Abstract
Bicuspid aortic valve (BAV) is the most common congenital heart disease with marked heterogeneity in many aspects. Fusion patterns of the aortic cusp are quite variable with different type and severity of valvular dysfunction. Moreover, non-valvular cardiovascular abnormalities are associated with BAV. Among them, aortic aneurysm/dissection is the most serious clinical condition with variable patterns of segmental aortic dilatation. Potential association between BAV phenotype and valvulopathy or aortopathy has been suggested, but needs to be tested further. A lack of long-term outcome data at this moment is responsible for unresolved debate regarding appropriate management of patients with BAV, specifically to prevent development of aortic dissection. Long-term follow-up data of a well-characterized cohort or registry based on standardized classification of BAV phenotype and aortopathy are necessary for evidence-based medical practice. Advanced imaging techniques such as computed tomography or magnetic resonance imaging offer better opportunities for accurate phenotype classification and imaging specialists should play a central role to establish a collaborative multicenter cohort or registry.