If we postulate a genetic background to BAV aortopathy, it would be reasonable to assume that FBN1 variants would dominate the genetic cause of aortic root aneurysm, as common FBN1 variants have been associated with non-syndromic bicuspid aortic aneurysm, independent to the position of the aneurysm (LeMaire et al., 2011). Here, FBN1 is linked to Aortic root aneurysm.