Ochronosis of the Aortic Valve and Aorta

Miljenko Kovacevic, Ognjen Simic, Igor Medved, Ksenija Lucin, Marijan Padovan
Department of Cardiac Surgery, University Hospital Rijeka, Department of Pathology, School of Medicine, Rijeka, Department of Cardiology, University Hospital Rijeka, Croatia
 

A 64-year-old male patient with unknown alkaptonuria and severe aortic stenosis and ischemic heart disease was admitted to the authors’ institution for elective surgery. The patient underwent aortic valve replacement with a 25-mm aortic valve (ATS Medical, Inc.) and single venous aortocoronary artery bypass grafting for a right coronary artery. Aortotomy

revealed typical ochronotic pigmentation of a severely calcified aortic valve and aortic intima. A diagnosis of alkaptonuria was confirmed by evidence of homogentisic acid in the patient’s urine, together with histopathological analysis.

The Journal of Heart Valve Disease 2006;15:730-732
 
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