","family":"El-Battrawy","sequence":"additional","affiliation":[{"name":"University of Mannheim, University of Heidelberg, Germany"},{"name":"Department of Cardiology, University Heart Centre, University Hospital Zurich, Zurich, Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2021,9,13]]},"reference":[{"key":"2022030308001133800_euab214-B1","doi-asserted-by":"crossref","first-page":"S51","DOI":"10.1016\/j.hrthm.2009.02.009","article-title":"Mechanisms and clinical management of inherited channelopathies: long QT syndrome, Brugada syndrome, catecholaminergic polymorphic ventricular tachycardia, and short QT 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