Сardiovascular continuum in Marfan syndrome

Mechanisms of cardiovascular complications occurred in connective tissue disorders in general and in Marfan syndrome and related inherited disorders in particular are reviewed. New approaches to diagnostics of Marfan syndrome in accordance with the revised Ghent nosology were evaluated. It was noticed that cardiovascular complications could develop not only as aortic rupture and dissection but also as progressive heart failure due to Marfan syndrome cardiomyopathy or aortal or/ and mitral regurgitation. Some dysembriogenetic signs and their specificity and sensitivity for diagnostics of Marfan syndrome and related inherited disorders were assessed.