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* =  1.7 (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.

• Authors: Srikanth R Ambati, Rachel E Randolph, Kevin Mennitt, Dorothy A Kleinert, Jonathan W Weinsaft, Patricia J Giardina
• Journal: American journal of hematology (Am J Hematol) Vol. 88 Issue 8 Pg. 652-6 (Aug 2013) ISSN: 1096-8652 [Electronic] United States
• PMID: 23640778 (Publication Type: Clinical Trial, Journal Article, Research Support, N.I.H., Extramural, Research Support, Non-U.S. Gov't)
• Copyright: Copyright © 2013 Wiley Periodicals, Inc.

Chemical References

• Iron Chelating Agents

Topics

• Adolescent
• Adult
• Child
• Female
• Follow-Up Studies
• Heart (diagnostic imaging, physiopathology)
• Heart Diseases (diagnostic imaging, etiology, metabolism, physiopathology)
• Hemosiderosis (diagnostic imaging, etiology, physiopathology)
• Humans
• Iron Chelating Agents (administration & dosage)
• Liver (diagnostic imaging, metabolism)
• Magnetic Resonance Imaging
• Male
• Middle Aged
• Monitoring, Physiologic (methods)
• Myocardium (metabolism)
• Radiography
• Stroke Volume
• beta-Thalassemia (complications, diagnostic imaging, drug therapy, metabolism, physiopathology)