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.
Kovacevic
Miljenko
M
Department of Cardiac Surgery, University Hospital Rijeka, Croatia. m.kovacevic@inet.hr
Simic
Ognjen
O
Medved
Igor
I
Lucin
Ksenija
K
Padovan
Marijan
M
D000474:Alkaptonuria / Q000150:complications / Q000175:diagnosis* / Q000473:pathology; D001011:Aorta / Q000473:pathology*; D001021:Aortic Valve / Q000473:pathology*; D001024:Aortic Valve Stenosis / Q000209:etiology / Q000473:pathology / Q000601:surgery; D002315:Cardiopulmonary Bypass; D001026:Coronary Artery Bypass; D023921:Coronary Stenosis / Q000209:etiology / Q000473:pathology / Q000601:surgery; D019918:Heart Valve Prosthesis Implantation; D006801:Humans; D008297:Male; D008875:Middle Aged; D009794:Ochronosis / Q000209:etiology / Q000473:pathology* / Q000601:surgery