Özet:

Introduction and Purpose: Talasemias are hereditary diseases resulting from defects in globulin chain synthesis. In these patients, heart disfunction, which is a result of iron accumulation despite shellation treatment, is the cause of significant mortality and morbidity. Causes of cardiovascular dysfunction are ventricular dilatation and iron accumulation associated with chronic hemolytic anemia and ventricular compression dysfunction and increased cardiac output. The aim of this study is to study the cardiovascular functions in patients diagnosed with major beta talasemia receiving oral shellation therapy with Doppler ecocardiography. Method and Requirements: In this study, between January and September 2014, beta talasemia was diagnosed major, 49 patients between 2-16 years of age and the same number of 49 healthy patients were studied as a control group. All patients were subjected to an electrocardiographic and ecocardiographic examination after a detailed physical examination. The average ferritin value in the patient group was 1275+904 ng/ml. Compared to the ecocardiographic findings of the groups, the myocardial performance index in the patient group was statistically high (p=0.017). In addition, the patient group has diagnosed type 1 diastolic dysfunction in the left ventricle, while left ventricle sistolic, right ventricle sistolic and diastolic dysfunction are not observed. RELATED AND RESULTATED: In patients with talasemia major diagnosed, iron accumulation in many systems, primarily the cardiovascular system, has a toxic effect. Although the prognosis of the disease is correct with iron shellation treatment, high morality and morbidity remain a major problem. Our study shows that patients with beta-talasemia major develops subclinical left ventricular diastolic dysfunction in the early stages, but their right ventricular functions and left ventricular sistolic functions do not disrupt.

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