Table 2.

Hereditary factors—longitudinal extent—arch involvement of aortopathy.

Aortic dilation patterns are distributed similarly regardless of BAV type [1, 17]
BAV patients' relatives with tricuspid aortic valve have stiffer, less compliant, and somewhat enlarged aortas [20]
After isolated BAV replacement, aortic dilation progresses and the risk of aortic rupture and dissection remain higher [21]
Aortic diameter per se is a quantitative trait that exhibits significant familial heritability—bicuspid aortic valve is independent modifier [22]
TGF-beta signaling—differential splicing is specific for BAV and TAV patients [23]
Defective fibronectin splicing within the aortic wall of bicuspid aortic valve is associated with aortic aneurysm development [26]
ACE insertion/deletion polymorphism associated with aneurysm formation [27]