The main aim of the research is studying of the intracellular signaling pathways that can cause and give rise to congenital defects of aorta and left ventricle outflow tract — aneurysm, mitral valve and aortic coarctation. Aortic aneurysm is a fairly common disease that causes death in 1-2% of total death rate in developed countries. It was believed that Extra Cellular Matrix (ECM) played the key role in the pathogenesis of this disease, as ECM modifies with aneurysm and causes changes in vessel wall properties and eventuates in aneurismal dilatation and rupture. However, now it becomes more and more apparent that aortic aneurysm involves functional alterations in signaling pathways associated with the intercellular signaling in endothelial and smooth muscle cells, particularly, due to certain genetic defects. Among them, signaling cascades Notch and TGF-beta are the most important controlling mechanisms in morphosis process, for example identification of embryo-fetal development, cell proliferation and differentiation. Moreover, Notch and TGF-beta signaling cascades are considerable in regeneration and angenesis. Now it has been proved that Notch and TGF-beta are involved in heart development and performance, particularly it refers to ascending aorta. Therefore, present research work is aimed to studying Notch and TGF-beta signaling cascades in aortic endothelial and smooth muscle cells under normal and pathological conditions. The influence of endothelial cell origin on endothelial mesenchymal migration has been shown and differences in functional properties of aortic smooth muscle cells between normal and aortic aneurysm patients have been detected. The significant research work was focused on investigation of Notch1’s genetic defects in patients with aortic coarctation, aneurysm and bicuspid valve. All these results provided the basis for articles of 2013 and in 2012 this research was the topic for collaborative work with Karolinska Institutet.