The term bicuspid aortic valve is actually a misnomer; a raphe caused by commissural fusion of two leaflets usually exists. The valve is often dysplastic, with thickening and rolling of the leaflets.
The predominant pathophysiology results from a mildly obstructed nonlaminar (disturbed) flow across the abnormal valve. A left ventricle-to-aorta pressure gradient of variable severity occurs, setting the stage for the inevitable deterioration of the valve with long-term calcium deposition and progressive stenosis or regurgitation. In a study of young adults with aortic stenosis who presented for surgery between the ages of 21 and 38, diastolic murmurs were audible in 75%, and calcification was found at surgery in 75%. The valve is also at risk for endocarditis, which can lead to early destruction and regurgitation.
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