Complete repair of tetralogy associated with sickle cell anemia and G-6-PD deficiency.

The case of a 5-year-old black girl with sickle cell anemia and G-6-PD deficiency is described. The child underwent successful repair of tetralogy of Fallot. Open-heart surgery can be safely performed on these patients if certain guidelines are observed, particularly the avoidance of hypoxia, hypothermia, acidosis, and dehydration. The patients should be prepared for the operation with transfusion of normal red cells. Routine preoperative testing for the above hemoglobinopathologic conditions is urged. The pertinent literature is reviewed.