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.
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.
Authors: B Henninger; C Kremser; S Rauch; R Eder; H Zoller; A Finkenstedt; H J Michaely; M Schocke Journal: Eur Radiol Date: 2012-05-30 Impact factor: 5.315
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Authors: Dudley J Pennell; John B Porter; Maria Domenica Cappellini; Lee Lee Chan; Amal El-Beshlawy; Yesim Aydinok; Hishamshah Ibrahim; Chi-Kong Li; Vip Viprakasit; Mohsen S Elalfy; Antonis Kattamis; Gillian Smith; Dany Habr; Gabor Domokos; Bernard Roubert; Ali Taher Journal: Haematologica Date: 2012-01-22 Impact factor: 9.941
Authors: Declan P O'Regan; Martina F Callaghan; Julie Fitzpatrick; Rossi P Naoumova; Joseph V Hajnal; Stephan A Schmitz Journal: Eur Radiol Date: 2007-11-23 Impact factor: 5.315
Authors: Taigang He; Peter D Gatehouse; Gillian C Smith; Raad H Mohiaddin; Dudley J Pennell; David N Firmin Journal: Magn Reson Med Date: 2008-11 Impact factor: 4.668