Bicuspid aortic valve (BAV) is the most common adult congenital heart disease, with a prevalence as high as 2%.1 Almost 50% of aortic valve stenosis is attributable to BAV.1-3 Instead of the typical 3 leaflets in the normal heart, BAV is characterized by 2 unequal-sized leaflets that create flow disturbance. These changes in valve structure lead to flow turbulence that leads to aortic valve stenosis, regurgitation, or root dilatation.2 Systematic screening is required for at-risk populations because early recognition drives appropriate and timely intervention.BAV morphology has different variants. The morphology determines the natural history, clinical presentation, and long-term prognosis. The most common variant of BAV is fusion of the right and left aortic valve leaflets. This variant constitutes almost 70% of all BAV cases and is associated with aortic coarctation. The second most common variant is fusion of the right and noncoronary leaflets. This variant is seen in 28% of BAV cases and is associated with higher rates of aortic valve complications (stenosis and regurgitation) and less association with aortic root dilatation.4 The third variant of BAV is fusion of the left and noncoronary leaflets (1.4% of BAV cases). This variant is also associated with aortic valve structural complication but is less common than the other 2 variants (Figure).4.
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