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Prospective changes of left ventricular iron and function by MR in pediatric thalassemia major patients treated with different chelators or not chelated

Session Poster session 2

Speaker Antonella Meloni

Event : ESC Congress 2015

  • Topic : imaging
  • Sub-topic : Cardiac Magnetic Resonance (CMR)
  • Session type : Poster Session

Authors : A Meloni (Pisa,IT), L Pitrolo (Palermo,IT), MG Neri (Pisa,IT), C Salvatori (Pisa,IT), B Pagano (Locri (RC),IT), P Preziosi (Roma,IT), M Missere (Campobasso,IT), G Valeri (Ancona,IT), V Positano (Pisa,IT), A Pepe (Pisa,IT)

A. Meloni1 , L. Pitrolo2 , M.G. Neri1 , C. Salvatori3 , B. Pagano4 , P. Preziosi5 , M. Missere6 , G. Valeri7 , V. Positano1 , A. Pepe1 , 1Fondazione G. Monasterio CNR-Regione Toscana, CMR Unit - Pisa - Italy , 2Ospedale Cervello-Villa Sofia, Ematologia II con Talassemia - Palermo - Italy , 3Fondazione G. Monasterio CNR-Regione Toscana, Unità Operativa Sistemi Informatici - Pisa - Italy , 4PO Locri - A.S.L. n. 9, Centro Microcitemico, U.O. di Pediatria e Neonatologia - Locri (RC) - Italy , 5Policlinico “Casilino”, U.O.C. Diagnostica per Immagini e Interventistica - Roma - Italy , 6Università Cattolica del Sacro Cuore, Dipartimento di Radiologia - Campobasso - Italy , 7Azienda Ospedaliero-Universitaria Ospedali Riuniti “Umberto I-Lancisi-Salesi”, Dipartimento di Radiologia - Ancona - Italy ,

Cardiovascular Magnetic Resonance (CMR)

European Heart Journal ( 2015 ) 36 ( Abstract Supplement ), 247

Background: There are no prospective studies comparing the effectiveness of the three iron chelators commercially available in preventing or decreasing myocardial iron overload (MIO) in pediatric thalassemia major (TM) patients.

Purpose: Our aim was to evaluate the changes in cardiac iron and function by quantitative magnetic resonance imaging (MRI) over a follow-up (FU) of 18 months in pediatric TM patients treated with one of the 3 available iron chelators in monotherapy or non chelated.

Methods: Among the first 1611 TM patients enrolled in the MIOT (Myocardial Iron Overload in Thalassemia) network, we considered pediatric patients who had maintained the same chelation regimen between the two MRI scans. MIO was quantified by a multislice multiecho T2* sequence. Function parameters were evaluated by cine images.

Results: Four groups of patients were identified: 6 patients (3 F, 10.0±2.2 years) treated with desferioxamine (DFO– mean dosage 43.7±6.8 mg/kg/die), 7 patients (3 F, 15.5±1.7 yrs) treated with deferiprone (DFP– 75.0±9.2 mg/kg/die), 39 patients (13 F, 13.6±3.4 yrs) treated with deferasirox (DFX– 26.6±6.7 mg/kg/die), and 2 patients (2 F, 11.1±5.3 yrs) not chelated because they had performed a bone marrow transplantation.

At baseline in DFO, DFP and no-chelated groups no patient showed a global heart T2* value<20 ms. In all 4 groups all patients who showed no MIO at baseline maintained at the FU the same status. At baseline in DFX group 5 patients had heart T2* values<20 ms. The 4 patients with intermediate cardiac iron (T2* 10–20 ms) at the baseline showed no iron at the FU while the patient with severe MIO (T2*<10 ms) remained in the same status at the FU. Non chelated patients had higher global heart T2* values at baseline (non-chelated 37.7±0.5 ms > DFP 35.3±4.9 ms>DFX 32.7±9.6 ms>DFO 31.9±10.5 ms) while DFP patients had higher global heart T2* values at FU (DFP 39.5±6.1 ms>DFX 34.2±7.3 ms DFO 33.6±7.9 ms> on-chelated 28.9±4.0 ms).

In the DFO group at baseline 1 patient showed pathological left ventricular ejection fraction (LVEF) and he recovered at the follow up. In the DFP group at baseline 2 patients showed pathological LVEF and both recovered at the FU. In the DFX group at baseline 3 patients showed pathological LVEF: 2 recovered at the FU and 1 did not perform the evaluation of the cardiac function at FU due to technical reasons. Conversely 9 patients with normal LVEF at baseline showed pathological LVEF at the FU.

Conclusion: In this young population, DFP and DFO seem to be more effective versus the MIO with a concordant positive effect on the global systolic function.

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