An Exploratory Look at Bicuspid Aortic Valve (Bav) Aortopathy: Focus on Molecular and Cellular Mechanisms

Bicuspid aortic valve (BAV) is the most common congenital heart malformation. BAV patients are at increased risk for aortic valve disease (stenosis/regurgitation), infective endocarditis, thrombi formation and, in particular, aortic dilatation, aneurysm and dissection. This review aims at exploring the possible interplay among genetics, extracellular matrix remodeling, abnormal signaling pathways, oxidative stress and inflammation in contributing to BAV-associated aortopathy (BAV-A-A). Novel circulating biomarkers have been proposed as diagnostic tools able to improve risk stratification in BAV-A-A. However, to date, the precise molecular and cellular mechanisms that lead to BAV-A-A remain unknown. Genetic, hemodynamic and cardiovascular risk factors have been implicated in the development and progression of BAV-A-A. Oxidative stress may also play a role, similarly to what observed in atherosclerosis and vulnerable plaque formation. The identification of common pathways between these 2 conditions may provide a platform for future therapeutic solutions.