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Observational study of iron overload as assessed by magnetic resonance imaging in an adult population of transfusion-dependent patients with β thalassaemia: significant association between low cardiac T2* < 10 ms and cardiac events

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Abstract:

Abstract Background:

Thalassaemia major patients usually die from cardiac haemosiderosis. Improved strategies are required to modify this risk. Aims:

To assess the significance of cardiac iron overload in patients with β thalassaemia. Method:

Observational study of cardiac iron overload as assessed by magnetic resonance imaging (MRI) cardiac T2* relaxometry in 30 adult patients with transfusion-dependent β thalassaemia. Results:

11/30 patients (37%) had cardiac T2* < 10 ms, 8/30 (27%) in range 10–20 ms and 11/30 (37%) > 20 ms. There was significant inverse correlation between T2* values and values for serum ferritin (SF) and liver iron concentration (LIC) and positive correlation with left ventricular ejection fraction (LVEF). Median LVEF values were 49% in patients with T2* < 10 ms and 58% in patients with T2* > 10 ms (P= 0.02). Very low T2* values <10 ms were strongly associated with the occurrence of cardiac events (congestive heart failure, arrhythmia, cardiac death): occurring in 5/11 patients with T2* < l0 ms and in 0/19 in patients with T2* > 10 ms (P= 0.003 Fisher's exact test; P= 0.002 log rank Kaplan-Meier time to event analysis). There was no significant association between T2* < 10 ms or cardiac events and traditional measures of iron overload, such as SF levels >2500 µg/L and LIC (evaluated at thresholds of >7 or >15 mg/g dry weight). Conclusion:

Very low cardiac T2* values <10 ms are common in adults with β thalassaemia and are significantly associated with risk of cardiac events. This permits the use of individually targeted chelation strategies which are more effective in removing cardiac iron.

Keywords: iron chelation; iron overload; magnetic resonance imaging; β thalassaemia

Document Type: Research Article

DOI: http://dx.doi.org/10.1111/j.1445-5994.2009.01981.x

Affiliations: 1: Department of Radiology, Royal Adelaide Hospital and 2: Cardiovascular Research Centre University of Adelaide, Adelaide, South Australia, Australia 3: Division of Haematology, Institute of Medical and Veterinary Science,

Publication date: June 1, 2010

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