Although bicuspid aortic valve occurs in an estimated 1% of adults and mitral valve prolapse in an estimated 5% of adults, occurrence of the 2 in the same patient is infrequent. During examination of operatively excised aortic and mitral valves because of dysfunction (stenosis and/or regurgitation), we encountered 16 patients who had congenitally bicuspid aortic valves associated with various types of dysfunctioning mitral valves. Eleven of the 16 patients had aortic stenosis (AS): 5 of them also had mitral stenosis, of rheumatic origin in 4 and secondary to mitral annular calcium in 1; the other 6 with aortic stenosis had pure mitral regurgitation (MR) secondary to mitral valve prolapse in 3, to ischemia in 2, and to unclear origin in 1. Of the 5 patients with pure aortic regurgitation, each also had pure mitral regurgitation: in 1 secondary to mitral valve prolapse and in 4 secondary to infective endocarditis. In conclusion, various types of mitral dysfunction severe enough to warrant mitral valve replacement occur in patients with bicuspid aortic valves. A proper search for mitral valve dysfunction in patients with bicuspid aortic valves appears warranted.

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