Cardiac ochronosis with severe aortic and mitral valve stenosis: a challenge case

Ochronosis is a hereditary disorder characterized by black pigmentation in tissues. Cardiac ochronosis is most commonly associated with aortic stenosis, while mitral stenosis is a less frequently observed involvement. In this case presentation, we will discuss ochronosis diagnosed during open heart surgery in a patient who had not been previously diagnosed. Our patient underwent surgery for aortic valve and mitral valve replacement. The aortic root, mitral valve, and aortic-mitral curtain were found to be calcified, and a mandatory Commando procedure was performed. The coronary buttons could not be mobilized, and the Cabrol procedure was applied. In cases of ochronosis, it is a fact that decalcification can lead to significant complications, and the involvement of the aortic-mitral curtain must also be considered by the surgeon.