MITRAL-VALVE RECONSTRUCTION IN SICKLE-CELL DISEASE

As survival improves in patients with sickle cell anemia, the prospect s of performing cardiac surgical procedures on older patients with thi s genetic defect increase. We describe the successful management of a 52-year-old patient with sickle cell disease (homozygous for hemoglobi n S) and a history of multiple sickle crisis undergoing cardiopulmonar y bypass for mitral valve repair. Preoperative partial exchange transf usion followed by total exchange transfusion at the time of operation was performed to reduce the level of hemoglobin S to 5.4% during bypas s. Other management strategies included high-flow normothermic bypass with aortic crossclamping, topical hypothermia, and cold crystalloid c ardioplegia.