Electro Cardiographic Assessment in Patients with Transfusion Dependant Beta Thalassemia Major Using Stress ECG and 24hrs ECG Monitoring

Abstract

Thalassemia is the most common monogenic disorder causing decreased globin, a protein composition of hemoglobin synthesis. By clinical manifestation, beta-thalassemia major (TM) is the most severe form apart from hemoglobin Bart’s disease, which is always fatal. TM patients require intensive blood transfusions due to severe anemi from ineffective erythropoiesis hence, the inevitably pursuant complications are from iron excess in various organs such as the heart, liver and pancreas. Although the heart is not the first target organ, cardiac iron overload or iron overload cardiomyopathy is regarded as the most serious condition. Iron overload represents the most important cause of mortality and morbility in patients affected by thalassemia major even patients treated with periodical transfusions and intensive chelation suffer at a certain point from cardiomyopathy and of arrhythmic symptoms, most frequently ventricular, which still constitute a recurrent complication in the natural development of the disease. Despite adequate iron chelation, myocardial function is still worsening due to iron deposition, fibrosis, and chronic anemia. So the aim of the present study to investigate electrocardiographic and echocardiographic markers in patients with β-TM for the early prediction of arrhythmia risk. Additionally this study investigated the effect of various iron chelator therapy on different cardiac parameters Results from the current thalassemic cohort showed that 64 % of the screened cohort developed arrthymia. 64% (29) of patients developed frequent PVC, 22 %, (10) of all patients developed SVT and PVC. The patients that developed arrhythmia had significantly higher serum ferritin with a mean 4347.50±1614.87 in patients with arrhythmia compared to 2286.71±1229.72 in other patients. Patients with arrhythmia had significantly higher values of left atrial diameter (p< 0.05), IVSD (p< 0.05) and LVPWd (p< 0.05).