Cardiac Iron Overload – Role of Cardiac Magnetic Resonance Imaging (MRI)

Cardiac Iron Overload – Role of Cardiac Magnetic Resonance Imaging (MRI)

Thalassemia is an inherited autosomal recessive disorder resulting in abnormality of adult haemoglobin (HbA). This results in poor oxygen carrying capacity of haemoglobin and reduced life of circulating red blood cells. These patients thus develop severe anaemia. B-thalassemia presents as major, intermediate or minor variants. B-thalassemia major results in severe anaemia and patients are usually symptomatic within the first two years of life. These patients present with extreme fatigue, failure to thrive, poor muscle growth, jaundice, hepatosplenomegaly and skeletal abnormalities due to expanding bone marrow. Patients with Beta thalassemia major need repeated blood transfusions for life usually starting from the first one or two years of age. There is an increase in the red blood cell (RBC) turnover due to the severe anaemia prompting the marrow to increase the RBC generation and due to rapid RBC loss due to defective haemoglobin. Regular, almost monthly, blood transfusions are needed to counter the anaemia in most thalassemia major cases. This results in accumulation of iron from the dying RBCs which accumulates in all body tissues, notably, the liver, spleen, heart and endocrine glands. Cardiac failure, rhythm disturbances and sudden cardiac death are some of the common causes of mortality resulting from cardiac iron deposition.