CARDIAC INVOLVEMENT IN Beta-THALASSAEMIA: Current treatment strategies.

CARDIAC INVOLVEMENT IN Beta-THALASSAEMIA: Current treatment strategies. Postgrad Med. 2019 Apr 19;: Authors: Paul A, Thomson VS, Refat M, Al-Rawahi B, Taher A, Nadar SK Abstract Despite the advances in the management of thalassaemia major, heart disease remains the leading cause of mortality in patients afflicted with this disorder. Cardiac involvement in thalassaemia 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 aetiologic 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 thalassaemia 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. PMID: 31002266 [PubMed - as supplied by publish...
Source: Postgraduate Medicine - Category: Internal Medicine Tags: Postgrad Med Source Type: research