Cardiac abnormalities in patients with beta thalassemia

Abstract

Background: Thalaseemias are a group of chronic, inherited anemias characterized by defective hemoglobin synthesis and ineffective erythropoiesis. The presence of chronic anemia and transfusional iron overload exacerbates congestive cardiac failure. Cardiac disease remains the major cause of death in thalassemia major. The objective was to assess cardiac status in patients with transfusion dependent thalassemia. Methods: A prospective observational study was conducted at thalassemia Centre in tertiary care institute. 32 patients with transfusion dependent beta thalassemia were enrolled. A detailed history, clinical examination and cardiac investigations were conducted in enrolled patients. Results: Symptoms and signs of cardiac disease were present in 5 (15.6%) patients. Cardiomegaly was observed on chest radiography in 23 (71.87%) patients. Only 3 (9.37%) patients had electrocardiographic abnormalities. Left Ventricular Ejection Fraction (LVEF) on 2-D echocardiography was ≥60% in all the 32 patients. Diastolic dysfunction and restrictive physiology on Doppler echocardiography was present in 19 (59.37%) patients and absent in remaining 13 (40.63%) patients. Conclusions: Diastolic dysfunction is an early sign of myocardial dysfunction in patients with beta thalassemia.