Abstract
Patients with a bicuspid aortic valve (BAV) and Marfan syndrome (MFS) have increased susceptibility for development ofaortopathy. In the heart, epicardial cells expressing Wilms tumor suppressor protein (Wt1) are known to become activated after myocardial infarction. We hypothesize that epicardium covering the aorta might show a similar response in BAV and MFS in pathologic conditions.Non- and dilated ascending aorta specimen of BAV (n = 36), tricuspid aortic valve (TAV) (n = 23), and MFS (8) were investigated. The aorta was studied by immunohistochemistry and immunofluorescence, using Wt1 and endothelial nitric oxide, which regulates Wt1 expression. Functionality and therefore activity of Wt1 was confirmed by co-expression with retinaldehyde dehydrogenase-II enzyme in the same cells.
Highlights
Patients with a bicuspid aortic valve (BAV) and patients with Marfan syndrome (MFS) carry an increased risk for aortic dilation as compared to patients with a tricuspid aortic valve (TAV)
A significant increase in Wt1 activity is solely seen in the dilated TAV
Immature vascular smooth muscle cells and absence of pathologic features of cardiovascular ageing in BAV and MFS might explain the observed lack of activation potential in these patients
Summary
Patients with a bicuspid aortic valve (BAV) and patients with Marfan syndrome (MFS) carry an increased risk for aortic dilation as compared to patients with a tricuspid aortic valve (TAV). Dia architecture, characterized by an increased MMP activity, decreased fibrillin-1 expression, immature vascular smooth muscle cells (VSMCs) and lack of features of cardiovascular ageing in the dilated aorta [1,2,3,4]. This is in contrast to the aortic wall in TAV, which harbours differentiated VSMCs and shows a significant increase in expression of progerin [4], a marker of cardiovascular ageing in the dilated aorta [5,6]. In adult life the epicardium and epicardium derived cells are in a quiescent stage, but it has been shown that these inactive cells can become active in response to pathological processes such as myocardial ischemia [14]
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