Perioperative anaesthetic management of an elderly high-risk male with cardiac ochronosis undergoing aortic valve replacement with coronary artery bypass graft: A case report.

Abstract

Alkaptonuria is a rare autosomal recessive congenital disorder of metabolism that affects 1 in 250,000 live births. It manifests as ochronosis and degenerative arthritis due to the accumulation of homogentistic acid in cartilage and heart valves along with precipitation of renal, salivary, pancreatic and gall bladder calculi. It is noted to cause cardiac valve stenosis and regurgitation secondary to calcification leading to cardiac failure in 10% of patients. Through this report, we present a successful perioperative anaesthetic management of a 74-year-old man with cardiac ochronosis, who underwent an aortic valve replacement with coronary artery bypass graft surgery at our centre.