scholarly journals Spatial Repolarization Heterogeneity Detected by Magnetocardiography Correlates with Cardiac Iron Overload and Adverse Cardiac Events in Beta-Thalassemia Major

PLoS ONE ◽  
2014 ◽  
Vol 9 (1) ◽  
pp. e86524 ◽  
Author(s):  
Chun-An Chen ◽  
Meng-Yao Lu ◽  
Shinn-Forng Peng ◽  
Kai-Hsin Lin ◽  
Hsiu-Hao Chang ◽  
...  
Hematology ◽  
2015 ◽  
Vol 21 (1) ◽  
pp. 46-53 ◽  
Author(s):  
Galila M. Mokhtar ◽  
Eman M. Sherif ◽  
Nevin M. Habeeb ◽  
Abeer A. Abdelmaksoud ◽  
Eman A. El-Ghoroury ◽  
...  

2020 ◽  
Vol 21 (8) ◽  
Author(s):  
Yazdan Ghandi ◽  
Danial Habibi ◽  
Aziz Eghbali

Background: Cardiac involvement in beta-thalassemia major patients is an important cause of mortality. Therefore, in these patients, timely diagnosis of cardiac disorder is essential. Objectives: The present study aimed at determining the association between cardiac iron overload and fragmented QRS (fQRS). Methods: This cross-sectional study was conducted on 40 β-TM patients, aged 5 - 40 years. The presence of fQRS was evaluated in 12-lead surface electrocardiograms. Cardiac T2* MRI was performed to determine the iron overload. The patients were divided into four groups of chelation therapy. Results: The mean age of patients was reported to be 22.50 ± 6.75 years. The groups showed no significant difference regarding gender, age, or left ventricular ejection fraction. The presence of fQRS was detected in 10 patients (25%), while T2* value was lower than 20 ms in 10 patients (25%). The mean age of patients with and without fQRS was 26.23 ± 2.71 and 19.40 ± 2.61 years, respectively (P = 0.001). The univariate analysis indicated that fQRS had a significant relationship with cardiac iron overload (OR = 5; 95% CI: 1.04 - 23.99; P < 0.044). The multiple logistic regression analysis represented a significant association between iron overload and fQRS (OR = 5.556; 95% CI: 1.027 - 30.049). The sensitivity and specificity of the fQRS against MRI were equal to 50% and 83.3% respectively. Conclusions: The absence of fQRS on ECGs could be a good predictor of the lack of cardiac iron overload in β-TM patients. The results showed that fQRS might indicate the no need for close monitoring for cardiac overload with cardiac MRI and aggressive chelation therapy.


2013 ◽  
Vol 30 (8) ◽  
pp. 755-760 ◽  
Author(s):  
Aysen Turedi ◽  
Yesim Oymak ◽  
Timur Meşe ◽  
Yöntem Yaman ◽  
Selen Bayraktaroglu ◽  
...  

2019 ◽  
Vol 6 (6) ◽  
pp. 269-272
Author(s):  
Subash Chandra Majhi ◽  
Nihar Ranjan Mishra ◽  
Prakash Chandra Panda ◽  
Sumeet Soumyaranjan Biswal

Blood ◽  
2011 ◽  
Vol 118 (21) ◽  
pp. 5308-5308
Author(s):  
Vassilios Perifanis ◽  
Efthimia Vlachaki ◽  
Nikolaos Neokleous ◽  
Aikaterini Teli ◽  
Antonia Kondou ◽  
...  

Abstract Abstract 5308 Iron overload in the myocardium causes cardiomyopathy and increases the mortality of the transfusion-dependent patients with beta-thalassemia major. Myocardial T2* magnetic resonance imaging (MRI) provides a rapid and reproducible measure of cardiac iron loading and is being increasingly used worldwide for monitoring of transfusion-dependent thalassemia patients. Recent studies associate myocardial siderosis (T2* < 20 ms) with left ventricular (LV) dysfunction. However its relation with the right ventricular (RV) dysfunction has not yet been fully addressed. The aim of this study is to compare the relationship between cardiac T2* and RV function in transfusion-dependent beta-thalassemia major patients treated in a single institution in two different time points four years apart. Patients and Methods: Hundred patients (55 men; mean age, 25.8 ± 8,28 years) systematically transfused and iron chelated with different regimens were enrolled in the study. All MRI scans were performed at the beginning of the study and 4 years later. Each scan, acquired with an imager equipped with a 1.5 T magnet, included the measurement of heart T2* (mid-septum) together with LV and RV volumes, ejection fraction (EF ) and mass using previously published techniques. Results :Mean ferritin, mean T2* and mean RVEF at baseline was 1517 ± 1117 ng/ml, 33,39 ± 15,6 ms and 68 ± 5,37% respectively. There was no evident correlation between T2* and RVEF for the entire group as well as for the group with abnormal T2*. Four years later the T2* MRI was 36,64 ± 14,46 % ms expressing a statistical significant change (p<0.05). Mean ferritin and mean RVEF was 1275 ± 1045 ng/ml and 67,4±8,3% respectively. There was no other significant correlation between T2* and RESV, REDV for the entire group in the checked time points. At the beginning of the study, 20 out of the 100 patients had a T2* measurement below 20ms. At the end of the study 11 patients continued to have abnormal T2*.Four of the responders were treated with Deferiprone (out of 5), two with Deferasirox (out of 4) and three out of 5 with combination therapy (Desferrioxamine and deferiprone). All 6 patients treated with Desferrioxamine remained abnormal, although with better T2* measurements. Although there was a significant change in T2* measurements (p=0,018) in the group with abnormal T2*, RVEF did not show equally alteration. Conclusions: Myocardial iron deposition by MRI does not seem to be associated with RV dysfunction. The number of responders was too small to allow conclusions for most effective therapy. The only limitation of our study is that in contrast with other reports the percentage of patients with abnormal T2* was smaller (20%). Larger studies are required to determine the relation of right ventricular function and cardiac iron overload. Disclosures: Vlachaki: Novartis Hellas S.A.C.I.:. Oikonomou:Novartis Hellas S.A.C.I.:.


Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 2699-2699
Author(s):  
Yelena Z. Ginzburg ◽  
Radma Mahmood ◽  
Steven Brunnert ◽  
Mary E. Fabry ◽  
Ronald L. Nagel

Abstract Despite the use of transfusion and iron chelation therapy, patients with β-thalassemia major have a shortened life expectancy. Many of those deaths are attributable to cardiac iron overload. Nevertheless, the process by which cardiac iron overload occurs is not well understood. We have used the homozygous βmajor deletion [Hbbth-1] (THL) mouse model to assess hepatic and cardiac iron load. RBC indices for 3 THL mice and 2 C57BL/6 wildtype control mice prior to and post therapy with parenteral iron were evaluated with Advia. Intraperitoneal iron dextran injection at 10mg/25gm body weight daily 5 days per week for 12 days was performed and then switched to 1.25mg/25gm body weight of iron injection for another 10 days for a total of 4 weeks. Histological samples of liver and heart were stained with Prussian blue in mice prior to and post administration of parenteral iron. Immunohistochemistry with antibody to F4/80, specific for macrophages, was performed and counterstained with Prussian blue in livers and hearts of THL and C57 mice. The RBC indices in THL mice reveal an anemia (HCT 29.5±2.3 vs 45±2.1%, P=0.005) and reticulocytosis (2218±501 vs 406±101 x 109 cells, P=0.018) prior to therapy relative to the C57 mice (values presented as mean ± standard deviation). In THL mice after parenteral iron, HCT (41.8±6.8 vs 29.5±2.3%, P=0.04) and reticulocyte counts (2218±501 vs 3760±633 x 109 cells, P=0.03) increased significantly from pre-treatment values while in C57 mice, the HCT (53.8±6 vs 45±2.1%, NS) and reticulocyte count (406±101 vs 210±49 x109 cells, NS) did not change appreciably from baseline. Prior to therapy, the liver of THL mice exhibit 20–25% Kupffer cells staining with Prussian blue, with no Prussian blue staining in hepatocytes. The hearts of THL mice have no macrophages and no iron deposition at baseline. Prior to therapy, the livers of C57 mice had similar numbers of Kupffer cells compared to THL mice though none stain with Prussian blue. After treatment with parenteral iron, the livers of THL and C57 mice became significantly iron loaded (75–80% of Kupffer cells are positive for Prussian blue), the number of Kupffer cells increased 4-fold, and the majority of the Prussian blue staining was limited to Kupffer cells (90–95%). After treatment with parenteral iron, the hearts of THL and C57 mice became significantly iron loaded as well, but unlike the liver, most (90%) of the Prussian blue positive cells were myocytes. Only a small fraction of the myocytes in the heart was involved (5%). THL mice appear to be iron deficient and show bone marrow reserve with reticulocytosis significantly above baseline when excess iron is administered. Iron overload secondary to intraperitoneal iron dextran administration affects THL mice as well as C57 mice. In the liver of THL mice, Kupffer cells normally resident in the liver become laden with iron; little iron is deposited in hepatocytes. In the heart, an organ without resident macrophages and few macrophages migrating into the tissue during parenteral iron administration, both THL and C57 mice reveal myocyte deposition of iron. In conclusion, parenteral iron administration leads to a noticeable increase in RBCs in THL mice. Furthermore, both the livers and hearts of THL mice accumulate iron. Finally, these findings correlate well with the natural history of cardiac iron overload in human β-thalassemia major, leading to the conclusion that THL mice are a suitable model for the study of cardiac iron overload in thalassemia.


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