In-vivo Aortic Valve of a Patient with Alkaptonuria

A 73-year-old man underwent surgery due to severe aortic valve disease. The aortic valve cusps and the wall of the ascending aorta, which are usually pearly white, had a dark discoloration (ochronosis). He also presented typical dark spots in his sclerae and ears and suffered from severe osteoarthritis of weight-bearing joints, having undergone bilateral knee and hip replacements years before. Histology of the aortic valve cusps confirmed the suspicion of cardiac ochronosis.

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