Impact of magnetic resonance imaging on cardiac mortality in thalassemia major

Chouliaras, Giorgos; Berdoukas, Vasilios; Ladis, Vassilis; Kattamis, Antonis; Chatziliami, Antonia; Fragodimitri, Christina; Karabatsos, Fotis; Youssef, Jacqueline; Karagiorga-Lagana, Markissia

doi:10.1002/jmri.22621

Cited by 45 publications

(31 citation statements)

References 20 publications

(23 reference statements)

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Since several studies showed a remarkably strong correlation of heart T2* value with clinical cardiac complications, including heart failure and arrhythmia, CMR T2* had been applied to monitor cardiac iron deposition in TM patients in UK [63,64] . Interestingly, the mortality rate was significantly reduced.…”

Section: Cross Sectionalmentioning

confidence: 99%

Early detection of cardiac involvement in thalassemia: From bench to bedside perspective

Koonrungsesomboon

Chattipakorn²,

Fucharoen³

et al. 2013

WJC

View full text Add to dashboard Cite

Myocardial siderosis is known as the major cause of death in thalassemia major (TM) patients since it can lead to iron overload cardiomyopathy. Although this condition can be prevented if timely effective intensive chelation is given to patients, the mortality rate of iron overload cardiomyopathy still remains high due to late detection of this condition. Various direct and indirect methods of iron assessment, including serum ferritin level, echocardiogram, non-transferrin-bound iron, cardiac magnetic resonance T2*, heart rate variability, and liver biopsy and myocardial biopsy, have been pro- REVIEW 270August 26, 2013|Volume 5|Issue 8|

show abstract

Section: Cross Sectionalmentioning

confidence: 99%

Early detection of cardiac involvement in thalassemia: From bench to bedside perspective

Koonrungsesomboon

Chattipakorn²,

Fucharoen³

et al. 2013

WJC

View full text Add to dashboard Cite

show abstract

“…Liver R2 magnetic resonance imaging (MRI) has almost completely replaced invasive biopsy for the assessment of liver iron in patients with thalassemia in the United States, and in parallel, liver iron concentration (LIC) has significantly improved in this patient population over the past decade . Cardiac T2* MRI, which enables accurate prediction of the risk of developing iron‐related cardiac disease, is increasingly utilized to tailor iron chelation treatment in patients with thalassemia, and is a factor contributing to improved survival in this patient population . Finally, the availability of oral treatment options, including deferasirox and deferiprone, may improve adherence and control of iron burden compared to subcutaneous infusion with deferoxamine.…”

Section: Introductionmentioning

confidence: 99%

Transfusional Iron Overload in a Cohort of Children with Sickle Cell Disease: Impact of Magnetic Resonance Imaging, Transfusion Method, and Chelation

Stanley

Friedman

Webb

et al. 2016

Pediatr Blood Cancer

View full text Add to dashboard Cite

BackgroundTransfusions prevent a number of complications of sickle cell disease (SCD), but cause inevitable iron loading. With magnetic resonance imaging (MRI), liver iron can be monitored noninvasively. Erythrocytapheresis can limit iron loading and oral chelation provides a more tolerable alternative to subcutaneous administration. The impact of these factors on control of iron burden in SCD has not been well studied.ProcedureIron monitoring practices, chelation use, and transfusion methods were assessed in our cohort of pediatric patients with SCD receiving chronic transfusion. The impact of these factors on iron burden was assessed.ResultsAmong 84 subjects, the proportion that underwent appropriate liver iron concentration (LIC) assessment rose from 21% before to 81% after implementation of R2‐MRI in 2006. Among subjects with at least two R2‐MRI examinations, median LIC improved (13.2–7.9 mg/g dw, P = 0.027) from initial to final study. Most (67.9%) subjects initially received simple transfusions and subsequently transitioned to erythrocytapheresis. After switching, LIC improved from 13.1 to 4.3 mg/g dw (P < 0.001) after a median of 2.7 years and ferritin improved (2,471–392 ng/ml, P < 0.001) after a median of 4.2 years. Final serum ferritin and LIC correlated negatively with the proportion of transfusions administered by erythrocytapheresis and chelation adherence.ConclusionsRoutine liver R2‐MRI should be performed in individuals with SCD who receive chronic red cell transfusions. Adherence with chelation should be assessed regularly and erythrocytapheresis utilized when feasible to minimize iron loading or reduce iron stores accumulated during periods of simple transfusion.

show abstract

“…Advances in the management of cardiac complications have led to a longer life expectancy in patients with transfusion-dependent anemias, 29,30 and other complications, including those related to liver iron overload, are increasingly being seen as a cause of death. 31,32 Therefore, the rapid and clinically significant decrease in liver iron burden from severe levels at baseline in HYPERION is important.…”

mentioning

confidence: 99%

Effects of deferasirox-deferoxamine on myocardial and liver iron in patients with severe transfusional iron overload

Aydınok

Kattamis

Cappellini

et al. 2015

Blood

Self Cite

View full text Add to dashboard Cite

Key Points• DFX-DFO combination followed by DFX monotherapy led to a meaningful decrease in myocardial and liver iron in severe siderosis patients.• Substantial liver iron reduction may be helpful in patients needing rapid control of liver iron (eg, pretransplant or planned pregnancy). Geometric mean mT2* ratios (Gmean month12/24 /Gmean baseline ) were 1.09 and 1.30, respectively, increasing from 7.2 ms at baseline (n 5 60) to 7.7 ms at 12 (n 5 52) and 9.5 ms at 24 months (n 5 36). Patients (17 of 60; 28.3%) achieved mT2* ‡10 ms and ‡10% increase from baseline at month 24; 15 switched to monotherapy during the study based on favorable mT2*. LIC decreased substantially from a baseline of 33.4 to 12.8 mg Fe/g dry weight at month 24 (252%). LVEF remained stable with no new arrhythmias/cardiac failure. Five patients discontinued with mT2* <5 ms and 1 died (suspected central nervous system infection). Safety was consistent with established monotherapies. Results show clinically meaningful improvements in mT2* in about one-third of patients remaining on treatment at month 24, alongside rapid decreases in LIC in this heavily iron-overloaded, difficult-to-treat population. Combination therapy may be useful when rapid LIC reduction is required, regardless of myocardial iron overload. This trial was registered at www.clinicaltrials.gov as #NCT01254227. (Blood. 2015;125(25):3868-3877)

show abstract

Impact of magnetic resonance imaging on cardiac mortality in thalassemia major

Cited by 45 publications

References 20 publications

Early detection of cardiac involvement in thalassemia: From bench to bedside perspective

Early detection of cardiac involvement in thalassemia: From bench to bedside perspective

Transfusional Iron Overload in a Cohort of Children with Sickle Cell Disease: Impact of Magnetic Resonance Imaging, Transfusion Method, and Chelation

Effects of deferasirox-deferoxamine on myocardial and liver iron in patients with severe transfusional iron overload

Contact Info

Product

Resources

About