Assessment of the Relationship Between Iron Overload Based on Cardiac T2* MRI and Fragmented QRS in Beta-Thalassemia Major Patients

2020 ◽  
Vol 21 (8) ◽  
Author(s):  
Yazdan Ghandi ◽  
Danial Habibi ◽  
Aziz Eghbali
Keyword(s):  
Iron Overload ◽  
Chelation Therapy ◽  
Thalassemia Major ◽  
Beta Thalassemia ◽  
Cardiac Iron ◽  
Fragmented Qrs ◽  
Beta Thalassemia Major ◽  
Cardiac Iron Overload ◽  
The Mean ◽  
T2 Mri

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.

scholarly journals Correlation between serum ferritin level, cardiac and hepatic T2-star MRI in patients with β-thalassemia major in Al-Diwaniya thalassemia center

2018 ◽  
Vol 9 (SPL1) ◽  
Author(s):  
Alaa Mutter Jabur Al-Shibany ◽  
AalanHadi AL-Zamili
Keyword(s):  
Iron Overload ◽  
Serum Ferritin ◽  
Serum Ferritin Level ◽  
Chelation Therapy ◽  
Ferritin Level ◽  
Thalassemia Major ◽  
Beta Thalassemia ◽  
Iron Level ◽  
The Mean ◽  
T2 Mri

Patients with transfusion dependent thalassemia major is often associated with iron overload. Proper use of iron chelators to treat iron overload requires an accurate measurement of iron levels. Magnetic resonance T2-star (T2* MRI) is the preferred method to measure iron level in the liver andthe heart. The goal of our study was to see if there is an association exists between serum ferritin level and T2* MRI results in patients with beta thalassemia major.This study was done in Al-Diwaniya Thalassemia center,Maternity and children teaching hospital,Iraq. During the period from 1st of January to 31st of October. Fifty eight patients with a diagnosis of beta thalassemia major were enrolled in the study. They were older than five years old,transfusion dependent and on chelation therapy. Hepatic and Myocardial T2*MRI and the mean serum ferritin levels were measured during the study period for all patients.There is a significant correlation was observed between serum ferritin level and cardiac T2*MRI (p=0.018 ). also a significant correlation was observed between serum ferritin and hepatic T2*MRI (p=0.02). Neither cardiac T2* MRI nor hepatic T2* MRI show any correlation with the mean age.our study also showa positive correlation between the patients withcardiac T2* MRI and the development of diabetes mellitus in contrast to hepatic T2* MRI in which there is no any correlation. Hypothyroidism was observedno correlation with either cardiac or hepatic T2* MRI.Our results showed a positiveassociation between hepatic, cardiac T2*MRI and serum ferritin levels.

GlutathioneS-transferase gene polymorphism: Relation to cardiac iron overload in Egyptian patients with Beta Thalassemia Major

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 ◽  
...  
Keyword(s):  
Gene Polymorphism ◽  
Iron Overload ◽  
Thalassemia Major ◽  
Beta Thalassemia ◽  
Cardiac Iron ◽  
Beta Thalassemia Major ◽  
Transferase Gene ◽  
Egyptian Patients ◽  
Cardiac Iron Overload

The Effect of HFE Polymorphisms on Cardiac Iron Overload in Patients with Beta-Thalassemia Major

2013 ◽  
Vol 30 (8) ◽  
pp. 755-760 ◽  
Author(s):  
Aysen Turedi ◽  
Yesim Oymak ◽  
Timur Meşe ◽  
Yöntem Yaman ◽  
Selen Bayraktaroglu ◽  
...  
Keyword(s):  
Iron Overload ◽  
Thalassemia Major ◽  
Beta Thalassemia ◽  
Cardiac Iron ◽  
Beta Thalassemia Major ◽  
Cardiac Iron Overload

scholarly journals SERUM FERRITIN AS A DIAGNOSTIC MARKER FOR CARDIAC IRON OVERLOAD AMONG BETA-THALASSEMIA MAJOR CHILDREN

2019 ◽  
Vol 6 (6) ◽  
pp. 269-272
Author(s):  
Subash Chandra Majhi ◽  
Nihar Ranjan Mishra ◽  
Prakash Chandra Panda ◽  
Sumeet Soumyaranjan Biswal
Keyword(s):  
Iron Overload ◽  
Serum Ferritin ◽  
Diagnostic Marker ◽  
Thalassemia Major ◽  
Beta Thalassemia ◽  
Cardiac Iron ◽  
Beta Thalassemia Major ◽  
Cardiac Iron Overload

