Our study aims to evaluate the cardiac status of patients with thalassemia major and intermedia to determine those with left ventricular diastolic dysfunction using pulsed Doppler echocardiography at an early stage of cardiac involvement, to investigate the effect of chelation therapy on cardiac involvement. We have studied 15 patients with thalassemia intermedia (5 to 23 years of age) and 50 patients with thalassemia major (1 to 21 years of age) on hypertransfusion program with a control group of 65 healthy children of the same age and sex. Hematologic findings (the initial age of transfusion therapy, total transfusion amount, serum ferritin level) were recorded. Cardiac evaluation of 36 of the 65 patients with thalassemia was made both 2 hours before and 4 hours after transfusion therapy. With M-mode echocardiography, left ventricular systolic function and with pulsed Doppler echocardiography left ventricular diastolic filling patterns were recorded. Measurements showed that in respect to the control group, patients with thalassemia major and intermedia had a restrictive pattern of left ventricular diastolic filling without evidence of systolic dysfunction (increased peak early diastolic flow velocity (E) and decreased peak late (atrial) diastolic flow velocity (A) and increased ratio of these two velocities (E/A) (p<0.05). Six (40 %) of 15 patients with thalassemia intermedia and 16 (32 %) of 50 patients with tahalassemia major had diastolic dysfunction. It was also shown that a significant difference existed in left ventricular diastolic filling measurements between patients having serum ferritin level below and above 2000 ng/ml. The patients who had serum ferritin level above 2000 ng/ml showed more significant diastolic dysfunction.
Keywords: Thalassemia, echocardiography, diastolic functionCopyright © 2024 Archives of the Turkish Society of Cardiology