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Journal of Cardiovascular Magnetic Resonance

Open Access

Myocardial iron overload in sickle/thalassemia patients of Italian origin

  • Antonella Meloni1,
  • Giovan Battista Ruffo2,
  • Petra Keilberg1,
  • Domenico D'Ascola3,
  • Alessandra Quota4,
  • Claudio Ascioti5,
  • Vincenzo Positano1,
  • Cristina Salvatori6,
  • Letizia Gulino1,
  • Massimo Lombardi1 and
  • Alessia Pepe1
Journal of Cardiovascular Magnetic Resonance201315(Suppl 1):E106

https://doi.org/10.1186/1532-429X-15-S1-E106

Published: 30 January 2013

Keywords

Iron OverloadThalassemiaIron DepositionItalian PatientMyocardial Iron

Background

Sickle-thalassemia is an inherited hemoglobin disorder resulting from the combined heterozygosity for sickle-cell and β-thalassemia genes. Myocardial iron overload in patients with sickle-thalassemia has been poorly studied; however, a report has shown no evidence of cardiac iron in a small group (n=10) of multitransfused Arab patients. The current study aims to further evaluate cardiac iron overload in a larger group of Italian patients using a T2* multislice approach and explore its correlation with transfusions, age and sex.

Methods

Fifty-nine sickle-thalassemia patients (29 males, mean age 35.6±14.1 years), enrolled in the MIOT (Myocardial Iron Overload in Thalassemia) network 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 T2* values and the T2* value in the mid-ventricular segment averaged over the mid-anterior and the mid-inferior septum.

Results

We found 55 (93%) 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* values >20 ms and other segments with T2* values <20 ms) and none showed an homogeneous MIO (all segment with T2* values <20 ms). Out of the 4 patients with heterogeneous MIO, only one had a global T2* global <20 ms.

The mean global heart T2* value was 34.4±6.2 ms.

We did not find significant differences among sickle-thalassemia regularly (N=20), sporadically (N=32) and no transfused (N=7) in the T2* global value (33.4±7.3 ms versus 35.5±5.4 ms versus 32.4±6.3 ms; P=0.425).

On linear regression analysis, there was a statistically significant positive correlation between global T2* and age but with poor linearity (R=0.368; P=0.004).

The global T2* value was not significant different between males and females (35.6±4.9 ms versus 35.2±7.2 ms; P=0.118).

Conclusions

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

Funding

The MIOT project receives "no-profit support" from industrial sponsorships (Chiesi and Apotex). This study was also supported by: "Ministero della Salute, fondi ex art. 12 D.Lgs. 502/92 e s.m.i., ricerca sanitaria finalizzata anno 2006" e "Fondazione L. Giambrone".

Authors’ Affiliations

(1)
CMR Unit, Fondazione G.Monasterio CNR-Regione Toscana and Institute of Clinical Physiology, Pisa, Italy
(2)
ARNAS Ospedale Civico, Palermo, Italy
(3)
U.O. Microcitemie, A.O. "Bianchi-Melacrino-Morelli", Reggio Calabria, Italy
(4)
Serv. Talassemia, Osp. "V. Emanuele III", Gela, Italy
(5)
Struttura Complessa di Cardioradiologia, P.O. "Giovanni Paolo II", Lamezia Terme, Italy
(6)
Fondazione G.Monasterio CNR-Regione Toscana, Pisa, Italy

Copyright

© Meloni et al; licensee BioMed Central Ltd. 2013

This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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