ROLE OF CARDIAC MRI (1.5T)IN THALASSAEMIA

Abstract

Summary T halassemia is one of the most common single gene disorders and is widely distributed in the Mediterranean region . Beta-thalassemia is an inherited hemoglobin disorder resulting from either homozygous or double heterozygous inheritance of two abnormal genes from the β-globin locus, leading to defective synthesis of the β-globin chain with the resultant chronic dyserythro-poietic anemia and subsequently hemolytic anemia. Thalassemia is an autosomal recessive anemia that requires lifelong blood transfusions and medical care to maintain adequate hemoglobin levels.. The transfusions that these patients receive are life sustaining; however chronic transfusions result in iron overload in multiple organs, including the heart and liver, which can lead to failure of these organs. Heart failure due to iron overload is the most common cause of death in `thalassemia patients. Fortunately, the iron overload can be successfully treated with iron chelation therapy.Recent developments in the treatment of the iron overload with improved chelation therapy have dramatically increased the expected lifespan of patients with `thalassemia from less than twenty years in the 1960s to greater than forty years today.