[The cardiac changes in thalassemia major: their assessment by Doppler echocardiography]

Dilated cardiomyopathy with impaired left ventricular function is the most common cause of death in patients (pts) with Thalassemia Major (TM) undergoing multiple transfusions. To assess the cardiac status in a young population with TM, 25 pts (mean age 15.8 +/- 5.7 years) and 25 controls (sex and age matched), underwent clinical, echocardiographic and Doppler evaluation. Thirteen pts who received a correct chelation therapy had serum Ferritin (F) below, and nine pts up to 1300 ng/ml. Three out of 9 pts with F > 1300 ng/ml were symptomatic for heart failure, and echocardiography showed a dilated cardiomyopathy. All pts with F < 1300 ng/ml had a normal systolic function. Mean left ventricular (LV) diastolic dimension and LV mass index were significantly increased in pts with TM versus controls (respectively: 37.2 +/- 7.9 mm vs 30.5 +/- 4.3 mm--p < 0.001; 78.6 +/- 16.7 g vs 65.2 +/- 19.4 g--p < 0.05). Moreover, LV end-diastolic dimension was significantly increased in patients with TM having normal systolic function versus controls (36.1 +/- 7.5 mm vs 30.5 +/- 4.3 mm). No difference was found between patients with TM and controls for wall thickness nor for Doppler diastolic indexes obtained from analysis of transmitral flow. Our study suggests that a correct chelation therapy may protect pts with TM from early development of a dilated cardiomyopathy. The first echocardiographic abnormality in pts still asymptomatic and with normal systolic function seems to be an increased end diastolic LV dimension. In our experience, left ventricular filling is not altered in asymptomatic patients.