A 62-year-old male developed aortic valve stenosis. Surgery was performed.
Thank you to Dr. Joseph Maleszewski for sharing this case.
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Ochronosis of the aortic valve is a manifestation of chronic alkaptonuira that is the caused by deposition of bluish-black pigment consisting of oxidized homogentisic acid. The metabolic derangement is the result of a recessive trait. Pigment can deposited in intervertebral discs, cartilage, skin and cardiac valves (particularly the aortic and mitral valves). Heavy calcification is often seen in association with the pigment (as in this case) and there is a general belief that the pigment hastens development of both atherosclerosis and calcification. This may, in part, explain the relatively young age (60-years-old) that this patient developed hemodynamically significant (tricuspid) aortic valve stenosis, which is usually seen in the 8th or 9th decades of life.
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