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Familial aortic dissection

Orpha number ORPHA229
Synonym(s) Annuloaortic ectasia
Cystic medial necrosis of aorta
Erdheim disease
Prevalence -
Inheritance -
Age of onset All ages
ICD-10
  • I71.0
ICD-O -
OMIM
UMLS -
MeSH -
MedDRA -

Summary

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.


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