scholarly journals Prevention of Cardiomyopathy in Transfusion-Dependent Homozygous Thalassaemia Today and the Role of Cardiac Magnetic Resonance Imaging

2009 ◽  
Vol 2009 ◽  
pp. 1-4 ◽  
Author(s):  
Athanassios Aessopos ◽  
Vasilios Berdoukas ◽  
Maria Tsironi

Transfusion and iron chelation therapy revolutionised survival and reduced morbidity in patients with transfusion-dependent beta thalassaemia major. Despite these improvements, cardiac disease remained the most common cause of death in those patients. Recently the ability to determine the degree of cardiac iron overload, through cardiac magnetic resonance imaging (CMR) has allowed more logical approaches to iron removal, particularly from the heart. The availability of two oral chelators, deferiprone and deferasirox has reduced the need for the injectable chelator deferrioxamine and an additional benefit has been that deferiprone has been shown to be more cardioprotective than deferrioxamine. This review on the prevention of cardiac disease makes recommendations on the chelation regime that would be desirable for patients according to their cardiac iron status as determined by CMR determined by CMR. It also discusses approaches to chelation management should CMR not be available.

2021 ◽  
Vol 22 (Supplement_1) ◽  
Author(s):  
D Patsourakos ◽  
C Aggeli ◽  
K Gatzoulis ◽  
S Delicou ◽  
Y Dimitroglou ◽  
...  

Abstract Funding Acknowledgements Type of funding sources: None. Introduction Beta-thalassemia major (β-TM) patients are subjected to iron overload as a consequence of chronic blood transfusions. The redundant iron affects, among other organs, the heart resulting in myocardial dysfunction. Iron deposition does not only affect ventricular myocardium but atrial myocardium as well. According to current practice, the overall myocardial iron status is estimated by calculating T2* at the middle section of the ventricular septum using cardiac magnetic resonance imaging (CMR). Current CMR technology limits the direct estimation of atrial iron status.  Purpose The current study evaluates left atrial function by novel echocardiographic techniques and correlates these findings with CMR data. Methods 37 β-TM patients (mean age 41.17 years (SD = 8.33), 51.4% male), were subjected to electrocardiography, echocardiography and CMR imaging and P wave axis, PR segment duration, left atrial volume index (LAVI), left atrial strain at reservoir phase (LASr) and T2* were measured.  Results No correlation between T2* and LASr (r = 0.253, p = 0.131) or T2* and LAVI (r = 0.044, p = 0.796) were found. However, a correlation was found between the echocardiographic parameters LAVI and LASr (r = -0.676, p <0.001). P wave axis did not correlate with any other parameter, while PR segment duration was correlated with LASr (r = -0.399, p = 0.015). Regression analysis revealed correlation between LAVI and LASr (r2 = 0.457, p <0.001). Conclusions The current study suggests that despite the undisputed contribution of CMR in left ventricular iron load estimation, it may be less accurate in atrial iron status estimation. Considering the limitations of current CMR technology regarding the iron status of the thin atrial and right ventricular walls, our study highlights the role of speckle tracking in combination with CMR imaging for a more comprehensive evaluation of β-TM patients. Correlation table P axis PR segment T2* LAVI LASr P axis 0,151 (p = 0,373) 0,112 (p = 0.,508) -0,140 (p = 0,410) -0,117 (p = 0,489) PR segment 0,151 (p = 0,373) 0,051 (p = 0,766) 0,278 (p = 0,096) -0,399 (p = 0,015) T2* 0,112 (p = 0.,508) 0,051 (p = 0,766) 0,044 (p = 0,796) 0,253 (p = 0,131) LAVI -0,140 (p = 0,410) 0,278 (p = 0,096) 0,044 (p = 0,796) -0,676 (p <0,001) LASr -0,117 (p = 0,489) -0,399 (p = 0,015) 0,253 (p = 0,131) -0,676 (p <0,001) T2*: obtained by cardiac magnetic resonance imaging LAVI left atrial volume index (ml/m2) LASr left atrial strain at reservoir phase Abstract Figure. Correlation between LASr and T2*


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