The bicuspid aortic valve (BAV) is a congenital heart defect which is characterized by the formation of two aortic leaflets instead of the normal three leaflets within the tricuspid aortic valve ... Show moreThe bicuspid aortic valve (BAV) is a congenital heart defect which is characterized by the formation of two aortic leaflets instead of the normal three leaflets within the tricuspid aortic valve (TAV). Whilst all BAV patients have a bicuspid valve, extended patient monitoring has revealed a large variation of disease progression trajectories during a patient’s lifetime. This large variation troubles clinical decision making due to the uncertain proliferation of BAV disease. More knowledge of the biological mechanisms underlying BAV could address that uncertainty and thus help stratify patient risk with more accuracy. Therefore this thesis aims to advance our current understanding regarding the biological impact and developmental mechanisms underlying congenital BAV and BAV related aortopathy. Show less