Despite the advances in the management of thalassemia major, heart disease remains the leading cause of mortality in patients afflicted with this disorder. Cardiac involvement in thalassemia encompasses a spectrum of disorders including myocardial dysfunction, arrhythmias, pulmonary hypertension, and peripheral vascular disease. Although cardiac siderosis (accumulation of iron in cardiac myocytes) as a consequence of repeated blood transfusions is deemed to be the main etiologic factor for myocardial dysfunction in transfusion-dependent patients, the significance of other pathophysiologic mechanisms is being increasingly recognized especially in non-transfusion dependent patients. Management of cardiac complications in thalassemia major hinges on the treatment of the underlying pathophysiology, which often is unmitigated iron overload. The prevalence and predictors of cardiac complications in 'ex-thalassaemics' [thalassaemic patients undergoing allogeneic hematopoietic stem cell transplantation (HSCT) is unknown at present. In this review, we look at the pathogenesis of cardiac involvement in patients with beta-thalassemia major, the advances in the management of these patients and the future prospects.
Keywords: Beta-Thalassaemia; Cardiomyopathy; siderosis; treatment.