Abstract

Cardiac complications such as heart failure and arrhythmias caused by "iron-induced" cardiomyopathy are considered as the primary cause of death in the patients with β-thalassemia major. The aim of this study was to evaluate electrocardiography, echocardiography according cardiac T2* and ferritin findings of patients followed-up for β-thalassemia major, and to investigate the importance of these findings for early detection of cardiac complications. The study included 41 patients and 25 healthy individuals with matched age and gender. The cardiac T2* results revealed a cardiac iron load below 20ms in 12 (29.27%) patients, and above 20ms in 29 (70.73%) patients. All electrocardiography parameters significantly increased in the patient group when compared to the control group (p < 0.05). All parameters except P wave segment in electrocardiography and T peak-end/QT ratio were significantly higher in the group with cardiac T2* < 20ms than the group with cardiac T2* > 20ms (p < 0.05). Intraventricular septum thickness, left ventricular posterior wall thickness, left ventricular mass and left ventricular mass index detected by echocardiography were significantly higher in the group with T2* < 20ms (p < 0.05). Electrocardiography, echocardiography, cardiac T2* and ferritin findings should be carefully evaluated in these patients in order to detect early signs of cardiac complications.

Highlights

  • Thalassemia syndromes are haemoglobin disorders characterized by decreased synthesis or failure to synthesize globin chains in the haemoglobin structure

  • Hemoglobin level was lower in the patient group than the control group; platelet count, white blood cells (WBC) count, uric acid level, total bilirubin level and ferritin levels were higher than the control group (p = 0.001, p = 0.001, p = 0.021, p = 0.017, p = 0.001 and p = 0.008, respectively)

  • A negative correlation was found between the values obtained with left ventricular mass (LVM) and left ventricular mass index (LVMI) in ECHO and the cardiac T2 * score. These results indicate that both cardiac T2* and serum ferritin level are important to monitor the development of cardiac complications

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Summary

Introduction

Thalassemia syndromes are haemoglobin disorders characterized by decreased synthesis or failure to synthesize globin chains in the haemoglobin structure. If there is a disorder in the alpha chain production of haemoglobin, alpha-thalassemia appears; and if there is a production disorder in the beta chain, betathalassemia major appears (β-TM). Life expectancy in patients who do not receive adequate transfusion and chelation therapy is less than 5 years. These patients usually die due to anaemia, heart failure, liver failure [3,4,5]. Cardiac complications such as heart failure and arrhythmias caused by "iron-induced" cardiomyopathy are considered as the primary cause of death in the patients with β-TM [5,6,7]

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