Prognostic Role of Multiparametric Cardiac Magnetic Resonance in Neo Transfusion-Dependent Thalassemia

Author:

Meloni Antonella12ORCID,Pistoia Laura23ORCID,Ricchi Paolo4ORCID,Maggio Aurelio5,Cecinati Valerio6,Longo Filomena7,Sorrentino Francesco8,Borsellino Zelia9,Salvo Alessandra10ORCID,Rossi Vincenza11,Grassedonio Emanuele12ORCID,Restaino Gennaro13ORCID,Renne Stefania14,Righi Riccardo15,Positano Vincenzo12ORCID,Cademartiri Filippo2ORCID

Affiliation:

1. Bioengineering Unit, Fondazione G. Monasterio CNR-Regione Toscana, 56124 Pisa, Italy

2. Department of Radiology, Fondazione G. Monasterio CNR-Regione Toscana, 56124 Pisa, Italy

3. Unità Operativa Complessa Ricerca Clinica, Fondazione G. Monasterio CNR-Regione Toscana, 56124 Pisa, Italy

4. Unità Operativa Semplice Dipartimentale Malattie Rare del Globulo Rosso, Azienda Ospedaliera di Rilievo Nazionale “A. Cardarelli”, 80131 Napoli, Italy

5. Ematologia II con Talassemia, Ospedale “V. Cervello”, 90100 Palermo, Italy

6. Struttura Semplice di Microcitemia, Ospedale “SS. Annunziata”, 74123 Taranto, Italy

7. Unità Operativa Day Hospital della Talassemia e delle Emoglobinopatie, Azienda Ospedaliero-Universitaria “S. Anna”, 44124 Cona, FE, Italy

8. Unità Operativa Semplice Dipartimentale Day Hospital Talassemici, Ospedale “Sant’Eugenio”, 00143 Rome, Italy

9. Unità Operativa Complessa Ematologia con Talassemia, ARNAS Civico “Benfratelli-Di Cristina”, 90134 Palermo, Italy

10. Operativa Semplice Talassemia, Presidio Ospedaliero “Umberto I”, 96100 Siracusa, Italy

11. Unità Operativa Complessa Ematologia, Ospedale di Cosenza, 87100 Cosenza, Italy

12. Sezione di Scienze Radiologiche, Dipartimento di Biopatologia e Biotecnologie Mediche, Policlinico “Paolo Giaccone”, 90127 Palermo, Italy

13. Unità Operativa Complessa Radiodiagnostica, Gemelli Molise SpA, Fondazione di Ricerca e Cura “Giovanni Paolo II”, 86100 Campobasso, Italy

14. Struttura Complessa di Cardioradiologia-UTIC, Presidio Ospedaliero “Giovanni Paolo II”, 88046 Lamezia Terme, CZ, Italy

15. Diagnostica per Immagini e Radiologia Interventistica, Ospedale del Delta, 44023 Lagosanto, FE, Italy

Abstract

Background: We prospectively evaluated the predictive value of multiparametric cardiac magnetic resonance (CMR) for cardiovascular complications in non-transfusion-dependent β-thalassemia (β-NTDT) patients who started regular transfusions in late childhood/adulthood (neo β-TDT). Methods: We considered 180 patients (38.25 ± 11.24 years; 106 females). CMR was used to quantify cardiac iron overload, biventricular function, and atrial dimensions, and to detect left ventricular (LV) replacement fibrosis. Results: During a mean follow-up of 76.87 ± 41.60 months, 18 (10.0%) cardiovascular events were recorded: 2 heart failures, 13 arrhythmias (10 supraventricular), and 3 cases of pulmonary hypertension. Right ventricular (RV) end-diastolic volume index (EDVI), RV mass index (MI), LV replacement fibrosis, and right atrial (RA) area index emerged as significant univariate prognosticators of cardiovascular complications. The low number of events prevented us from performing a multivariable analysis including all univariable predictors simultaneously. Firstly, a multivariable analysis including the two RV size parameters (mass and volume) was carried out, and only the RV MI was proven to independently predict cardiovascular diseases. Then, a multivariable analysis, including RV MI, RA atrial area, and LV replacement fibrosis, was conducted. In this model, RV MI and LV replacement fibrosis emerged as independent predictors of cardiovascular outcomes (RV MI: hazard ratio (HR) = 1.18; LV replacement fibrosis: HR = 6.26). Conclusions: Our results highlight the importance of CMR in cardiovascular risk stratification.

Funder

Chiesi Farmaceutici S.p.A.

ApoPharma Inc.

Publisher

MDPI AG

Reference66 articles.

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5. Cappellini, M.D., Cohen, A., Porter, J., Taher, A., and Viprakasit, V. (2014). Guidelines for the Management of Transfusion Dependent Thalassaemia (TDT), Thalassaemia International Federation. [3rd ed.].

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