[Dystrophic aortic insufficiency]. / Insuffisance aortique dystrophique.

ABSTRACT

Dystrophic AR is the combination of two features including ascending aorta enlargement and aortic regurgitation due to change in aortic root geometry Marfan syndrome, an inherited connective tissue disorder, is the most frequent genetic aetiology of dystrophic AR. The main clinical manifestations involve the cardiovascular, ocular and skeletal system. Aortic dilatations and ruptures are the most serious complications. Thus management of the disease require frequent follow-up visit with measurement of aorta diameters, examination of family members, medical treatment with beta blockers and evaluation of the need of surgery based on dilatation of ascending aorta and on tolerance of aortic regurgitation.