Evaluation of the efficacy of oral deferiprone in beta-thalassemia major by multislice multiecho T2*

Abstract

Objectives: Oral deferiprone (L1) appears to be promising in the treatment of beta-thalassemia major (TM) patients. T2* magnetic resonance imaging (MRI) with a single measurement in the mid-ventricular septum was validated as a quantitative evaluation of myocardial iron overload. Previous studies suggested a marked heterogeneity of iron distribution in the myocardium. We set up a multislice multiecho T2* MRI for the detection of this heterogeneity. The aim of our study was to investigate differences between the L1 vs. the subcutaneous desferrioxamine (DF)-treated patients using this new approach.

Methods: Thirty-six beta-TM patients (age 29 +/- 8 yr) underwent MRI. Eighteen patients received long-term L1, and 18 other patients matched for age and sex received DF. T2* multiecho sequences on three short axis views of the left ventricle were obtained and analyzed by custom-made software. In each slice, the myocardium was automatically segmented into four segments. Cine-dynamic images were also obtained to evaluate biventricular function.

Results: For multislice T2* technique, the coefficient of variation for intra- and inter-observer, and inter-study reproducibility was 3.9%, 4.7%, and 5.5%, respectively. The global heart T2* value was significantly higher in the L1 vs. DF group (35 +/- 7 vs. 27 +/- 2 ms; P = 0.02). The number of segments with normal T2* value (>20 ms) was significantly higher in the L1 vs. the DF group (11 +/- 1 vs. 8 +/- 5 segments; P = 0.03). We did not detect significant differences in biventricular function parameters.

Conclusions: This new approach confirms that L1 could be more effective than DF in removal of myocardial iron.