In patients with AM and preserved LVEF, LGE in the midwall layer of the AS myocardial segment is associated with a worse prognosis than other patterns of presentation.

BackgroundDue to the limited data available in literature, the aim of this multi-centre study was to prospectively compare in thalassemia major (TM) patients the efficacy of combined deferiprone (DFP) and deferoxamine (DFO) regimen versus either DFP and DFO in monotherapy by cardiovascular magnetic resonance (CMR) over a follow up of 18 months.MethodsAmong the first 1135 TM patients in the MIOT (Myocardial Iron Overload in Thalassemia) network, we evaluated those who had received either combined regimen (DFO + DFP, N=51) or DFP (N=39) and DFO (N=74) monotherapies between the two CMR scans. Iron overload was measured by T2* multiecho technique. Biventricular function parameters were quantitatively evaluated by cine images.ResultsThe percentage of patients that maintained a normal global heart T2* value was comparable between DFP+DFO versus both monotherapy groups. Among the patients with myocardial iron overload at baseline, the changes in the global heart T2* and in biventricular function were not significantly different in DFP+DFO compared with the DFP group. The improvement in the global heart T2* was significantly higher in the DFP+DFO than the DFO group, without a difference in biventricular function. Among the patients with hepatic iron at baseline, the decrease in liver iron concentration values was significantly higher with combination therapy than with either monotherapy group.ConclusionsIn TM patients at the dosages used in the real world, the combined DFP+DFO regimen was more effective in removing cardiac iron than DFO, and was superior in clearing hepatic iron than either DFO or DFP monotherapy. Combined therapy did not show an additional effect on heart function over DFP.

Purpose:To assess the tissue iron concentration of the left ventricle (LV) using a multislice, multiecho T2* MR technique and a segmental analysis.Materials and Methods: T2* multiecho MRI was performed in 53 thalassemia major patients. Three short-axis views of the LV were obtained and analyzed with custom-written software. The myocardium was automatically segmented into 12 segments. The T2* value on each segment as well as the global T2* value were calculated. Cine dynamic images were also obtained to evaluate biventricular function parameters by quantitative analysis.Results: For the T2* global value, the coefficient of variation (CoV) for intra-/interobserver and interstudy reproducibility was 3.9% (r ϭ 0.98), 5.5% (r ϭ 0.98), and 4.7% (r ϭ 0.99) respectively. Three groups were identified based on analysis of myocardial T2*: homogeneous (21%), heterogeneous (38%), and no myocardial iron overload (41%). The mean serum ferritin, liver iron concentration, and urinary iron excretion were significantly different among the groups. We did not find significant differences among groups in biventricular function. There was a correlation between the global T2* value and the T2* value in the mid-ventricular septum (r ϭ 0.95, P Ͻ 0.0001). Conclusion:Multislice multiecho T2* MRI provides a noninvasive, fast, reproducible means of assessing myocardial iron distribution. The single measurement of mid-septal T2* correlated well with the global T2* value.