Sickle cell anemia and mitral valve replacement:Case Report

Abstract
An 8-year-old black boy with sicle cell disease and severe hemolytic anemia crisis (95% hemoglobin S) also had mitral incompetence due to rheumatic valve disease. A 27 mm monostrut Björk–Shiley valve prosthesis was implanted after partial exchange transfusions had reduced the hemoglobin S to <40%. High-flow normothermic perfusion was used during extracorporeal circulation, with care taken to avoid hypoxia and acidosis. Postoperative recovery was uneventful.