Purpose
Cardiac complications are the main cause of death in thalassemia major (TM) patients [1].
Cardiovascular Magnetic Resonance (CMR) plays a key role in the management of TM,
allowing to assess cardiac iron burden,
biventricular dimension and function,
atrial dimensions,
and myocardial fibrosis.
The aim of this study was to determine the predictive value of CMR parameters for heart failure and arrhythmias in TM.
Methods and Materials
We followed prospectively 537 white TM patients enrolled in the Myocardial Iron Overload in Thalassemia (MIOT) Network [2].
In the MIOT Network CMR is performed using standardized techniques and myocardial iron overload (MIO) is assessed by a multislice approach [3].
Fifty patients were excluded from the analysis because a cardiac disease was present at the time of the first CMR.
The prognostic variables analyzed were retrieved from the MIOT database.
All variables showing an association with the outcome at the univariate Cox proportional hazards model...
Results
At baseline the mean age of the patients was 29.5±9.0 years,
222 were males and the mean serum ferritin level were 1742.49 ± 1592.72 ng/l.
The mean follow-up time was 58 ± 18 months.
After the first CMR scan only the 37.8% of the patients did not change the chelation regimen or the frequency/dosage of the chelators.
We recorded 19 episodes of heart failure,
diagnosed by clinicians based on symptoms,
signs and instrumental findings (electrocardiogram,
echocardiography and CMR) according to the current guidelines.
Male sex,...
Conclusion
We detected few cardiac events thanks to a CMR-guided,
patient-specific adjustment of the chelation therapy.
Severe and homogeneous myocardial iron overload,
myocardial fibrosis and ventricular dysfunction identify patients at high risk of heart failure.
Heart T2* doesn’t have any power in predicting arrhythmias while male sex and atrial dilation are independent prognosticators.
References
[1] Borgna-Pignatti C et al.
Haematologica 2004;89:1187-93.
[2] Meloni A et al.
Int J Med Inform 2009;78:503-12.
[3] Pepe A et al.
JMRI 2006;23(5):662-8.