[Cardiovascular continuum in Marfan syndrome].

Cardiovascular continuum in Marfan syndrome and clinically similar manifestations of inherited connective tissue pathology (ICTP) is discussed. New approaches to diagnostics and treatment of Marfan syndrome in conformity with revised Ghent criteria are described, the role of systemic involvement of connective tissue is discussed with reference to the relationship between transient cardiovascular continuum and complications of Marfan syndrome related to progression of aortic aneurysm and cardiomyopathy. The existence of dysplastic cardiomyopathy underlain by systemic involvement of connective tissue is postulated. The second part of the lecture deals with spatial phenotypic continuum the understanding of which is indispensable for diagnostics of ICTP. Marked variability of the signs of dysembryogenesis, its sensitivity and specificity are emphasized. It is concluded that connective tissue dysplasia can not be diagnosed based only on a few signs of dysembryogenesis.