In this review, we summarize previously reported case reports (n=66) in which the presence of ochronotic pigment was found in one or more cardiovascular structures either at necropsy or after operative excision of a cardiac valve or portions of arteries or both. As illustration, we describe black pigment in operatively excised aortic valves and aorta in 2 patients, both probably examples of secondary ochronosis. Ochronosis appears to have fascinated a number of prominent historical figures in medicine, and this review also summarizes their important contributions to this topic.
Keywords: Alkaptonuria; Aortic valve; Minocycline; Ochronosis.
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