Longitudinal monitoring of cardiac siderosis using cardiovascular magnetic resonance T2* in patients with thalassemia major on various chelation regimens: A 6-year study Journal Article


Authors: Ambati, S. R.; Randolph, R. E.; Mennitt, K.; Kleinert, D. A.; Weinsaft, J. W.; Giardina, P. J.
Article Title: Longitudinal monitoring of cardiac siderosis using cardiovascular magnetic resonance T2* in patients with thalassemia major on various chelation regimens: A 6-year study
Abstract: Cardiovascular magnetic resonance (CMR) and hepatic magnetic resonance imaging (MRI) have become reliable noninvasive tools to monitor iron excess in thalassemia major (TM) patients. However, long-term studies are lacking. We reviewed CMR and hepatic MRI T2* imaging on 54 TM patients who had three or more annual measurements. They were managed on various chelation regimens. Patients were grouped according to their degree of cardiac siderosis: severe (T2*, <10 msec), mild to moderate (T2*=10-20 msec), and no cardiac siderosis (T2*, >20 msec). We looked at the change in cardiac T2*, liver iron concentration (LIC) and left ventricular ejection fraction (LVEF) at years 3 and 5. In patients with severe cardiac siderosis, cardiac T2* (mean±SD) improved from 6.9±1.6 at baseline to 13.6±10.0 by year 5, mean ΔT2*=6.7 (P=0.04). Change in cardiac T2* at year 3 was not significant in the severe group. Patients with mild to moderate cardiac siderosis had mean cardiac T2* of 14.6±2.9 at baseline which improved to 26.3±9.5 by year 3, mean ΔT2*=11.7 (P=0.01). At baseline, median LICs (mg/g dry weight) in patients with severe, mild-moderate, and no cardiac siderosis were 3.6, 2.8, and 3.3, whereas LVEFs (mean±SD) (%) were 56.3±10.1, 60±5, and 66±7.6, respectively. No significant correlation was noted between Δ cardiac T2* and Δ LIC, Δ cardiac T2*, and Δ LVEF at years 3 and 5. Throughout the observation period, patients with no cardiac siderosis maintained their cardiac T2* above 20 msec. The majority of patients with cardiac siderosis improve cardiac T2* over time with optimal chelation. Am. J. Hematol. 88:652-656, 2013. © 2013 Wiley Periodicals, Inc.
Keywords: adolescent; adult; child; school child; treatment outcome; middle aged; major clinical study; follow-up studies; magnetic resonance imaging; heart disease; liver; disease severity; heart failure; iron; beta-thalassemia; heart arrhythmia; heart left ventricle ejection fraction; erythrocyte transfusion; heart; stroke volume; deferoxamine; myocardium; ferritin; heart diseases; nuclear magnetic resonance scanner; iron chelation; thalassemia major; iron chelating agents; deferasirox; monitoring, physiologic; hemosiderosis; deferiprone; cardiac siderosis; cardiovascular magnetic resonance
Journal Title: American Journal of Hematology
Volume: 88
Issue: 8
ISSN: 0361-8609
Publisher: John Wiley & Sons, Inc.  
Date Published: 2013-08-01
Start Page: 652
End Page: 656
Language: English
DOI: 10.1002/ajh.23469
PROVIDER: scopus
PUBMED: 23640778
PMCID: PMC4030379
DOI/URL:
Notes: --- - "Export Date: 4 September 2013" - "CODEN: AJHED" - "Source: Scopus"
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  1. Srikanth Reddy Ambati
    31 Ambati