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A prospective CMR study of cardiac iron and function in non-trasfusion-dependent thalassemia intermedia patients treated with desferrioxamine

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), MP Carta (Cagliari,IT), C Paci (Montevarchi (AR),IT), S Macchi (Ravenna,IT), MG Neri (Pisa,IT), P Preziosi (Roma,IT), G Restaino (Campobasso,IT), V Positano (Pisa,IT), D De Marchi (Pisa,IT), A Pepe (Pisa,IT)

A. Meloni1 , M.P. Carta2 , C. Paci3 , S. Macchi4 , M.G. Neri1 , P. Preziosi5 , G. Restaino6 , V. Positano1 , D. De Marchi1 , A. Pepe1 , 1Fondazione G. Monasterio CNR-Regione Toscana, CMR Unit - Pisa - Italy , 2Microcitemico Hospital, Centro Talassemici Adulti - Cagliari - Italy , 3Ospedale S Maria alla Gruccia, Centro Trasfusionale - Montevarchi (AR) - Italy , 4Santa Maria delle Croci Hospital, Servizio Trasfusionale - Ravenna - Italy , 5Policlinico “Casilino”, U.O.C. Diagnostica per Immagini e Interventistica - Roma - Italy , 6Università Cattolica del Sacro Cuore, Dipartimento di Radiologia - Campobasso - Italy ,

Cardiovascular Magnetic Resonance (CMR)

European Heart Journal ( 2015 ) 36 ( Abstract Supplement ), 248

Background: In thalassemia intermedia (TI) patients no observational study prospectively evaluated in the real life the efficacy of the desferrioxamine (DFO) therapy in removing or preventing myocardial iron overload.

Purpose: The efficacy endpoint of this study is represented by the changes in cardiac T2* values and biventricular function parameters in non-transfusion dependent (NTD) TI patients after 18 months of desferrioxamine therapy.

Methods: Among the 325 TI patients enrolled in the MIOT (Myocardial Iron Overload in Thalassemia) network, we selected 129 TI patients NTD. We considered 29 patients who had been received DFO alone between the two MRI scans. Cardiac iron overload was assessed by the multislice multiecho T2* technique. Biventricular function parameters were quantified by cine SSFP sequences. Myocardial fibrosis was evaluated by late gadolinium enhancement (LGE) acquisitions.

Results: Mean age was 39.69±8.12 years and 14 (48.3%) patients were females. Patients started regular chelation therapy at a mean age of 21.92±15.89 years. The mean administered dosage of DFO via subcutaneous route was 38.46±10.27 mg/kg body weight on 3.32±1.54 days/week. The percentage of patients with excellent/good levels of compliance to the chelation treatment was 82.1%.

At baseline only one patient showed cardiac iron overload (global heart T2*=15.23 ms) but he recovered at the follow-up (FU) (global heart T2*=26.93 ms). All patients without cardiac iron maintained the same status at the FU.

Due mainly to technical reasons, cardiac function was assessed at both baseline and FU MRIs in 24 patients. At baseline all patients had a normal LV ejection fraction (EF) and 4 of them showed a reduced LV ejection fraction (LVEF) at the FU. No patient had a pathological RV EF. No significant change between the two MRIs was detected in biventricular volume indexes, biventricular EFs and LV mass index.

For 21 patients the presence of myocardial fibrosis was investigated at both baseline and FU MRIs, and this subgroup was considered. Three (14.3%) patients had myocardial fibrosis at the baseline, all with a non ischemic pattern. At the FU two new occurrences of non-ischemic myocardial fibrosis were detected.

Conclusions: In this small population of sporadically or non transfused TI patients, the DFO therapy showed 100% efficacy in maintaining a normal global heart T2* value but it did not prevent the worsening of the LV function and the occurrence of new myocardial fibrosis.

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