Stenosis of the bicuspid aortic valve with systemic lupus erythematosus.

We herein present a rare case of severe aortic valve stenosis with a bicuspid valve in a patient with systemic lupus erythematosus. The symptoms resulted from aortic valve stenosis, such as chest pain, dyspnea and syncope, which subsided after the insertion of an intra-aortic balloon pump. Thereafter, a calcified bicuspid aortic valve was successfully replaced with a mechanical valve. The pathological findings of the resected valve included irregular fibrotic thickening and marked calcification without any vegetation or thrombus formation. The efficacy of an intra-aortic balloon pump for the relief of symptoms associated with severe aortic valve stenosis indicates its usefulness for such critically ill patients prior to undergoing valvular surgery.