ABSTRACT
A 17-year-old girl with drepanocytic (sickle-cell) anemia who was being treated with hydroxyurea and periodic blood transfusions through a Hickman-type catheter was admitted for periodic episodes of fever. Blood cultures were positive for methicillin-sensitive Staphylococcus aureus. Massive right atrial thrombosis with pulmonary embolism and bacterial endocarditis were detected by computed tomography. Surgery with a beating heart and cardiopulmonary bypass was undertaken. Drepanocytic anemia in individuals homozygous for hemoglobin S is a rare condition in Spain but we are beginning to see a few cases, in which management during anesthesia will be more complicated. High-risk surgery can be carried out in these patients without adverse events if the anesthesiologist is guided by a complete blood workup and takes precautions during and after surgery to control hydration, oxygenation, temperature, and the acid-base balance.