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* =  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: Department of Pediatrics, Memorial Sloan-Kettering Cancer Center, New York, NY, USA.
authors: Srikanth R
authors: SR
authors: Ambati
authors: Rachel E
authors: RE
authors: Randolph
authors: Kevin
authors: K
authors: Mennitt
authors: Dorothy A
authors: DA
authors: Kleinert
authors: Jonathan W
authors: JW
authors: Weinsaft
authors: Patricia J
authors: PJ
authors: Giardina
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D005260:Female; D005500:Follow-Up Studies; D006321:Heart / Q000000981:diagnostic imaging / Q000503:physiopathology; D006331:Heart Diseases / Q000000981:diagnostic imaging / Q000209:etiology / Q000378:metabolism / Q000503:physiopathology; D006486:Hemosiderosis / Q000000981:diagnostic imaging / Q000209:etiology / Q000503:physiopathology; D006801:Humans; D007502:Iron Chelating Agents / Q000008:administration & dosage; D008099:Liver / Q000000981:diagnostic imaging / Q000378:metabolism; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D008991:Monitoring, Physiologic / Q000379:methods; D009206:Myocardium / Q000378:metabolism*; D011859:Radiography; D013318:Stroke Volume; D017086:beta-Thalassemia / Q000150:complications / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000378:metabolism / Q000503:physiopathology
chemical_list: D007502:Iron Chelating Agents
references: J Cardiovasc Magn Reson. 2010 Apr 23;12:24
references: 20416084
references: Eur J Haematol. 1989 Apr;42(4):382-8
references: 2470615
references: Haematologica. 2004 Oct;89(10):1187-93
references: 15477202
references: Am J Hematol. 2011 Jan;86(1):92-5
references: 21061309
references: Circulation. 2009 Nov 17;120(20):1961-8
references: 19801505
references: N Engl J Med. 1994 Sep 1;331(9):574-8
references: 8047081
references: Blood. 2005 Aug 15;106(4):1460-5
references: 15860670
references: Eur Heart J. 2001 Dec;22(23):2171-9
references: 11913479
references: Blood. 2004 Jul 1;104(1):34-9
references: 14988152
references: Am J Hematol. 2012 Jul;87(7):732-3
references: 22622672
references: Blood. 2012 Mar 22;119(12):2746-53
references: 22279056
references: J Magn Reson Imaging. 2007 Jun;25(6):1147-51
references: 17520718
references: Radiology. 2008 Sep;248(3):1004-12
references: 18710989
references: Br J Haematol. 2004 Nov;127(3):348-55
references: 15491298
references: Blood Rev. 2008 Dec;22 Suppl 2:S14-21
references: 19059052
references: Haematologica. 2011 Jan;96(1):41-7
references: 20884710
references: Haematologica. 2011 Feb;96(2):e5-6; author reply e7-8
references: 21282714
references: Circulation. 2011 Apr 12;123(14):1519-28
references: 21444881
references: Blood. 2008 Oct 1;112(7):2973-8
references: 18650452
references: Ann N Y Acad Sci. 2005;1054:273-82
references: 16339675
references: Blood. 2010 Mar 25;115(12):2364-71
references: 19996412
references: Br J Haematol. 2005 Jan;128(1):2
references: 15606544
references: Am J Med. 1971 Aug;51(2):209-21
references: 5095527
references: Haematologica. 2012 Jun;97(6):842-8
references: 22271905
references: Haematologica. 2011 Jan;96(1):48-54
references: 21071497
references: Haematologica. 2011 Jul;96(7):1055-8
references: 21393329
references: JACC Cardiovasc Imaging. 2008 Sep;1(5):572-8
references: 19356483
references: J Magn Reson Imaging. 2010 Apr;31(4):845-53
references: 20373428
references: Am J Hematol. 2012 Feb;87(2):139-44
references: 22052662
references: Circulation. 1964 Nov;30:698-705
references: 14226168
references: Am J Hematol. 2011 May;86(5):433-6
references: 21523808
references: J Cardiovasc Magn Reson. 2006;8(3):543-7
references: 16755844
references: Eur J Haematol. 2007 Jan;78(1):58-65
references: 17038018
references: Blood. 2010 Jul 29;116(4):537-43
references: 20421452