Electrocardiographic Presentation, Cardiac Arrhythmias, and Their Management in β‐Thalassemia Major Patients. Issue 4 (20th June 2016)

• Record Type: Journal Article
• Authors: Russo, Vincenzo; Rago, Anna; Papa, Andrea Antonio; Nigro, Gerardo
• Is Part Of: Annals of noninvasive electrocardiology. Volume 21:Issue 4(2016:Jul.)
• Page Start: 335
• Page End: 342
• Publication Date: 2016-06-20
• Subjects: beta‐thalassemia -- electrocardiography -- arrhythmias -- heart -- cardiac function -- thalassemic cardiomyopathy -- miocardial iron overload; Electrocardiography -- Periodicals; Arrhythmia -- Periodicals; 616.1207547
• DOI: 10.1111/anec.12389
• Languages: English
• ISSNs: 1082-720X

Abstract

Abstract : Beta‐thalassemia major (β‐TM) is a genetic hemoglobin disorder characterized by an absent synthesis of globin chains that are essential for hemoglobin formation, causing chronic hemolytic anemia. Clinical management of thalassemia major consists in regular long‐life red blood cell transfusions and iron chelation therapy to remove iron introduced in excess with transfusions. Iron deposition in combination with inflammatory and immunogenic factors is involved in the pathophysiology of cardiac dysfunction in these patients. Heart failure and arrhythmias, caused by myocardial siderosis, are the most important life‐limiting complications of iron overload in beta‐thalassemia patients. Cardiac complications are responsible for 71% of global death in the beta‐thalassemia major patients. The aim of this review was to describe the most frequent electrocardiographic abnormalities and arrhythmias observed in β‐TM patients, analyzing their prognostic impact and current treatment strategies.