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Reduction of cardiac outcomes in thalassemia major thanks to a ten-year national Italian networking

Session New insights from translational CMR

Speaker Antonella Meloni

Event : ESC Congress 2019

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

Authors : A Meloni (Pisa,IT), L Pistoia (Pisa,IT), N Giunta (Palermo,IT), N Schicchi (Ancona,IT), P Giuliano (Palermo,IT), V Vinci (Catania,IT), P Fina (Rome,IT), S Campisi (Siracusa,IT), A Massa (Olbia,IT), A Quarta (Brindisi,IT), V Positano (Pisa,IT), A Pepe (Pisa,IT)

Authors:
A. Meloni1 , L. Pistoia1 , N. Giunta2 , N. Schicchi3 , P. Giuliano2 , V. Vinci4 , P. Fina5 , S. Campisi6 , A. Massa7 , A. Quarta8 , V. Positano1 , A. Pepe1 , 1Fondazione Toscana Gabriele Monasterio - Pisa - Italy , 2Ospedale Civico - Palermo - Italy , 3University Hospital Riuniti of Ancona - Ancona - Italy , 4Garibaldi Hospital - Catania - Italy , 5Sandro Pertini Hospital - Rome - Italy , 6Umberto I Hospital - Siracusa - Italy , 7Osp. Giovanni Paolo II - Olbia - Italy , 8Ospedale “A. Perrino” - Brindisi - Italy ,

Topic(s):
Cardiac Magnetic Resonance

Citation:
European Heart Journal ( 2019 ) 40 ( Supplement ), 2565

Introduction: The MIOT (Myocardial Iron Overload in Thalassemia) Network was a network of thalassemia and CMR centers built in 2006 in order to assure homogeneous and standardized cardiac iron overload assessment for a significant number of patients.

Purpose: We describe the impact of this ten-year Network on cardiac iron, complications and deaths in patients with thalassemia major (TM).

Methods: 1746 TM patients (911 F; age 31.17±9.09 yrs) were enrolled in the MIOT Network. Myocardial iron overload (MIO) was quantified by the multislice multiecho T2* technique. Biventricular function was quantified by cine images.

Results: 1392 TM patients performed an end-of-study CMR. At the last CMR significantly higher global heart T2* values (35.44±10.69 ms vs 29.16±12.02 ms; P<0.0001) and a significant lower number of patients with global heart T2*<20 ms (26.3% vs 12.0%; P<0.0001) were detected. 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 T2*<20 ms). At the last CMR a significant higher frequency of patients with no MIO and a significant lower frequency for the other three patterns indicating MIO were found (Figure 1).

In patients with global heart T2*<20 ms a significant increase in left ventricular ejection fraction (EF) (difference: 3.2±8.5%, P<0.0001) as well as in right ventricular EF (difference: 1.2±8.9%, P=0.002) were detected.

Based on CMR results the 75% of the patients changed the chelation therapy. At the last CMR the percentage of patients with an excellent/good compliance was significantly higher (94.8% vs 92.2%%; P<0.0001).

The complete history of cardiac complications-CC (heart failure, arrhythmias, pulmonary hypertension, myocardial infarction, angina, myo/pericarditis, peripheral vascular disease) was present for 1062 patients. Out of the 1001 patients with resolved CC or without CC before the enrolment in the project, the 6.6% had a CC before the enrolment in the project. During the study, the frequency of CC was 4.4%, significantly lower (P=0.023). In particular, the frequency of heart failure (HF) was significantly lower (3.5% vs 0.8%, P<0.0001).

Forty-six patients died during the study. HF continues to be the leading cause of death (30.4% of all causes), but there was a consistent decline in HF mortality rate, that was 60.2% in an Italian study dated 2004. No patients died for arrhythmias while cancer was the second leading cause of death.

Conclusion: Over a period of 10 years, the continuous monitoring of cardiac iron levels and a tailored chelation therapy allowed a reduction of MIO in the 70% of patients, with consequent improvement of cardiac function and reduction of cardiac complications and mortality from MIO-related HF. So, a national networking was effective in improving the care and reducing cardiac outcomes of TM patients.

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