Tetralogy of fallot repair in a patient with β-thalassemia major

Abstract

Tetralogy of Fallot (TOF), the most common cyanotic congenital heart disease, is commonly associated with polycythemia to compensate for the severe tissue hypoxemia present in these patients. β-Thalassemia major is usually associated with severe anemia secondary to the deficiency of normal hemoglobin (Hb-A) as well as increased red cell destruction and ineffective erythropoiesis. The combination of TOF and β-thalassemia major can result in profound tissue hypoxemia. The longevity of patients with β-thalassemia major is significantly improved with the modern therapy of regular red cell transfusions.