The bicuspid aortic valve (BAV) is the most common congenital cardiac anomaly and is present in 2–3% of the general population. As compared to the normal tricuspid aortic valve (TAV) which consists of three leaflets, the most prevalent type-I BAV morphology forms with two as a result of left-/right-coronary cusp fusion. While the BAV anatomy may not intrinsically hamper valvular function, it is associated with a spectrum of secondary aortopathy such as aortic dilation and subsequent dissection. The dilation and thinning of the ascending aorta downstream of a BAV is marked by structural wall abnormalities including smooth muscle cell depletion, elastic fiber degeneration and abnormal extracellular matrix remodeling, which localize to the convexity of the aortic wall.

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