Involvement of cardiovascular system in hereditary disorders of connective tissue

Hereditary disorders of connective tissue are diseases caused by diferent gene mutation and involve into pathologic process many organ systems, especially, cardiovascular, skeletomotor systems, eyes and skin. Marfan syndrome, Ehlers-Danlos syndrome, Loeys-Dietz syndrome are hereditary disorders which frequently damage cardiovascular system. Tese syndromes have common feature ? formation of aneurysms of aorta or other vessels and risk of their dissection. Te article presents modern view on the etiology and pathogenesis of mentioned syndromes, current diagnostic criteria, novel achievements in therapeutic management and guidelines for surgical interventions in this group of patients.