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Long-term longitudinal prospective CMR study in patients with thalassemia major

Session CMR for outcome prediction

Speaker Antonella Meloni

Event : ESC Congress 2018

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

Authors : A Pepe (Pisa,IT), L Pistoia (Pisa,IT), T Casini (Firenze,IT), S Renne (Lamezia Terme,IT), L Tedesco (Locri,IT), S Pulini (Pescara,IT), V Santamaria (Vibo Valentia,IT), E Facchini (Bologna,IT), P Keilberg (Pisa,IT), A Riva (Taranto,IT), A Vallone (Catania,IT), V Positano (Pisa,IT), A Meloni (Pisa,IT)

A. Pepe1 , L. Pistoia1 , T. Casini2 , S. Renne3 , L. Tedesco4 , S. Pulini5 , V. Santamaria6 , E. Facchini7 , P. Keilberg1 , A. Riva8 , A. Vallone9 , V. Positano1 , A. Meloni1 , 1Fondazione G. Monasterio CNR-Regione Toscana - Pisa - Italy , 2Ospedale “Meyer” - Firenze - Italy , 3P.O. “Giovanni Paolo II” - Lamezia Terme - Italy , 4Presidio Ospedaliero di Locri - A.S.P. di Reggio Calabria - Locri - Italy , 5Osped. Civile “Spirito Santo” - Pescara - Italy , 6A.S.P. Vibo Valentia - Vibo Valentia - Italy , 7University Hospital Policlinic S. Orsola-Malpighi - Bologna - Italy , 8OSP. SS. Annunziata ASL Taranto - Taranto - Italy , 9Azienda Ospedaliera “Garibaldi” Presidio Ospedaliero Nesima - Catania - Italy ,

Cardiac Magnetic Resonance

European Heart Journal ( 2018 ) 39 ( Supplement ), 39

Introduction: According to the International Guidelines, thalassemia major (TM) patients should perform a complete cardiac evaluation, including a CMR scan, every year. However, prospective CMR studies are limited beyond 3 years and longer-term studies are, therefore, important.

Aim: We aimed to determine longitudinal changes in cardiac iron and function assessed by CMR over 6 years in a large cohort of TM patients.

Methods: We considered 426 TM patients (205 males; 30.87±8.21 years) consecutively enrolled in the MIOT (Myocardial Iron Overload in Thalassemia) Network with a CMR follow-up (FU) study at 72 months (6 years).

Myocardial iron overload (MIO) was quantified by the multislice multiecho T2* technique. Biventricular function was quantified by cine images.

Results: Four patterns of MIO were identified: no MIO (all segments with T2*≥20 ms), heterogeneous MIO and global heart T2*≥20 ms, heterogeneous MIO and global heart T2*<20 ms, and homogeneous MIO (all segments with T2*<20 ms). An improvement in cardiac iron levels was detected in the 72% of patients showing MIO at the baseline (at least one pathologic segment) while, globally, a worsening was detected in 40 patients (see Figure).

Biventricular end-diastolic volume indexes (EDVI) were significantly lower at the FU CMR. In patients with significant baseline MIO (global heart T2*<20 ms) a significant decrease in all biventricular volumes and a significant increase in left ventricular ejection fraction (EF) (mean difference: 3.83±8.48%, P<0.0001) as well as in right ventricular EF (mean difference: 1.79±9.04%, P=0.042) were detected with a concordant improvement of MIO status.

The 50.7% of the patients changed the type of chelator during the FU based on CMR results. The percentage of patients who changed the chelation therapy was significantly higher in patients with significant MIO than in patients without MIO (60.2% vs 46.2%; P=0.008).

Conclusion: Over a period of 6 years, the continuous monitoring of cardiac iron levels and a tailored chelation therapy allowed an improvement in more than 70% of patients with baseline MIO and a consequent improvement of biventricular function.

Figure 1

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