Key result
Double valve replacement reveals rare ochronotic involvement of aortic and mitral valves.
Why the study?
Ochronosis can cause valvular heart disease, but cardiovascular abnormalities are less frequently reported than other manifestations.
Case Report (n=1)
Alerts clinicians to rare ochronotic valvulopathy in older surgical patients; leaves open questions on prevalence and systematic screening in alkaptonuria.
Ochronosis, an autosomal recessive metabolic disorder, causes an excess of homogentisic acid that results in adverse pigmentation, calcification, and inflammation of cartilaginous and other tissues. Cardiovascular abnormalities are less frequently reported than are other manifestations. In rare cases, ochronosis can cause valvular heart disease. We report the case of a 72-year-old man with aortic stenosis and mitral insufficiency who was diagnosed with ochronosis while undergoing surgical aortic and mitral valve replacement. We discuss the history and surgical management of alkaptonuric ochronosis.
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Atalay et al. (2015) conducted a case report in Ochronosis with aortic stenosis and mitral insufficiency (n=1). Surgical aortic and mitral valve replacement was evaluated. Surgical aortic and mitral valve replacement revealed rare ochronotic involvement of the aortic and mitral valves in a 72-year-old man.
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