{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,7]],"date-time":"2026-06-07T03:37:27Z","timestamp":1780803447989,"version":"3.54.1"},"reference-count":58,"publisher":"SAGE Publications","issue":"1","license":[{"start":{"date-parts":[[2020,6,19]],"date-time":"2020-06-19T00:00:00Z","timestamp":1592524800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/journals.sagepub.com\/page\/policies\/text-and-data-mining-license"}],"content-domain":{"domain":["journals.sagepub.com"],"crossmark-restriction":true},"short-container-title":["Vascular"],"published-print":{"date-parts":[[2021,2]]},"abstract":"<jats:p>Loeys\u2013Dietz syndrome is an autosomal dominant genetic disorder which is associated with significant and often crucial vascular manifestations. This review is aimed to examine current evidence on pathophysiology and management of Loeys\u2013Dietz syndrome in current era. A comprehensive electronic search was done to identify the articles that discussed all the aspects of Loeys\u2013Dietz syndrome, combined key words and Medical Subject Headings (MeSH) terms were used. Relevant articles have been summarized in each relevant section. Loeys\u2013Dietz syndrome is an autosomal dominant genetic disorder which has combined and multi-systemic manifestations. The increased breakdown of extracellular matrix predisposes an individual to developing aneurysms in the aortic tree which is undoubtedly the most significant complication of this disorder. Understanding the pathophysiology and natural history of Loeys\u2013Dietz syndrome and regular surveillance is important to plan prophylactic interventions to prevent life-threatening aortic emergencies which can be fatal. Loeys\u2013Dietz syndrome is an aggressive genetic condition that predisposes an individual to the development of life-threatening aortic aneurysms. Our understanding of Loeys\u2013Dietz syndrome remains ever-changing and it is likely that the knowledge regarding its diagnosis and treatment will become more clearly defined in the coming years with deeper genetic studies.<\/jats:p>","DOI":"10.1177\/1708538120934582","type":"journal-article","created":{"date-parts":[[2020,6,19]],"date-time":"2020-06-19T13:54:41Z","timestamp":1592574881000},"page":"3-14","update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":14,"title":["Loeys\u2013Dietz syndrome pathology and aspects of cardiovascular management: A systematic review"],"prefix":"10.1177","volume":"29","author":[{"given":"Rizwan","family":"Iqbal","sequence":"first","affiliation":[{"name":"St John\u2019s Hospital, Livingston, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Samiha","family":"Alom","sequence":"additional","affiliation":[{"name":"School of Public Health, Imperial College London, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jalal","family":"BinSaeid","sequence":"additional","affiliation":[{"name":"Department of Cardiothoracic Surgery, Liverpool Heart and Chest Hospital, Liverpool, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5507-5841","authenticated-orcid":false,"given":"Amer","family":"Harky","sequence":"additional","affiliation":[{"name":"Department of Cardiothoracic Surgery, Liverpool Heart and Chest Hospital, Liverpool, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"179","published-online":{"date-parts":[[2020,6,19]]},"reference":[{"key":"bibr1-1708538120934582","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa055695"},{"key":"bibr2-1708538120934582","doi-asserted-by":"publisher","DOI":"10.1097\/HCO.0b013e32833f0220"},{"key":"bibr3-1708538120934582","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.a.33356"},{"key":"bibr4-1708538120934582","doi-asserted-by":"publisher","DOI":"10.1038\/gim.2014.11"},{"key":"bibr5-1708538120934582","doi-asserted-by":"publisher","DOI":"10.1136\/bmj.b2535"},{"key":"bibr6-1708538120934582","doi-asserted-by":"publisher","DOI":"10.1016\/j.jtcvs.2018.03.172"},{"key":"bibr7-1708538120934582","doi-asserted-by":"publisher","DOI":"10.1016\/j.avsg.2016.06.007"},{"key":"bibr8-1708538120934582","doi-asserted-by":"publisher","DOI":"10.1007\/s00246-009-9541-z"},{"key":"bibr9-1708538120934582","doi-asserted-by":"publisher","DOI":"10.1510\/icvts.2010.258681"},{"key":"bibr10-1708538120934582","first-page":"663","volume":"38","author":"Hughes GC.","year":"2011","journal-title":"Tex Heart Inst J"},{"key":"bibr11-1708538120934582","doi-asserted-by":"publisher","DOI":"10.1016\/j.athoracsur.2012.05.111"},{"key":"bibr12-1708538120934582","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCGENETICS.116.001485"},{"key":"bibr13-1708538120934582","doi-asserted-by":"publisher","DOI":"10.1016\/j.athoracsur.2010.08.077"},{"key":"bibr14-1708538120934582","doi-asserted-by":"publisher","DOI":"10.1016\/j.athoracsur.2017.01.053"},{"key":"bibr15-1708538120934582","doi-asserted-by":"publisher","DOI":"10.1016\/j.jtcvs.2016.10.088"},{"key":"bibr16-1708538120934582","doi-asserted-by":"publisher","DOI":"10.1016\/j.athoracsur.2008.10.049"},{"key":"bibr17-1708538120934582","doi-asserted-by":"publisher","DOI":"10.1016\/j.jvscit.2018.03.001"},{"key":"bibr18-1708538120934582","doi-asserted-by":"publisher","DOI":"10.1111\/j.1540-8191.2011.01224.x"},{"key":"bibr19-1708538120934582","doi-asserted-by":"publisher","DOI":"10.1016\/j.athoracsur.2006.10.091"},{"key":"bibr20-1708538120934582","doi-asserted-by":"publisher","DOI":"10.1016\/j.cytogfr.2017.09.003"},{"key":"bibr21-1708538120934582","doi-asserted-by":"crossref","unstructured":"Arslan-Kirchner M, Epplen JT, Faivre L, et al. 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