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

doi:10.1007/s11547-024-01821-y

. 2024 Jun;129(6):879-889.

doi: 10.1007/s11547-024-01821-y. Epub 2024 Apr 29.

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

Antonella Meloni^{1

2}, Laura Pistoia^{1

3}, Paolo Ricchi⁴, Filomena Longo⁵, Valerio Cecinati⁶, Francesco Sorrentino⁷, Liana Cuccia⁸, Elisabetta Corigliano⁹, Vincenza Rossi¹⁰, Riccardo Righi¹¹, Priscilla Fina¹², Stefania Renne¹³, Luigi Barbuto¹⁴, Vincenzo Positano^{1

2}, Filippo Cademartiri¹⁵

Affiliations

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 U.O.C. Ricerca Clinica, Fondazione G. Monasterio CNR-Regione Toscana, Pisa, Italy.
⁴ U.O.S.D. Malattie Rare del Globulo Rosso, Azienda Ospedaliera Di Rilievo Nazionale "A. Cardarelli", Naples, Italy.
⁵ Unità Operativa Day Hospital Della Talassemia e delle Emoglobinopatie, Azienda Ospedaliero-Universitaria ''S. Anna'', Cona, FE, Italy.
⁶ Struttura Semplice di Microcitemia, Ospedale "SS. Annunziata" ASL Taranto, Taranto, Italy.
⁷ Day Hospital Talassemici, Ospedale ''Sant'Eugenio'', Rome, Italy.
⁸ Unità Operativa Complessa di Radiologia, ''ARNAS'' Civico, Di Cristina Benfratelli, Palermo, Italy.
⁹ Ematologia Microcitemia, Ospedale San Giovanni di Dio - ASP Crotone, Crotone, Italy.
¹⁰ U.O.C. Ematologia, Ospedale di Cosenza, Cosenza, Italy.
¹¹ Diagnostica per Immagini e Radiologia Interventistica, Ospedale del Delta, Lagosanto, FE, Italy.
¹² Unità Operativa Complessa Diagnostica per Immagini, Ospedale ''Sandro Pertini'', Rome, Italy.
¹³ Struttura Complessa di Cardioradiologia-UTIC, Presidio Ospedaliero "Giovanni Paolo II", Lamezia Terme, Italy.
¹⁴ U.O.C. Radiologia Generale e di Pronto Soccorso, Azienda Ospedaliera di Rilievo Nazionale "A. Cardarelli", Naples, Italy.
¹⁵ Department of Radiology, Fondazione G. Monasterio CNR-Regione Toscana, Via Moruzzi, 1, 56124, Pisa, Italy. fcademartiri@ftgm.it.

PMID: 38683500
DOI: 10.1007/s11547-024-01821-y

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

Antonella Meloni et al. Radiol Med. 2024 Jun.

. 2024 Jun;129(6):879-889.

doi: 10.1007/s11547-024-01821-y. Epub 2024 Apr 29.

Authors

Affiliations

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 U.O.C. Ricerca Clinica, Fondazione G. Monasterio CNR-Regione Toscana, Pisa, Italy.
⁴ U.O.S.D. Malattie Rare del Globulo Rosso, Azienda Ospedaliera Di Rilievo Nazionale "A. Cardarelli", Naples, Italy.
⁵ Unità Operativa Day Hospital Della Talassemia e delle Emoglobinopatie, Azienda Ospedaliero-Universitaria ''S. Anna'', Cona, FE, Italy.
⁶ Struttura Semplice di Microcitemia, Ospedale "SS. Annunziata" ASL Taranto, Taranto, Italy.
⁷ Day Hospital Talassemici, Ospedale ''Sant'Eugenio'', Rome, Italy.
⁸ Unità Operativa Complessa di Radiologia, ''ARNAS'' Civico, Di Cristina Benfratelli, Palermo, Italy.
⁹ Ematologia Microcitemia, Ospedale San Giovanni di Dio - ASP Crotone, Crotone, Italy.
¹⁰ U.O.C. Ematologia, Ospedale di Cosenza, Cosenza, Italy.
¹¹ Diagnostica per Immagini e Radiologia Interventistica, Ospedale del Delta, Lagosanto, FE, Italy.
¹² Unità Operativa Complessa Diagnostica per Immagini, Ospedale ''Sandro Pertini'', Rome, Italy.
¹³ Struttura Complessa di Cardioradiologia-UTIC, Presidio Ospedaliero "Giovanni Paolo II", Lamezia Terme, Italy.
¹⁴ U.O.C. Radiologia Generale e di Pronto Soccorso, Azienda Ospedaliera di Rilievo Nazionale "A. Cardarelli", Naples, Italy.
¹⁵ Department of Radiology, Fondazione G. Monasterio CNR-Regione Toscana, Via Moruzzi, 1, 56124, Pisa, Italy. fcademartiri@ftgm.it.

