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Multiparametric cardiac magnetic resonance in patients with thalassemia intermedia: new insights from the E-MIOT network

  • Cardiac Radiology
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Abstract

Purpose

In a relatively large cohort of thalassemia intermedia (TI) patients, we systematically investigated myocardial iron overload (MIO), function, and replacement fibrosis using cardiac magnetic resonance (CMR), we assessed the clinical determinants of global heart T2* values, and we explored the association between multiparametric CMR findings and cardiac complications.

Materials and methods

We considered 254 beta-TI patients (43.14 ± 13.69 years, 138 females) consecutively enrolled in the Extension-Myocardial Iron Overload in Thalassemia project. MIO was quantified by T2* technique and biventricular function and atrial areas by cine images. Macroscopic myocardial fibrosis was detected by late gadolinium enhancement technique.

Results

Compared to never/sporadically transfused patients, regularly transfused (RT)-TI patients exhibited significantly lower global heart T2* values, biventricular end-diastolic volume indexes, left ventricular mass index, and cardiac index. In RT-TI patients, age and serum ferritin levels were the strongest predictors of global heart T2* values. Independently from the transfusional state, cardiac T2* values were not associated with biventricular function.

Of the 103 (40.6%) patients in whom the contrast medium was administrated, 27 (26.2%) had replacement myocardial fibrosis. Age, sex distribution, cardiac iron, and biventricular function parameters were comparable between patients without and without replacement myocardial fibrosis. Twenty-five (9.8%) patients had a history of cardiac complications (heart failure and arrhythmias). Increased age and replacement myocardial fibrosis emerged as significant risk markers for cardiac complications.

Conclusions

In TI, regular transfusions are associated with less pronounced cardiac remodeling but increase the risk of MIO. Replacement myocardial fibrosis is a frequent finding associated with cardiac complications.

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Acknowledgements

We would like to thank all the colleagues involved in the E-MIOT project (https://emiot.ftgm.it/). We thank all patients for their cooperation.

This work is generated within the European Reference Network on Rare Hematological Diseases (ERN-EuroBloodNet).

Funding

The E-MIOT project received “no-profit support” from industrial sponsorships (Chiesi Farmaceutici S.p.A. and Bayer). The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript.

Author information

Authors and Affiliations

  1. Department of Radiology, Fondazione G. Monasterio CNR-Regione Toscana, Via Moruzzi, 1, 56124, Pisa, Italy

    Antonella Meloni, Laura Pistoia, Vincenzo Positano & Filippo Cademartiri

  2. U.O.C. Bioingegneria, Fondazione G. Monasterio CNR-Regione Toscana, Pisa, Italy

    Antonella Meloni & Vincenzo Positano

  3. U.O.C. Ricerca Clinica, Fondazione G. Monasterio CNR-Regione Toscana, Pisa, Italy

    Laura Pistoia

  4. U.O.S.D. Malattie Rare del Globulo Rosso, Azienda Ospedaliera Di Rilievo Nazionale “A. Cardarelli”, Naples, Italy

    Paolo Ricchi

  5. Unità Operativa Day Hospital Della Talassemia e delle Emoglobinopatie, Azienda Ospedaliero-Universitaria ‘‘S. Anna’’, Cona, FE, Italy

    Filomena Longo

  6. Struttura Semplice di Microcitemia, Ospedale “SS. Annunziata” ASL Taranto, Taranto, Italy

    Valerio Cecinati

  7. Day Hospital Talassemici, Ospedale ‘‘Sant’Eugenio’’, Rome, Italy

    Francesco Sorrentino

  8. Unità Operativa Complessa di Radiologia, ‘‘ARNAS’’ Civico, Di Cristina Benfratelli, Palermo, Italy

    Liana Cuccia

  9. Ematologia Microcitemia, Ospedale San Giovanni di Dio – ASP Crotone, Crotone, Italy

    Elisabetta Corigliano

  10. U.O.C. Ematologia, Ospedale di Cosenza, Cosenza, Italy

    Vincenza Rossi

  11. Diagnostica per Immagini e Radiologia Interventistica, Ospedale del Delta, Lagosanto, FE, Italy

    Riccardo Righi

  12. Unità Operativa Complessa Diagnostica per Immagini, Ospedale ‘‘Sandro Pertini’’, Rome, Italy

    Priscilla Fina

  13. Struttura Complessa di Cardioradiologia-UTIC, Presidio Ospedaliero “Giovanni Paolo II”, Lamezia Terme, Italy

    Stefania Renne

  14. U.O.C. Radiologia Generale e di Pronto Soccorso, Azienda Ospedaliera di Rilievo Nazionale “A. Cardarelli”, Naples, Italy

    Luigi Barbuto

Authors
  1. Antonella Meloni
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  2. Laura Pistoia
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  3. Paolo Ricchi
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  4. Filomena Longo
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  5. Valerio Cecinati
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  6. Francesco Sorrentino
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  7. Liana Cuccia
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  8. Elisabetta Corigliano
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  9. Vincenza Rossi
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  14. Vincenzo Positano
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  15. Filippo Cademartiri
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Contributions

AM designed the study, analyzed the data, and drafted the initial manuscript. LP was responsible for data collection. PR, FL, VC, FS, LC, MA, VR, RR, PF, SR, and LB collected the data. VP developed the software for image analysis. FC supervised the study and is the guarantor of this work. All authors assisted with interpretation, commented on drafts of the manuscript, and approved the final version.

Corresponding author

Correspondence to Filippo Cademartiri.

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Conflict of interest

The authors have nothing to disclose.

Ethical approval

This study was performed in line with the principles of the Declaration of Helsinki. Approval was granted by the Ethics Committee of all MRI centers involved in the E-MIOT project.

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Informed consent was obtained from all patients included in the study.

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Meloni, A., Pistoia, L., Ricchi, P. et al. Multiparametric cardiac magnetic resonance in patients with thalassemia intermedia: new insights from the E-MIOT network. Radiol med (2024). https://doi.org/10.1007/s11547-024-01821-y

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