Hematoma of a congenitally bicuspid aortic valve in a patient with polycythemia vera and the antiphospholipid antibody syndrome.

We present a novel case of a hematoma involving a congenitally bicuspid aortic valve. The patient was a 47-year-old woman with polycythemia vera and antiphospholipid antibody syndrome. She was treated with anagrelide for thrombocytosis. The patient developed a valvular hematoma which caused clinically significant aortic stenosis and regurgitation. Consequently, the patient underwent surgery with insertion of a porcine bioprosthesis. Eighteen months postoperatively the patient developed a large prosthetic thrombus with severe aortic stenosis requiring reoperation.