Description of the disease * EnglishPolish Pobierz sekcję do PDF 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 *Soruce Extended description of the disease Pobierz sekcję do PDF No additional description. Orphanet - interntowa baza danych dotyczących rzadkich chorób i sierochych leków. ©INSERM 1999 - Dostępna na stronie www.orphanet.pl