Impact of heart magnetic resonance imaging on chelation choices, compliance with treatment and risk of heart disease in patients with thalassaemia major

Abstract

SummaryThis study aimed to verify the impact of heart magnetic resonance imaging on chelation choices and patient compliance in a single-institution cohort as well as its predictive value for heart failure and arrhythmias. Abnormal cardiac T2* values determined changes in treatment in most subjects. Heart T2* was confirmed to be highly predictive over 1 year for heart failure and arrhythmias. The choice of chelation regimens known to remove heart iron efficiently was not sufficient by itself to influence the risk. Compliance with treatment had a more remarkable role.Keywords: beta thalassaemia major, heart magnetic resonance imaging, compliance, heart failure, arrhythmia.The introduction of magnetic resonance imaging (MRI) technology to measure iron overload and the availability of the oral chelators have had a profound impact on the care and prognosis of patients with beta thalassaemia major (Modell et al, 2008;Chouliaras et al, 2011). Identifying those patients at risk of a fall in left ventricular ejection fraction, cardiac T2* also identifies those whose chelation treatment should be intensified. Kirk et al (2009) determined the predictive value of cardiac T2* magnetic resonance for heart failure and arrhythmia in thalassaemia major. The present study aimed to verify the impact of heart MRI on chelation choices and patient compliance as well as its predictive value for heart failure and arrhythmias in patients attending our Centre. MethodsA total of 313 patients (900 consecutive scans) with beta thalassaemia major followed at DH talassemia Et a EvolutivaOspedale Regionale per le Microcitemie-Cagliari, Italy were included in this study. MRI scans were performed with a 1Á5 T General Electric CVi scanner or with a 1Á5 T Magneton Siemens Avanto, using previously reported techniques (Westwood et al, 2003), between 2002 and 2012. Of these patients, 157 were male and 156 female, with a mean age at time of their first scan of 26Á7 AE 6Á2 years. Twenty-two had experienced one or more episodes of heart failure and 13 a history of arrhythmia. At the time of the first scan, 168 patients were receiving deferoxamine (20-50 mg/kg 5-6 d per week), 24 were on deferiprone (75-100 mg/kg per d), 76 were taking deferoxamine (20-50 mg/kg 2-7 d per week) combined with deferiprone (75 mg/kg per d) because of high serum ferritin values and 45 were receiving deferasirox at a dose of 20-35 mg/kg per d. Adherence to and acceptance of chelation therapy (compliance) was evaluated according to pharmacy records of the dispensed drug. It was classified as good, if the mean number of the doses taken was ≥80% of those prescribed, average if it was >50 < 80% and poor if it was <50%. The predictive value of T2* for cardiac events in the year after scan was evaluated including the MRI before heart failure and/or arrhythmias for patients with cardiac events, and a random MRI otherwise. To obtain the most similar control group we randomly sampled one MRI within 'time to first MRI' stratas, to respect case group distribution.Accuracy of T2* predict...

Effects of deferasirox-deferoxamine on myocardial and liver iron in patients with severe transfusional iron overload

“…As thalassemia patients require lifelong chelation therapy, patient satisfaction and adherence should also be considered. 22 Ultimately, the objective should be to maintain a normalized iron burden with oral monotherapy instead of cumbersome combination therapy. However, greater understanding of when patients can switch from combination to monotherapy is needed.…”

Cardiac Hemosiderosis in Transfusion DependentThalassemia: A Mini-Review

“…19,21 In fact, CMRI procedure could be a reliable method based on the chelation therapeutic strategy. 22 However, CMRI as the gold standard method for cardiac iron assessment is not available in many care centers especially in third world nations such as Iran. 8 Besides, due to late appearance of the sings of cardiac involvement in echocardiograms and electrocardiograms, using these two methods may not retrieve favorable results for detection of initial phases of cardiac iron deposition.…”

How we manage iron overload in sickle cell patients

“…All three chelators are very effective at controlling iron individually or in combination. Overwhelmingly, the dominant reason for failure of chelation therapy is failure of the patient to take the prescribed medicine (Origa et al, 2013). While there are data showing different absorption rates for some chelators (Chirnomas et al, 2009), the effect of poor adherence to recommended therapy is the dominant cause of treatment failure.…”