Abstract
Peri-operative management of cyanotic congenital heart disease in a patient of sickle cell disease (SCD) can be challenging. We report a case of Tetralogy of Fallot and homozygous SCD with history of multiple blood transfusions and sickle cell crises who underwent intracardiac repair. Hemoglobin S level was reduced from 75% pre-operative to 21.8% postoperative with a combination of pre-operative blood transfusion, intraoperative exchange transfusion, and normothermic cardiopulmonary bypass (CPB). Pre-operative optimization and safe intraoperative conduct were essential to avoid sickling crises.