Update in Combined Chelation Therapy with Deferoxamine and Deferiprone in Brazilian Patients with Thalassemia Major: Assessment of Three Years

Blood ◽  
2008 ◽  
Vol 112 (11) ◽  
pp. 5415-5415
Author(s):  
Sandra Regina Loggetto ◽  
Mônica Veríssimo ◽  
Antônio Fabron Júnior ◽  
Giorgio Roberto Baldanzi ◽  
Nelson Hamerschlak ◽  
...  
Keyword(s):  
Adverse Events ◽  
Iron Overload ◽  
Serum Ferritin ◽  
Chelation Therapy ◽  
Combined Therapy ◽  
Thalassemia Major ◽  
Liver Iron ◽  
Cardiac Iron ◽  
Cardiac Iron Overload

Abstract Introduction: Cardiac failure is a main cause of morbidity and mortality in patients with thalassemia major (TM) who are receiving regular blood transfusion due to iron overload. So, effective and adequate iron chelation is extremely important. Deferoxamine (DFO), the most widely used iron chelator, has poor compliance. Combined therapy with Deferiprone (DFP) increases chelation efficacy, decreases iron-induced complications, improves compliance increasing survival in thalassemia. Objectives: Assessment of efficacy and safety in combined chelation with DFP and DFO in thalassemic patients with iron overload. Methods and results: We have 50 thalassemia major patients in 4 Brazilian Centers (Boldrini Hospital, Sao Paulo Hematology Center, HEMEPAR and FAMEMA) receiving combined chelation therapy with follow up to three years. DFP (75–100 mg/kg/daily) and DFO (30–60 mg/kg, 4–7 days/week) are being administered during one to three years. Median age of this group is 21,5 y/o (range 8–35), with 48% female. Median age to start regular transfusions was 12 months (range 2–140) and to begin chelation therapy was 57 months (range 17–216). All patients were screened for Hepatitis C and 26% had positive sorology and/or PCR. Statistical analysis were made with Spearman test and Fisher test. All patients, except two, did cardiac and liver MRI in the initial phase of the study, resulting in 60,5% with cardiac iron overload (T2*&lt;20ms), being severe in 31,2%. Assessment of liver iron concentration (LIC) showed 95,7% with liver iron overload (&gt;3ug/g dry weight), being severe in 17,4%. During follow up, only 43 patients (86%) was screened with MRI. From these, 67,4% had cardiac iron overload (severe in 32,5%) and 78,6% had liver iron overload (severe in 11,9%). Mean serum ferritin before and after three years were 3095,7 ±1934,5 ng/ml and 2373,9±1987,6 ng/ml, respectively. Our data showed positive correlation between serum ferritin, LIC and ALT, even in initial data and after combined chelation therapy (p&lt;0,001), but there is no correlation between cardiac T2* and LIC and between cardiac T2* and ferritin. DFP adverse events included 8% agranulocytosis, 22% neutropenia, 20% arthralgia and 38% gastric intolerance. DFO adverse events were 2,6% deafness, 2,0% cataract and 12% growth deficit. Hepatic toxicity was found in 6%, but without necessity to stop treatment. Compliance in this group was excellent in 48%, good in 22% and poor in 30%. Conclusions: This is the first multicenter study to evaluate combined chelation therapy in Brazil based on cardiac MRI and LIC. Most patients had cardiac and hepatic iron overload probably because they began iron chelation lately, due to difficult access to iron chelators in the past. Cardiac iron overload didn’t have correlation with ferritin and LIC and these data need more understanding. Age of initial regular blood transfusion, increased transfusional requirement, inadequate chelation or delayed chelation may play a role in this question. Combined therapy with DFO and DFP is effective to decrease serum ferritin and LIC. Follow up and improving compliance may decrease cardiac iron overload. Adverse events are similar to literature. Combined therapy is safety in TM patients with transfusional iron overload.

No Correlation Between Myocardial T2* and Right Ventricular Function in Thalassemia Major. A Single Centre Four Years Experience

Blood ◽  
2011 ◽  
Vol 118 (21) ◽  
pp. 5308-5308
Author(s):  
Vassilios Perifanis ◽  
Efthimia Vlachaki ◽  
Nikolaos Neokleous ◽  
Aikaterini Teli ◽  
Antonia Kondou ◽  
...  
Keyword(s):  
Iron Overload ◽  
Right Ventricular ◽  
Ventricular Function ◽  
Right Ventricular Function ◽  
Thalassemia Major ◽  
Beta Thalassemia ◽  
Entire Group ◽  
Time Points ◽  
Cardiac Iron ◽  
Beta Thalassemia Major

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.:.

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