Search

Background In 2004 seven Italian centers reported survival data for patients with thalassemia major (TM) and showed that heart disease due to iron overload was the most common cause of death (Borgna et al Haematologica 2004). In the same years the accurate and noninvasive assessment of cardiac siderosis was made possible in Italy by the introduction of the T2* cardiovascular magnetic resonance (CMR). We aimed to evaluate if the deployment of T2* CMR had an impact on the mortality rate.

Background Cardiovascular Magnetic Resonance (CMR) by late gadolinium enhancement (LGE) allows to detect myocardial fibrosis. Myocardial fibrosis was shown to be a relative common finding in large cohort of Italian thalassemia major (TM) patients mainly related to HCV infection, but specific studies involving only pediatric patients are not available. Our aim was to investigate the prevalence and clinical-instrumental correlates of myocardial fibrosis in pediatric TM patients.

Background Movement abnormalities in the left ventricle (LV) were shown to be not really frequent in thalassemia major (TM) patients but they were associated with age, myocardial iron overload, LV dilation and dysfunction, and myocardial fibrosis. No data are available about the prevalence and the correlates of right ventricular (RV) wall motion abnormalities. This study investigated the relationship between RV and LV motion abnormalities and between RV motion and function in a large cohort of well-treated TM patients. Methods CMR was performed in 1092 TM patients (537 male; 30.6 ± 8.5 years) enrolled in the Myocardial Iron Overload in Thalassemia (MIOT) Network. Cine images were acquired to evaluate wall motion and to quantify RV volumes and ejection fraction (EF). Results

Background: Due 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. Methods: Among 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. Results: The 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. Conclusions: In 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.

Background Chronically anemic patients develop compensatory ventricular dilation, even when maintained on chronic transfusion regimens. Our primary goal was to compare right and left ventricular dimensions and function assessed by Cardiovascular Magnetic Resonance (CMR) in pediatric, chronically-transfused sickle-cell disease (SCD) and thalassemia major (TM) patients who lacked cardiac iron. Moreover we explored systematic sex differences in ventricular dimensions in both populations. Methods We reviewed all CMRs identifying 261 studies suitable for analysis from 64 SCD patients (34 females and 30 males) and 49 TM patients (29 males and 20 females). All demographic and CMR parameters were inversely weighted by the number of exams. Analysis of covariance (ANCOVA) models were used to evaluate the impact of potential covariates (variables unbalanced between groups and associated with the outcome) on group differences in CMR parameters. Results In both populations, males had larger left ventricular (LV) and right ventricular (RV) dimensions than females, with a more marked effect observed in SCD patients. The percentage difference for the RV was larger than that one seen in normal subjects (from 8 to 14%). Table 1 shows the comparison of LV parameters between SCD and TM with the differentiation by sex. All LV volumes as well as the LV mass were significantly higher in SCD than in TM patients, also adjusting for the covariates. Table 2 shows the comparison of RV parameters between SCD and TM, by sex. Overall findings are similar to those from the LV. All RV volumes remained significantly higher in SCD also after ANCOVA adjustments, except RV ESVI. Conclusions Compared to TM patients, SCD patients showed significantly greater biventricular dilation and LV hypertrophy. This difference could not be explained by different hemoglobin levels, cardiac iron overload and systolic blood pressure. Our results represent important baseline findings that place changes introduced by iron overload as well as systemic and pulmonary vasculopathy in proper context.

Background Aortic pulse wave velocity (PWV) is considered as the “gold standard” measurement of arterial stiffness and is commonly calculated as the ratio between the distance separating two locations along the artery and the transit time (Δt) needed for the pressure or velocity wave to cover this distance. PWV is increasingly assessed by means of cardiovascular magnetic resonance (CMR). Our goal was evaluate the efficiency of a novel method for Δt estimation, based on the principle of group delay (TT-GD method).

Excellent/good levels of compliance were similar in the 3 groups (DFX 99%, DFP 95%; DFO 96%, P =0 .6). There were no significant differences in all 3 groups to maintain the patients without significant myocardial iron overload (global heart T2*≥20 ms) (DFX 98%; DFP 100%; DFO 98%; P=1.0). The percentage of patients that maintained a normal LVEF (>57%) was significantly lower in DFX (77%) versus DFP (100%) (P=0.018), it was no significantly different in DFX and DFO group (82%) (P=0.59). Among the patients with myocardial iron overload at baseline in all 3 groups, there was a significant improvement in the global heart T2* value and in the number of segment with a normal T2* value; only in the DFP group there was a significant improvement in the right global systolic function (+ 6.8% P =0.016). The improvement in the global heart T2* was significantly lower in the DFX versus the DFP group (P=0.0026), but it was not significantly different in the DFX versus the DFO group (mean difference global heart T2* 3.5±4.7 ms versus 8.8±8.6 ms and versus 3.7±5.5 ms, respectively; P=0.90) (Figure 1). The changes in the global systolic biventricular function were not significantly different among groups. Conclusions