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

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 T...

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Veröffentlicht in:The International Journal of Cardiovascular Imaging 2008-12, Vol.24 (8), p.849-854
Hauptverfasser: Mavrogeni, Sophie, Gotsis, Efstathios, Ladis, Vassilios, Berdousis, Eleni, Verganelakis, Dimitrios, Toulas, Panagiotis, Cokkinos, Dennis V.
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Sprache:eng
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Zusammenfassung: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.
ISSN:1569-5794
1573-0743
DOI:10.1007/s10554-008-9332-2