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Severe aortic stenosis with alkaptonuria: a case report
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  • Ahmet Arnaz,
  • Vusal MAHMUDOV,
  • abdullah dogan,
  • Dilek ALTUN
Ahmet Arnaz
Saint Francis Hospital The Heart Center Department of Cardiovascular Surgery
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Ogilvy Istanbul
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abdullah dogan
Ogilvy Istanbul
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University of Colorado Denver Department of Anesthesiology

Corresponding Author:[email protected]

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Alkaptonuria, a rare metabolic disease, is caused by a genetic deficiency of the homogentisic acid oxidase enzyme involved in phenylalanine and tyrosine metabolism. Ochronotic arthropathy and cardiovascular involvement, which manifests itself in 4-6 decades as a result of homogentisic acid accumulation in cartilage and connective tissue, are the most important causes of mortality and morbidity. We present a 75-year-old case with a diagnosis of severe aortic stenosis who was diagnosed with alkaptonuria after the operation. Severe calcification and pigmentation were observed in the patient's aortic valve leaflets and at the ascending aortic wall