Bicuspid aortic valve; optimal diagnosis and latest interventional treatment.

Bicuspid aortic valve (BAV) is one of the most common congenital heart defects with a population prevalence of 0.5 to 1.3 % [1]. The defect is considered to be a heritable disorder with a family recurrence rate of approximately 35 %. Recent studies show that mutations in the NOTCH1 gene are associated with BAV [2]. BAV progresses more rapidly into regurgitation or stenosis of the valve [3]. This results in a higher occurrence of aortic valve replacement, especially at younger age. Additionally, BAV is more susceptible than a tricuspid aortic valve (TAV) to nest bacteria or other organisms, leading to endocarditis. BAV is not only a peculiar valve morphology leading to specific valve pathology, it is also frequently associated with (asymptomatic) ascending aorta dilatation which leads to an increased susceptibility to ascending aortic aneurysms and aortic dissection [4]. Aortic elasticity measurements of BAV patients suggest that diminished aortic elasticity is at least part of its causation.