Myocardial iron clearance during reversal of siderotic cardiomyopathy with intravenous desferrioxamine: a prospective study using T2* cardiovascular magnetic resonance.
Heart failure from iron overload causes 71% of deaths in thalassaemia major, yet reversal of siderotic cardiomyopathy has been reported. In order to determine the changes in myocardial iron during treatment, we prospectively followed thalassaemia patients commencing intravenous desferrioxamine for iron-induced cardiomyopathy during a 12-month period. Cardiovascular magnetic resonance assessments were performed at baseline, 3, 6 and 12 months of treatment, and included left ventricular (LV) function and myocardial and liver T2*, which is inversely related to iron concentration. One patient died. The six survivors showed progressive improvements in myocardial T2* (5.1 +/- 1.9 to 8.1 +/- 2.8 ms, P = 0.003), liver iron (9.6 +/- 4.3 to 2.1 +/- 1.5 mg/g, P = 0.001), LV ejection fraction (52 +/- 7.1% to 63 +/- 6.4%, P = 0.03), LV volumes (end diastolic volume index 115 +/- 17 to 96 +/- 3 ml, P = 0.03; end systolic volume index 55 +/- 16 to 36 +/- 6 ml, P = 0.01) and LV mass index (106 +/- 14 to 95 +/- 13, P = 0.01). Iron cleared more slowly from myocardium than liver (5.0 +/- 3.3% vs. 39 +/- 23% per month, P = 0.02). These prospective data confirm that siderotic heart failure is often reversible with intravenous iron chelation with desferrioxamine. Myocardial T2* improves in concert with LV volumes and function during recovery, but iron clearance from the heart is considerably slower than from the liver.
Anderson
Lisa J
LJ
Cardiovascular Magnetic Resonance Unit, Royal Brompton Hospital, London, UK.
Westwood
Mark A
MA
Holden
Sally
S
Davis
Bernard
B
Prescott
Emma
E
Wonke
Beatrix
B
Porter
John B
JB
Walker
J Malcolm
JM
Pennell
Dudley J
DJ
D000328:Adult; D009202:Cardiomyopathies / Q000188:drug therapy / Q000209:etiology / Q000378:metabolism*; D016022:Case-Control Studies; D003676:Deferoxamine / Q000008:administration & dosage*; D005260:Female; D006801:Humans; D007262:Infusions, Intravenous; D007501:Iron / Q000378:metabolism*; D007502:Iron Chelating Agents / Q000008:administration & dosage*; D019190:Iron Overload / Q000188:drug therapy / Q000209:etiology / Q000378:metabolism*; D008099:Liver / Q000378:metabolism; D008279:Magnetic Resonance Imaging; D008297:Male; D009206:Myocardium / Q000378:metabolism*; D011446:Prospective Studies; D013789:Thalassemia / Q000150:complications / Q000378:metabolism; D018487:Ventricular Dysfunction, Left
D007502:Iron Chelating Agents; D007501:Iron; D003676:Deferoxamine