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A case with an exceptional form of congenitally bicuspid aortic valve is described. A fibrous strand connected to the supra-aortic ridge supported the conjoined cusp, and severe pure aortic regurgitation was present although the fibrous strand remained intact. Aortic valve replacement was successfully performed.

An exceptional form of congenitally bicuspid aortic valve resulting in pure aortic regurgitation.