Ochronosis of the aortic valve and aorta

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.