Abstract
Objective: Cardiac T2* magnetic resonance imaging (MRI) has recently attracted considerable attention as a non-invasive method for detecting iron overload in various organs in thalassemia major patients. This study aimed to identify the prevalence of cardiac siderosis in thalassemia major patients and evaluate cardiac T2* MRI for monitoring cardiac siderosis before and after patients receive iron chelation therapy and its relation to serum ferritin, left ventricular ejection fraction, and liver iron concentration. The information gathered would be used for the direct monitoring, detection, and treatment of complications early on. Methods: A total of 119 thalassemia major patients were recruited in the present study. The cardiac T2* MRI was compared to serum ferritin levels, liver iron concentration (LIC), and left ventricular ejection fraction. All patients were classified into four groups based on their cardiac siderosis as having normal, marginal, mild to moderate, or severe cardiac iron overload. At the follow-up at years one, three, and five, the cardiac T2* MRI, LIC, serum ferritin, and left ventricular ejection fraction (LVEF) were determined. Results: The prevalence of cardiac siderosis with cardiac T2* MRI ≤ 25 ms was 17.6% (n = 21). There was no correlation between cardiac T2* MRI and serum ferritin, liver iron concentration, and LVEF (p = 0.39, 0.54, and 0.09, respectively). During one year to five years’ follow-up periods, cardiac T2* MRI (ms) in patients with severe cardiac siderosis had significantly improved from 8.5 ± 1.49 at baseline to 33.9 ± 1.9 at five years (p < 0.0001). Patients with severe, mild-moderate, marginal, and no cardiac siderosis had median LIC (mg/g dw) of 23.9 ± 6.5, 21.6 ± 13.3, 25.3 ± 7.7, and 19.9 ± 5.5 at baseline, respectively. Conclusions: This study supports the use of cardiac T2* MRI to monitor cardiac iron overload in patients who have had multiple blood transfusions. Early diagnosis and treatment of patients at risk of cardiac siderosis is a reasonable method of reducing the substantial cardiac mortality burden associated with myocardial siderosis. Cardiac T2* MRI is the best test that can identify at-risk patients who can be managed with optimization of their chelation therapy.
Highlights
Introduction iationsPatients with thalassemia major typically require regular blood transfusions beginning in childhood
There was no significant difference in age, sex, thalassemia type, mean pre-transfusion hemoglobin level, mean baseline ferritin levels, liver iron concentration, and left ventricular ejection fraction among the four groups at baseline
As seen in recent studies in patients with thalassemia major (TM), cardiac T2* magnetic resonance imaging (MRI) tends to comply with iron measures by myocardial biopsy and post-mortem hearts [16,17,18]
Summary
Patients with thalassemia major typically require regular blood transfusions beginning in childhood. Heart failure due to post-transfusion iron overload is still the leading cause of death in this disease [1,2,3]. Chelation therapy has been shown to be effective in eliminating cardiac iron and in improving survival. Late in the course of the disease, life-threatening complications such as heart failure and arrhythmias develop. Cardiomyopathy is more common in patients with transfusion-dependent thalassemia (TDT), while pulmonary hypertension is more common in non-transfusiondependent thalassemia (NTDT), after splenectomy. If intense chelation is undertaken early, dilated cardiomyopathy induced by severe myocardial siderosis may be reversed, but clinical diagnosis is frequently delayed due to the late onset of symptoms
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