Antonella Meloni1,2, Laura Pistoia1, Vincenzo Positano1,2, Antonio De Luca3, Nicola Martini1,2, Anna Spasiano4, Ilaria Fotzi5, Pier Paolo Bitti6, Domenico Visceglie7, Gianna Alberini8, Gianfranco Sinagra3, Alessia Pepe9, Filippo Cademartiri10. 1. Department of Radiology, Fondazione G. Monasterio CNR-Regione Toscana, Via Moruzzi, 1 -, 56124, Pisa, Italy. 2. U.O.C. Bioingegneria, Fondazione G. Monasterio CNR-Regione Toscana, Pisa, Italy. 3. Cardiovascular Department, University of Trieste, Trieste, Italy. 4. Unità Operativa Semplice Dipartimentale Malattie Rare del Globulo Rosso, Azienda Ospedaliera di Rilievo Nazionale "A. Cardarelli", Napoli, Italy. 5. Centro Talassemie ed Emoglobinopatie, Ospedale "Meyer", Firenze, Italy. 6. Servizio Immunoematologia e Medicina Trasfusionale - Dipartimento dei Servizi, Presidio Ospedaliero "San Francesco" ASL Nuoro, Nuoro, Italy. 7. Servizio di Immunoematologia e Medicina Trasfusionale, A.S.L. di Bari, Ospedale "Di Venere", Bari, Italy. 8. U.O.C. INFOTEL Translational BioInformatics and eHealth, Fondazione G. Monasterio CNR-Regione Toscana, Pisa, Italy. 9. Institute of Radiology, Department of Medicine, University of Padua, Padua, Italy. 10. Department of Radiology, Fondazione G. Monasterio CNR-Regione Toscana, Via Moruzzi, 1 -, 56124, Pisa, Italy. fcademartiri@ftgm.it.
Abstract
OBJECTIVES: Myocardial extracellular volume (ECV) by cardiovascular magnetic resonance (CMR) is a surrogate marker of diffuse fibrosis. We evaluated the association between ECV and demographics, CMR findings, and cardiac involvement in patients with thalassemia major (TM). METHODS: A total of 108 β-TM patients (62 females, 40.16 ± 8.83 years), consecutively enrolled in the Extension-Myocardial Iron Overload in Thalassemia Network, and 16 healthy subjects (6 females, 37.12 ± 16.13 years) underwent CMR. The protocol included assessment of T2*, native T1, and T2 values in all 16 myocardial segments for myocardial iron overload (MIO) quantification, cine images for left ventricular (LV) function quantification, post-contrast T1 mapping for ECV calculation, and late gadolinium enhancement (LGE) technique for replacement myocardial fibrosis detection. RESULTS: Global ECV values were significantly higher in females than in males. Global ECV values were significantly higher in patients with significant MIO (global heart T2* < 20 ms) than in patients without significant MIO, and both groups exhibited higher global ECV values than healthy subjects. No association was detected between native T1 and ECV values, while patients with reduced global heart T2 values showed significantly higher global ECV values than patients with normal and increased global heart T2. Global ECV values were not correlated with LV function/size and were comparable between patients with and without LGE. Compared to patients without heart failure, patients with a history of heart failure (N = 10) showed significantly higher global heart ECV values. CONCLUSION: In TM, increased myocardial ECV, potentially reflecting diffuse interstitial fibrosis, is associated with MIO and heart failure. KEY POINTS: • CMR-derived myocardial extracellular volume is increased in thalassemia major patients, irrespective of the presence of late gadolinium enhancement. • In thalassemia major, myocardial iron overload contributes to the increase in myocardial ECV, which potentially reflects diffuse interstitial fibrosis and is significantly associated with a history of heart failure.
OBJECTIVES: Myocardial extracellular volume (ECV) by cardiovascular magnetic resonance (CMR) is a surrogate marker of diffuse fibrosis. We evaluated the association between ECV and demographics, CMR findings, and cardiac involvement in patients with thalassemia major (TM). METHODS: A total of 108 β-TM patients (62 females, 40.16 ± 8.83 years), consecutively enrolled in the Extension-Myocardial Iron Overload in Thalassemia Network, and 16 healthy subjects (6 females, 37.12 ± 16.13 years) underwent CMR. The protocol included assessment of T2*, native T1, and T2 values in all 16 myocardial segments for myocardial iron overload (MIO) quantification, cine images for left ventricular (LV) function quantification, post-contrast T1 mapping for ECV calculation, and late gadolinium enhancement (LGE) technique for replacement myocardial fibrosis detection. RESULTS: Global ECV values were significantly higher in females than in males. Global ECV values were significantly higher in patients with significant MIO (global heart T2* < 20 ms) than in patients without significant MIO, and both groups exhibited higher global ECV values than healthy subjects. No association was detected between native T1 and ECV values, while patients with reduced global heart T2 values showed significantly higher global ECV values than patients with normal and increased global heart T2. Global ECV values were not correlated with LV function/size and were comparable between patients with and without LGE. Compared to patients without heart failure, patients with a history of heart failure (N = 10) showed significantly higher global heart ECV values. CONCLUSION: In TM, increased myocardial ECV, potentially reflecting diffuse interstitial fibrosis, is associated with MIO and heart failure. KEY POINTS: • CMR-derived myocardial extracellular volume is increased in thalassemia major patients, irrespective of the presence of late gadolinium enhancement. • In thalassemia major, myocardial iron overload contributes to the increase in myocardial ECV, which potentially reflects diffuse interstitial fibrosis and is significantly associated with a history of heart failure.
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