Sickle-thalassemia is an inherited hemoglobin disorder resulting from the combined heterozygosity for sickle-cell and β-thalassemia genes [1]. Myocardial iron overload (MIO) in patients with sickle-thalassemia has been poorly studied. A report has shown no evidence of cardiac iron in a small group (n=10) of multitransfused Arab patients [2]. We aimed to evaluate MIO in a larger group of Italian patients using a T2* multislice approach [3] and explore its correlation with transfusions, age and sex.

Fifty-nine sickle-thalassemia patients (29 males, 35.6 ± 14.1 years), enrolled in the MIOT (Myocardial Iron Overload in Thalassemia) network [4] were considered. Three parallel short-axis views of the left ventricle were acquired and analyzed with a dedicated software (HIPPO MIOT) providing the T2* value on each of 16 segments as well as the global T2* value averaged over all segmental values and the T2* value in the mid-septum averaged over the mid-anterior and the mid-inferior septum [3, 5] (Figure 1).

We found 55 patients with all 16 segmental T2* values normal (>20 ms). Of the 4 patients with abnormal segmental T2* values, all showed an heterogeneous MIO (some segments with T2*>20 ms and other segments with T2*<20 ms) and none showed an homogeneous MIO (all segment with T2*<20 ms). Out of the 4 patients with heterogeneous MIO, only one had a global T2* <20 ms (Figure 2). The mean global heart T2* value was 34.4 ± 6.2 ms. The mean mid-septum T2* value was 37.1...

In respect of myocardial iron deposition, the sickle-thalassemia patients are similar to patients with homozygous SCD for which myocardial iron overload is relatively rare.

[1] Mukherjee MB et al. Indian J Hum Genet 2010;16(3):154–158. [2] Ghoti H et al. Eur J Haematol 2010;84(1):59-63. [3] Pepe A et al. JMRI 2006;23(5):662-668. [4] Meloni A et al. Int J Med Inform 2009;78(8):503-512. [5] Positano V et al. NMR Biomed 2007;20(6):578-590.