Review
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World J Cardiol. Aug 26, 2013; 5(8): 270-279
Published online Aug 26, 2013. doi: 10.4330/wjc.v5.i8.270
Early detection of cardiac involvement in thalassemia: From bench to bedside perspective
Nut Koonrungsesomboon, Siriporn C Chattipakorn, Suthat Fucharoen, Nipon Chattipakorn
Nut Koonrungsesomboon, Siriporn C Chattipakorn, Nipon Chattipakorn, Cardiac Electrophysiology Research and Training Center, Department of Physiology, Faculty of Medicine, Chiang Mai University, Chiang Mai 50200, Thailand
Nut Koonrungsesomboon, Department of Pharmacology, Faculty of Medicine, Chiang Mai University, Chiang Mai 50200, Thailand
Siriporn C Chattipakorn, Department of Oral Biology and Diagnostic Science, Faculty of Dentistry, Chiang Mai University, Chiang Mai 50200, Thailand
Suthat Fucharoen, Thalassemia Research Center, Institute of Molecular Medicine, Mahidol University, Bangkok 3310, Thailand
Author contributions: Koonrungsesomboon N, Chattipakorn SC, Fucharoen S and Chattipakorn N solely contributed to this paper.
Supported by Thailand Research Fund grants RTA5580006 and BRG5480003
Correspondence to: Nipon Chattipakorn, MD, PhD, Cardiac Electrophysiology Research and Training Center, Department of Physiology, Faculty of Medicine, Chiang Mai University, Huay Kaew Road, Tambon Suthep, Muang District, Chiang Mai 50200, Thailand. nchattip@gmail.com
Telephone: +66-53-945329 Fax: +66-53-945329
Received: July 9, 2013
Revised: July 31, 2013
Accepted: August 5, 2013
Published online: August 26, 2013
Abstract

Myocardial siderosis is known as the major cause of death in thalassemia major (TM) patients since it can lead to iron overload cardiomyopathy. Although this condition can be prevented if timely effective intensive chelation is given to patients, the mortality rate of iron overload cardiomyopathy still remains high due to late detection of this condition. Various direct and indirect methods of iron assessment, including serum ferritin level, echocardiogram, non-transferrin-bound iron, cardiac magnetic resonance T2*, heart rate variability, and liver biopsy and myocardial biopsy, have been proposed for early detection of cardiac iron overload in TM patients. However, controversial evidence and limitations of their use in clinical practice exist. In this review article, all of these iron assessment methods that have been proposed or used to directly or indirectly determine the cardiac iron status in TM reported from both basic and clinical studies are comprehensively summarized and presented. Since there has been growing evidence in the past decades that cardiac magnetic resonance imaging as well as cardiac autonomic status known as the heart rate variability can provide early detection of cardiac involvement in TM patients, these two methods are also presented and discussed. The existing controversy regarding the assessment of cardiac involvement in thalassemia is also discussed.

Keywords: Thalassemia, Iron overload, Cardiomyopathy, Serum ferritin, Heart rate variability, Magnetic resonance, Non-transferrin-bound iron

Core tip: The mortality of thalassemia major (TM) patients due to iron overload cardiomyopathy is still high even though it can be prevented with effective chelation. The role of reliable methods to determine cardiac iron status is very important in order to give a timely effective treatment. This review article provides a comprehensive summary and discussion of various iron assessment methods as well as their existing controversy for use from both basic and clinical reports that have been proposed or used to directly or indirectly determine the cardiac iron status in TM.