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Magnetic resonance evaluation of liver and myocardial iron deposition in thalassemia intermedia and b-thalassemia major

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Abstract

Introduction b-Thalassemia major (TM) and thalassemia intermedia (TI) are forms of inherited hemoglobinopathies. Our aim was to evaluate a population of asymptomatic TM and TI patients using cardiovascular magnetic resonance (CMR). We hypothesized that the TI group could be differentiated from the TM group based on T2*. We also hypothesized that the TI group would demonstrate significantly higher cardiac output compared to the TM group. Patients and methods Twenty-one consecutive TI patients aged 23(19–25) years, 21 TM patients and 21 age and sex matched controls were studied. Evaluation of heart, liver T2* relaxation time and right and left ventricular parameters was performed using a 1.5 T system. Results Myocardial and liver T2* values were significantly higher in TI patients compared to TM (34.35 ± 2.36 vs 15.77 ± 3.53 m, P < 0.001 and 5.12 ± 6.52 vs 1.36 ± 0.53 ms, P < 0.001, respectively). Controls had myocardial T2* 35.07 ± 4.52 ms (similar to TI patients, but significantly increased compared to TM patients, P < 0.001) and liver T2* 26.28 ± 2.37 ms (significantly increased compared to both TI and TM patients, P < 0.001). Left ventricular end-diastolic (LVEDV), end-systolic (LVESV) volumes and left ventricular ejection fraction (LVEF) were higher in TI patients compared to TM (P < 0.001). Stroke volume (LVSV), cardiac output (LVCO) and cardiac index (LVCI) were similarly increased in TI patients compared to TM (P < 0.001). Right ventricular end-diastolic volume (RVEDV), right ventricular end-systolic volume (RVESV) and right ventricular ejection fraction (RVEF) were higher in TI patients compared to TM (P < 0.001). Conclusions Although in TM iron plays a crucial role in the evolution of the disease, in TI the high output cardiac state seems to be the most prominent finding.

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Abbreviations

TI:

Thalassemia Intermedia

TM:

Thalassemia Major

T2*liver:

T2*relaxation time of liver

T2*heart:

T2*relaxation time of heart

BSA:

Body surface area

LVEDV:

Left ventricular end-diastolic volume

LVESV:

Left ventricular end- systolic volume

LVEF:

Left ventricular ejection fraction

LVSV:

Left ventricular stroke volume

LVCO:

Left ventricular cardiac output

LVCI:

Left ventricular cardiac index

RVEDV:

Right ventricular end-diastolic volume

RVESV:

Right ventricular end-systolic volume

RVEF:

Right ventricular ejection fraction

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Mavrogeni, S., Gotsis, E., Ladis, V. et al. Magnetic resonance evaluation of liver and myocardial iron deposition in thalassemia intermedia and b-thalassemia major. Int J Cardiovasc Imaging 24, 849–854 (2008). https://doi.org/10.1007/s10554-008-9332-2

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