Cardiac manifestations of ochronosis

A 68-year-old woman with ochronosis was admitted with congestive heart failure. A typical ejection murmur of aortic stenosis was ausculated and was documented on a phonocardiogram. The patient subsequently died, and at autopsy extensive deposition of ochronotic pigment was found on the aortic valve. This valve had no other anatomic abnormalities. Thus ochronosis should be considered in the differential diagnosis of aortic stenosis.