Left atrial strain in patients with β-thalassemia major: a cross-sectional CMR study

Objectives The aim of this cross-sectional study was to investigate the association of left atrial (LA) strain parameters with demographics, clinical data, cardiovascular magnetic resonance (CMR) fndings, and cardiac complications (heart failure and arrhythmias) in a cohort of patients with β-thalassemia major (β-TM). Materials and methods We considered 264 β-TM patients (133 females, 36.79 ± 11.95 years) consecutively enrolled in the Extension-Myocardial Iron Overload in Thalassemia (E-MIOT) project. Moreover, we included 35 sex- and agematched healthy controls (14 females, mean age 37.36 ± 17.52 years). Reservoir, conduit, and booster LA functions were analysed by CMR feature tracking using dedicated software. Results Compared to the healthy control group, β-TM patients demonstrated lower LA reservoir strain and booster strains, as well as LA reservoir and booster strain rates. However, no diferences were found in LA conduit deformation parameters. In β-TM patients, ageing, sex, and left ventricle (LV) volume indexes were independent determinants of LA strain parameters. The number of segments with late gadolinium enhancement (LGE) signifcantly correlated with all LA strain parameters, with the exception of the LA conduit rate. Patients with cardiac complications exhibited signifcantly impaired strain parameters compared to patients without cardiac complications. Conclusion In patients with β-TM, LA strain parameters were impaired compared to control subjects, and they exhibited a signifcant correlation with the number of LV segments with LGE. Furthermore, patients with cardiac complications had impaired left atrial strain parameters. Clinical relevance statement In patients with β-thalassemia major, left atrial strain parameters were impaired compared to control subjects and emerged as a sensitive marker of cardiac complications, stronger than cardiac iron levels

CMR T2* Technique for Segmental and Global Quantification of Myocardial Iron: Multicentre Transferability and Healthcare Impact Evaluation

The multislice multiecho T2* technique is transferable among scanners with good reproducibility. The network seems to be a robust and scalable system in which T2* CMR based cardiac iron overload assessment is available, accessible and reachable for a significant and increasing number of thalassemia patients, reducing the mean distance from the patients\u27 locations to the CMR sites

Longitudinal follow‐up of patients with thalassaemia intermedia who started transfusion therapy in adulthood: a cohort study

SummaryWe longitudinally evaluated the effects of regular blood transfusions (BTs), in the real‐life context of the Myocardial Iron Overload in Thalassaemia network, in patients with thalassaemia intermedia (TI). We considered 88 patients with TI (52 females) who started regular BTs after the age of 18 years. Magnetic resonance imaging was used to quantify iron overload and biventricular function. For 56·8% of the patients there were more than two indications for the transition to regular BTs, with anaemia present in 94·0% of the cases. A significant decrease in nucleated red blood cells, platelets, lactate dehydrogenase, bilirubin, and uric acid levels was detected 6 months after starting regular BTs. After the transition to the regular BT regimen there was a significant increase only in the frequency of hypothyroidism and osteopenia, and a significant decrease in liver iron and cardiac index. The percentage of chelated patients increased significantly after starting regular BTs. The decision to regularly transfuse patients with TI may represent a way to prevent or slow down the natural progression of the disease, despite the more complex initial management

Fast generation of <i>T2</i>* maps in the entire range of clinical interest: application to thalassemia major patients

T2* maps obtained by the processing of multiecho MR sequences can be useful in several clinical applications. T2* map generation procedures should join a processing time compatible with on-line image analysis with a good precision in the entire T2* range of clinical interest. Fast generation of T2* maps can be achieved by the estimation of the T2* values by the weighted linear fitting of the logarithm of the signal (WLSL) method. This approach fails if the signal decay diverges from a pure exponential decay, as happens at low T2* values where the rapid decay in the signal intensity leads to a plateau in the later echo times (TE). The proposed method implements the automatic truncation of the signal decay curves to be fitted in order to compensate for the signal collapse at low T2* values, allowing the extension of the WLSL method through the entire clinical range of T2* values. Validation was performed on synthetic images and on 60 thalassemia major patients with different levels of myocardial iron overload. Phantom experiments showed that a 5% fitting error threshold represented the best compromise between T2* value measurement precision and processing time. A good agreement was found between T2* map pixel-wise measurements and ROI-based measurements performed by expert readers (CoV=1.84% in global heart T2*, CoV=5.8% in segmental analysis). In conclusion, the developed procedure was effective in generating correct T2* maps for the entire T2* clinical range.</br

Prognostic value of multiparametric cardiac magnetic resonance in sickle cell patients

The aim of this multicenter study was to prospectively assess the predictive value of multiparametric cardiac magnetic resonance (CMR) for cardiovascular complications in sickle cell disease (SCD) patients. Among all patients with hemoglobinopathies consecutively enrolled in the Myocardial Iron Overload in Thalassemia (MIOT) Network, we selected 102 SCD patients (34.38 +/- 12.67 years, 49 females). Myocardial iron overload (MIO) was measured by the multislice multiecho T2* technique. Atrial dimensions and biventricular function parameters were quantified by cine images. Late gadolinium enhancement (LGE) images were acquired to detect focal myocardial fibrosis. At baseline CMR, only two patients had significant MIO (global heart T2* 31 g/m2 were optimal cut-off values. Reduced left ventricular ejection fraction and increased right ventricular mass index showed a significant prognostic value in patients with SCD. Our data seem to suggest that CMR may be added as a screening tool for identifying SCD patients at high risk for cardiopulmonary and vascular diseases

IL28B polymorphisms influence stage of fibrosis and spontaneous or interferon-induced viral clearance in thalassemia patients with hepatitis C virus infection

Polymorphisms in the interleukin-28B are important determinants in the spontaneous and drug-induced control of hepatitis C virus infection

Feasibility, reproducibility, and reliability for the T^*₂ iron evaluation at 3 T in comparison with 1.5 T

This study aimed to determine the feasibility, reproducibility, and reliability of the multiecho T*2 Magnetic resonance imaging technique at 3 T for myocardial and liver iron burden quantification and the relationship between T*2 values at 3 and 1.5 T. Thirty-eight transfusion-dependent patients and 20 healthy subjects were studied. Cardiac segmental and global T*2 values were calculated after developing a correction map to compensate the artifactual T*2 variations. The hepatic T*2 value was determined over a region of interest. The intraoperator and interoperator reproducibility for T*2 measurements at 3 T was good. A linear relationship was found between patients' R (1000/T*2) values at 3 and 1.5 T. Segmental correction factors were significantly higher at 3 T. A conversion formula returning T*2 values at 1.5 T from values at 3 T was proposed. A good diagnostic reliability for T*2 assessment at 3 T was demonstrated. Lower limits of normal for 3 T T*2 values were 23.3 ms, 21.1 ms, and 11.7 ms, for the global heart, mid-ventricular septum, and liver, respectively. In conclusion, T*2 quantification of iron burden in the mid-ventricular septum, global heart, and no heavy–moderate livers resulted to be feasible, reproducible, and reliable at 3 T. Segmental heart T*2 analysis at 3 T may be challenging due to significantly higher susceptibility artifacts. Magn Reson Med, 2012