ABSTRACT
The bicuspid aortic valve (BAV) is the most common congenital cardiovascular malformation. Patients with BAV are at higher risk of other congenital cardiovascular malformations and valvular dysfunction, including aortic stenosis/regurgitation and infective endocarditis. BAV may also be related to aortic wall abnormalities such as aortic dilatation, aneurysm, and dissection. The morphology of the BAV varies with the presence and position of the raphe and is associated with the type of valvular dysfunction and aortopathy. Therefore, accurate diagnosis and effective treatment at an early stage are essential to prevent complications in patients with BAV. This pictorial essay highlights the characteristics of BAV and its related congenital cardiovascular malformations, valvular dysfunction, aortopathy, and other rare cardiac complications using multimodal imaging.