The aortopathy associated with bicuspid aortic valve (BAV) is an epidemiologically relevant
source of chronic and acute aortic disease (aneurysm and dissection). However, its …

Herein we summarize the current knowledge on the bicuspid aortic valve (BAV)-associated
aortopathy regarding clinical presentation and disease sub-classification, genetic …

The current management of aortic dilatation associated with congenital bicuspid aortic valve
(bicuspid aortic valve aortopathy) is based on dimensional parameters (diameter of the …

Bicuspid aortic valve (BAV) exhibits a clinical incline toward aortopathy, in which aberrant
tensile and shear stress generated by BAV can induce differential expression of matrix …

Abstract About 1–2% of the population present with bicuspid aortic valves (BAV), a defect of
the aortic valve resulting in the formation of two leaflets instead of three. This disease leads …

Marfan syndrome is an inherited disorder characterized by genetic abnormality of
microfibrillar connective tissue proteins. Endothelial dysfunction is thought to cause aortic …

The clinical phenotypic variability seen in BAV patients suggests complex interactions
between genetic variants, epigenetic regulation modifications and the variable effect of valve …

Abstract Alpha1-antitrypsin (AAT) is one of the major inhibitors involved in
protease/antiprotease homeostasis, and it is mainly produced by hepatocytes and …

Progressive aortic dilation occurs in patients with tetralogy of Fallot (TOF), possibly due to
abnormal histopathology of the aortic media that weakens the aortic wall. This medial …

Background Coarctation of the aorta coexists with other cardiac anomalies and has long‐
term complications, including recoarctation, which may require intervention after the primary …