Dataset Information

Deferasirox for up to 3 years leads to continued improvement of myocardial T2* in patients with ?-thalassemia major.

ABSTRACT: Prospective data on cardiac iron removal are limited beyond one year and longer-term studies are, therefore, important.Seventy-one patients in the EPIC cardiac substudy elected to continue into the 3(rd) year, allowing cardiac iron removal to be analyzed over three years.Mean deferasirox dose during year 3 was 33.6 ± 9.8 mg/kg per day. Myocardial T2*, assessed by cardiovascular magnetic resonance, significantly increased from 12.0 ms ± 39.1% at baseline to 17.1 ms ± 62.0% at end of study (P<0.001), corresponding to a decrease in cardiac iron concentration (based on ad hoc analysis of T2*) from 2.43 ± 1.2 mg Fe/g dry weight (dw) at baseline to 1.80 ± 1.4 mg Fe/g dw at end of study (P<0.001). After three years, 68.1% of patients with baseline T2* 10 to <20 ms normalized (? 20 ms) and 50.0% of patients with baseline T2* >5 to <10 ms improved to 10 to <20 ms. There was no significant variation in left ventricular ejection fraction over the three years. No deaths occurred and the most common investigator-assessed drug-related adverse event in year 3 was increased serum creatinine (n = 9, 12.7%).Three years of deferasirox treatment along with a clinically manageable safety profile significantly reduced cardiac iron overload versus baseline and normalized T2* in 68.1% (32 of 47) of patients with T2* 10 to <20 ms.

SUBMITTER: Pennell DJ

PROVIDER: S-EPMC3366648 | biostudies-literature | 2012 Jun

REPOSITORIES: biostudies-literature

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Deferasirox for up to 3 years leads to continued improvement of myocardial T2* in patients with β-thalassemia major.

Pennell Dudley J DJ Porter John B JB Cappellini Maria Domenica MD Chan Lee Lee LL El-Beshlawy Amal A Aydinok Yesim Y Ibrahim Hishamshah H Li Chi-Kong CK Viprakasit Vip V Elalfy Mohsen S MS Kattamis Antonis A Smith Gillian G Habr Dany D Domokos Gabor G Roubert Bernard B Taher Ali A

Haematologica 20120122 6

<h4>Background</h4>Prospective data on cardiac iron removal are limited beyond one year and longer-term studies are, therefore, important.<h4>Design and methods</h4>Seventy-one patients in the EPIC cardiac substudy elected to continue into the 3(rd) year, allowing cardiac iron removal to be analyzed over three years.<h4>Results</h4>Mean deferasirox dose during year 3 was 33.6 ± 9.8 mg/kg per day. Myocardial T2*, assessed by cardiovascular magnetic resonance, significantly increased from 12.0 ms ...[more]

PMID: 22271905

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Project description:PurposeTo develop and evaluate a free-breathing slice-interleaved T2 mapping sequence by proposing a new slice-selective T2 magnetization preparation (T2 prep) sequence that allows interleaved data acquisition for different slices in subsequent heartbeats.MethodsWe developed a slice-selective T2 prep for myocardial T2 mapping by adding slice-selective gradients to a conventional single-slice T2 prep sequence. In this sequence, five slices are acquired during five consecutive heartbeats, each using a slice-selective T2 prep. The scheme was repeated four times using different T2 prep echo times. We compared the performance of the proposed slice-interleaved T2 mapping sequence and the conventional single-slice T2 mapping sequence in term of accuracy, precision, and reproducibility using phantom experiments and in vivo imaging in 10 healthy subjects. We also evaluated the feasibility of the proposed sequence in 28 patients with cardiovascular disease, and the quality of the maps was scored subjectively. Furthermore, we investigated the impact of through-plane motion by comparing T2 measurements acquired during end-systole versus mid-diastole.ResultsT2 measurements using a slice-interleaved T2 mapping sequence were correlated with a spin echo (r(2) = 0.88) and single-slice T2 mapping sequence (r(2) = 0.98). The mean myocardial T2 values were correlated between slice-interleaved (48 ms) and single-slice (51 ms) T2 mapping sequences. Subjective scores of T2 map quality were good to excellent in 81% of the maps in patients. There was no difference in T2 measurements between end-systole versus mid-diastole.ConclusionsThe proposed free-breathing slice-interleaved T2 mapping sequence allows T2 measurements of five left ventricular slices in 20 heartbeats with similar reproducibility and precision as the single-slice T2 mapping sequence but with a four-fold reduction in acquisition time. Magn Reson Med 76:555-565, 2016. © 2015 Wiley Periodicals, Inc.

Dataset Information

Deferasirox for up to 3 years leads to continued improvement of myocardial T2* in patients with ?-thalassemia major.

Publications

Deferasirox for up to 3 years leads to continued improvement of myocardial T2* in patients with β-thalassemia major.

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