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Acute aortic syndrome is often the first sign of the disease, which needs rapid diagnosis and decision making to reduce the extremely poor prognosis. Aortic dilatation is a well-recognised risk factor for acute events and can occur as a result of trauma, infection, or, most commonly, from an intrinsic abnormality in the elastin and collagen components of the aortic wall. Over the years it has become clear that a few monogenic syndromes are strongly associated with aneurysms and often dictate a severe presentation in younger patients while the vast majority have a multifactorial pathogenesis. Conventional cardiovascular risk factors and ageing play an important role. Management strategy is based on prevention consisting of regular follow-up with cross-sectional imaging, chemoprophylaxis of further dilatation with drugs proved to slow down the disease progression and preventative surgery when dimension exceeds internationally recognised cut-off values for aortic diameters and the risk of rupture\/dissection is therefore deemed very high.<\/jats:p>","DOI":"10.1530\/erp-19-0049","type":"journal-article","created":{"date-parts":[[2020,1,28]],"date-time":"2020-01-28T14:43:22Z","timestamp":1580222602000},"page":"R1-R10","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":27,"title":["A Review of Thoracic Aortic Aneurysm Disease"],"prefix":"10.1186","volume":"7","author":[{"given":"Paul F.","family":"Clift","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elena","family":"Cervi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2020,3,1]]},"reference":[{"key":"701003_CR1","first-page":"2031","volume-title":"Circulation","author":"DP Howard","year":"2013","unstructured":"Howard DP, Banerjee A, Fairhead JF, Perkins J, Silver LE, Rothwell PM & Oxford Vascular Study. 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