Narrowing of the dark valve: aortic stenosis in alkaptonuria.

The commonest cardiac pathology in patients with alkaptonuria is aortic stenosis. Patients with alkaptonuria and aortic stenosis may remain asymptomatic until the 6th decade. Surgeons may have to deal with per-operative difficulties as alkaptonuria is a systemic disease. Proper preoperative planning is important. The mechanical valve prosthesis is advisable in a patient with alkaptonuria and aortic stenosis considering disease pathophysiology. We report a 70-year-old male diagnosed with alkaptonuria and aortic stenosis, who underwent aortic valve (mechanical valve prosthesis) and ascending aorta replacement. © Indian Association of Cardiovascular-Thoracic Surgeons 2020.