Familial aortic dissection

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Synonyms: Annuloaortic ectasia | Cystic medial necrosis of aorta

Familial aortic dissection is the term used to describe rupture of the aortic wall at the level of the media resulting in the formation of a false channel and deviation of part of the aortic flux. Familial predisposition to thoracic aortic aneurysms and type A dissections (concerning the ascending aorta and/or the aortic arch) has been demonstrated in around 19% of patients presenting with thoracic aortic dissections and several loci have been identified so far (16p12.2-p13.13 3p24-25). This predisposition is transmitted in an autosomal dominant manner.

Data from Orphanet are used to provide information on a disease's name, synonym(s), and overview. Reference: Access aggregated data from Orphanet at Orphadata. Orphadata: Free access data from Orphanet. © INSERM 1999. Available on http://www.orphadata.org. Data version June 2024

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