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Published on: March 14, 2017
Cardiac iron removal and functional cardiac improvement by different iron chelation regimens in thalassemia major
Elena Cassinerio1, Alberto Roghi, Patrizia Pedrotti
1Hereditary Anemia Centre, Department of Internal Medicine, Ca' Granda Foundation Ospedale Maggiore Policlinico IRCCS, University of Milan, Milan, Italy.
Insights
Iron chelation therapy in thalassemia major (TM) patients effectively removes cardiac iron, improving heart function. Deferasirox treatment showed significant improvements in left ventricular function, while combination therapy rapidly ameliorated cardiac iron levels.
Area of Science:
- Cardiology
- Hematology
- Medical Imaging
Background:
- Myocardial iron overload is a primary cause of heart failure and mortality in adult thalassemia major (TM) patients.
- Effective iron chelation therapy is crucial for managing cardiac complications in TM.
Purpose of the Study:
- To evaluate the efficacy of different iron chelation regimens in removing cardiac iron and improving cardiac function in TM patients.
- To assess changes in myocardial T2* values and left ventricular function using cardiac magnetic resonance (CMR).
Main Methods:
- Sixty-seven TM patients were divided into four groups based on chelation therapy: deferasirox, deferoxamine, combined deferoxamine/deferiprone, and deferiprone alone.
- T2* CMR was performed at baseline, 6-14 months, and 32 ± 7 months to assess myocardial iron levels.
- Left ventricular ejection fraction and volumes were analyzed to evaluate cardiac function.
Main Results:
- Progressive improvements in myocardial T2* were observed across different chelation groups.
- A significant improvement in left ventricular ejection fraction and reduction in ventricular volumes were noted in patients treated with deferasirox, particularly those with moderate baseline T2* values.
- Combination therapy demonstrated a rapid amelioration of cardiac T2* in patients with severe iron overload.
Conclusions:
- Rigorous compliance with iron chelation therapy, at appropriate doses, significantly improves myocardial T2* in TM patients.
- Deferasirox treatment is effective in improving left ventricular function.
- Combination therapy may offer faster improvement in cardiac T2* for severe siderosis.
Abstract:
Heart failure due to myocardial iron overload remains the leading cause of morbidity and mortality in adult thalassemia major (TM) patients. We evaluated the removal of cardiac iron and the changes of cardiac function by different iron chelation in TM patients by T2* cardiac magnetic resonance (CMR). Sixty-seven TM patients (27 males/40 females; mean age, 35 ± 6 years) on different chelation regimens underwent T2* CMR at baseline (t (0)), after 6-14 months (t (1)) and after 32 ± 7 months (t (2)). Patients were divided in four groups according to chelation treatment: group A (deferasirox), group B (deferoxamine), group C (combined treatment, deferoxamine plus deferiprone) and group D (deferiprone alone). Myocardial T2* at t (0) was <10 ms in 8 patients, between 10 and 20 ms in 22 patients and ≥ 20 ms in 37 patients. Progressive changes in T2* were observed at t (1) and t (2). Ten patients (10/36, 27.8 %) in group A, three patients (3/15, 20 %) in group B and three patients (3/12, 25 %) in group C moved from an abnormal T2* to normal values. We observed an improvement of left ventricular ejection fraction and a reduction of end-systolic and end-diastolic left ventricular volumes only in patients in group A with baseline cardiac T2* between 10 and 20 ms. Rigorous compliance to any chelation therapy at proper doses significantly improve myocardial T2*. Treatment with deferasirox significantly improves left ventricular function. Combination therapy seems to ameliorate cardiac T2* in a shorter period of time in severe siderosis.
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