Familial aortic dissection

Orpha code: 229OMIM code: 607086

Definition

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.

Disease data
Classification

Disease

Synonyms
Annuloaortic ectasia
Choroba Erdheima
Rodzinne poszerzenie i rozwarstwienie aorty
Torbielowata pośrodkowa martwica aorty
Cystic medial necrosis of aorta
ORPHA code
229
OMIM code
607086
ICD10 code
I71.0
ICD11 code
BD50.Z

No additional description.

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