Alkaptonuric ochronosis of congenital bicuspid aortic valve—a case report

Aortic valve diseases are most commonly caused by degenerative changes, congenital malformation and rheumatic affection of valves. A very rare cause is the deposition of foreign material in the valvular tissues as a result of alkaptonuria. In this case report, we present a case of alkaptonuric ochronosis of congenital bicuspid aortic valve, presented with severe aortic valve stenosis and treated with aortic valve replacement surgery.