PMID: 38683500
DOI: 10.1007/s11547-024-01821-y

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.

Keywords: Cardiac magnetic resonance; Myocardial iron overload; Replacement myocardial fibrosis; Thalassemia intermedia.

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Magnetic Resonance Evaluation of Tissue Iron Deposition and Cardiac Function in Adult Regularly Transfused Thalassemia Intermedia Compared with Thalassemia Major Patients.
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References

1. Weatherall DJ (1997) The thalassaemias. Bmj 314:1675–1678 - PubMed - PMC - DOI
1. Sturgeon P, Itano HA, Bergren WR (1955) Genetic and biochemical studies of intermediate types of Cooley’s anaemia. Br J Haematol 1:264–277 - PubMed - DOI
1. Taher AT, Musallam KM, El-Beshlawy A et al (2010) Age-related complications in treatment-naive patients with thalassaemia intermedia. Br J Haematol 150:486–489 - PubMed - DOI
1. Taher AT, Musallam KM, Karimi M et al (2010) Overview on practices in thalassemia intermedia management aiming for lowering complication rates across a region of endemicity: the optimal care study. Blood 115:1886–1892 - PubMed - DOI
1. Haddad A, Tyan P, Radwan A, Mallat N, Taher A (2014) Beta-thalassemia intermedia: a bird’s-eye view. Turk J Haematol 31:5–16 - PubMed - PMC - DOI

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[1] Weatherall DJ (1997) The thalassaemias. Bmj 314:1675–1678 - PubMed - PMC - DOI

[2] Weatherall DJ (1997) The thalassaemias. Bmj 314:1675–1678 - PubMed - PMC - DOI

[3] Sturgeon P, Itano HA, Bergren WR (1955) Genetic and biochemical studies of intermediate types of Cooley’s anaemia. Br J Haematol 1:264–277 - PubMed - DOI

[4] Sturgeon P, Itano HA, Bergren WR (1955) Genetic and biochemical studies of intermediate types of Cooley’s anaemia. Br J Haematol 1:264–277 - PubMed - DOI

[5] Taher AT, Musallam KM, El-Beshlawy A et al (2010) Age-related complications in treatment-naive patients with thalassaemia intermedia. Br J Haematol 150:486–489 - PubMed - DOI

[6] Taher AT, Musallam KM, El-Beshlawy A et al (2010) Age-related complications in treatment-naive patients with thalassaemia intermedia. Br J Haematol 150:486–489 - PubMed - DOI

[7] Taher AT, Musallam KM, Karimi M et al (2010) Overview on practices in thalassemia intermedia management aiming for lowering complication rates across a region of endemicity: the optimal care study. Blood 115:1886–1892 - PubMed - DOI

[8] Taher AT, Musallam KM, Karimi M et al (2010) Overview on practices in thalassemia intermedia management aiming for lowering complication rates across a region of endemicity: the optimal care study. Blood 115:1886–1892 - PubMed - DOI

[9] Haddad A, Tyan P, Radwan A, Mallat N, Taher A (2014) Beta-thalassemia intermedia: a bird’s-eye view. Turk J Haematol 31:5–16 - PubMed - PMC - DOI

[10] Haddad A, Tyan P, Radwan A, Mallat N, Taher A (2014) Beta-thalassemia intermedia: a bird’s-eye view. Turk J Haematol 31:5–16 - PubMed - PMC - DOI

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

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

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