title: Cardiac iron removal and functional cardiac improvement by different iron chelation regimens in thalassemia major patients.
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.
authors.affiliation: Hereditary Anemia Centre, Department of Internal Medicine, Ca' Granda Foundation Ospedale Maggiore Policlinico IRCCS, University of Milan, Milan, Italy.
authors.forename: Elena
authors.initials: E
authors.lastname: Cassinerio
authors.forename: Alberto
authors.initials: A
authors.lastname: Roghi
authors.forename: Patrizia
authors.initials: P
authors.lastname: Pedrotti
authors.forename: Francesca
authors.initials: F
authors.lastname: Brevi
authors.forename: Laura
authors.initials: L
authors.lastname: Zanaboni
authors.forename: Giovanna
authors.initials: G
authors.lastname: Graziadei
authors.forename: Paolo
authors.initials: P
authors.lastname: Pattoneri
authors.forename: Angela
authors.initials: A
authors.lastname: Milazzo
authors.forename: Maria Domenica
authors.initials: MD
authors.lastname: Cappellini
mesh_terms: D000328:Adult; D001565:Benzoates / Q000627:therapeutic use*; D015897:Comorbidity; D000077588:Deferasirox; D000077543:Deferiprone; D003676:Deferoxamine / Q000008:administration & dosage / Q000627:therapeutic use*; D004359:Drug Therapy, Combination; D005260:Female; D005500:Follow-Up Studies; D006321:Heart / Q000503:physiopathology*; D006333:Heart Failure / Q000188:drug therapy / Q000209:etiology / Q000517:prevention & control*; D006801:Humans; D007502:Iron Chelating Agents / Q000627:therapeutic use*; D008297:Male; D009206:Myocardium / Q000737:chemistry* / Q000473:pathology; D010349:Patient Compliance; D011446:Prospective Studies; D011728:Pyridones / Q000008:administration & dosage / Q000627:therapeutic use*; D013318:Stroke Volume; D014230:Triazoles / Q000627:therapeutic use*; D055815:Young Adult; D017086:beta-Thalassemia / Q000150:complications* / Q000378:metabolism / Q000473:pathology
chemical_list: D001565:Benzoates; D007502:Iron Chelating Agents; D011728:Pyridones; D014230:Triazoles; D000077543:Deferiprone; D003676:Deferoxamine; D000077588:Deferasirox