Open AccessOchronotic Involvement of the Aortic and Mitral Valves in a 72-Year-Old Man
Author(s) -
Atakan Atalay,
Uğur Göçen,
Yüksel Baştürk,
Erkan Kozanoğlu,
Hafize Yalınız
Publication year - 2015
Publication title -
texas heart institute journal
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.373
H-Index - 53
eISSN - 1526-6702
pISSN - 0730-2347
DOI - 10.14503/thij-14-4138
Subject(s) - ochronosis , medicine , alkaptonuria , homogentisic acid , stenosis , calcification , cardiology , calcinosis , mitral valve , valvular heart disease , mitral valve replacement , surgery , dermatology , biochemistry , chemistry
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.