Early left ventricular dysfunction and chelation therapy in thalassemia major.
Exercise radionuclide angiography was used to assess the incidence of subclinical abnormalities of left ventricular function in 23 asymptomatic patients with thalassemia major. Left ventricular ejection fraction (LVEF) at rest was normal in 18 patients and abnormal in 5. A normal LVEF response during exercise was shown in only 5 of the patients with normal resting left ventricular function. A normal response during exercise occurred more often in those patients who had received a smaller transfusional iron load and had a lower serum ferritin level (p less than 0.05). Twelve of the twenty-three patients were receiving chelation therapy with subcutaneous deferoxamine. Ejection fraction at rest was normal in 11 of these patients. During exercise a normal ventricular response was shown in 4 patients. After 1 year of intensive chelation therapy in these 12 patients, left ventricular function was reassessed. A normal exercise response was seen in an additional 4 patients; 3 of these showed an increase in peak exercise LVEF, and in the remaining patient no change of peak exercise LVEF was shown. The response during exercise was unchanged in 3 patients and had deteriorated in 1 patient.
D000293:Adolescent; D000328:Adult; D001803:Blood Transfusion; D002648:Child; D003331:Coronary Vessels / Q000000981:diagnostic imaging; D003676:Deferoxamine / Q000627:therapeutic use*; D005293:Ferritins / Q000097:blood; D006352:Heart Ventricles / Q000000981:diagnostic imaging / Q000503:physiopathology*; D006801:Humans; D007501:Iron / Q000008:administration & dosage / Q000378:metabolism; D005082:Physical Exertion; D011336:Probability; D011877:Radionuclide Imaging; D013318:Stroke Volume; D013789:Thalassemia / Q000097:blood / Q000188:drug therapy* / Q000503:physiopathology
D005293:Ferritins; D007501:Iron; D003676:Deferoxamine