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Published on: June 11, 2019
Multiparametric Cardiac Magnetic Resonance Survey in Children With Thalassemia Major: A Multicenter Study
Maddalena Casale1, Antonella Meloni1, Aldo Filosa1
1From the Centro per la Cura delle Microcitemie, Cardarelli Hospital, Napoli, Italy (M.C., A.F.); Dipartimento della Donna, del Bambino e di Chirurgia Generale e Specialistica, Seconda Università di Napoli, Napoli, Italy (M.C.); Cardiovascular MR Unit, Fondazione G. Monasterio CNR-Regione Toscana, Pisa, Italy (A.M., M.G.N., V.P., A.P.); Ematologia-Emoglobinopatie, Civico Hospital-ARNAS, Palermo, Italy (L.C.); Centro Microcitemia, "Garibaldi" Hospital, Catania, Italy (V.C.); Oncoematologia Pediatrica, Policlinico di Modena, Modena; Italy (G.P.); Pediatria, Adolescentologia e Talassemia, Arcispedale "S.Anna", Ferrara, Italy (M.R.G.); Ematologia II con Talassemia, Ospedale "V. Cervello", Palermo, Italy (L.P., A.M.); Clin. di Emato-Oncologia Pediatrica, Dip. di Pediatria, Università di Padova/Azienda Ospedaliera, Padova, Italy (M.C.P.); U.O. Microcitemie, A.O. "Bianchi-Melacrino-Morelli", Reggio Calabria, Italy (D.G.D'A.); Centro Talassemie ed Emoglobinopatie, Ospedale Meyer, Firenze, Italy (T.C.); Ematologia, Osp. "A. Perrino", SS per Mesagne, Km 7 Brindisi, Italy (A.Q.); Unità Operativa Sistemi Informatici, Fondazione G. Monasterio CNR-Regione Toscana, Pisa, Italy (C.S.); Istituto di Radiologia, Policlinico "Paolo Giaccone", Palermo, Italy (P.T., M.M.); and Radiology Department, University of Ancona, Ancona, Italy (G.V.).
Insights
Pediatric thalassemia major patients can experience early cardiac damage. Multiparametric cardiovascular magnetic resonance (CMR) is crucial for assessing myocardial iron overload and fibrosis, guiding early chelation therapy. Early T2* CMR assessment is recommended for pediatric patients.
Area of Science:
- Cardiology
- Pediatric Hematology
- Radiology
Background:
- Cardiovascular magnetic resonance (CMR) is vital for managing thalassemia major.
- Limited data exist on CMR in pediatric thalassemia major patients.
- This study retrospectively assessed myocardial iron overload, function, and fibrosis in pediatric patients.
Purpose of the Study:
- To evaluate myocardial iron overload, function, and fibrosis in pediatric thalassemia major patients using multiparametric CMR.
- To identify predictors of cardiac iron overload.
- To determine the optimal timing for CMR assessments in this population.
Main Methods:
- Retrospective analysis of 107 pediatric thalassemia major patients.
- T2* multiecho CMR for myocardial and liver iron quantification.
- Cine images for atrial dimensions and biventricular function.
- Late gadolinium enhancement (LGE) for myocardial fibrosis detection.
- Scans performed without sedation.
Main Results:
- 21.4% of patients had significant myocardial iron overload, linked to lower chelation compliance.
- Serum ferritin ≥2000 ng/mL and liver iron concentration ≥14 mg/g/dw predicted cardiac iron.
- Myocardial fibrosis (LGE) found in 15.8%, correlated with lower T2* values and negative remodeling.
- 77.6% had pathological liver iron concentration.
Conclusions:
- Cardiac damage is detectable early in pediatric thalassemia major patients via multiparametric CMR.
- Early T2* CMR assessment, without sedation, is recommended to tailor chelation.
- Late gadolinium enhancement CMR should be considered later, in teenage years.
Background:
Cardiovascular magnetic resonance (CMR) plays a key role in the management of thalassemia major patients, but few data are available in pediatric population. This study aims at a retrospective multiparametric CMR assessment of myocardial iron overload, function, and fibrosis in a cohort of pediatric thalassemia major patients.
Methods And Results:
We studied 107 pediatric thalassemia major patients (61 boys, median age 14.4 years). Myocardial and liver iron overload were measured by T2* multiecho technique. Atrial dimensions and biventricular function were quantified by cine images. Late gadolinium enhancement images were acquired to detect myocardial fibrosis. All scans were performed without sedation. The 21.4% of the patients showed a significant myocardial iron overload correlated with lower compliance to chelation therapy (P<0.013). Serum ferritin ≥2000 ng/mL and liver iron concentration ≥14 mg/g/dw were detected as the best threshold for predicting cardiac iron overload (P=0.001 and P<0.0001, respectively). A homogeneous pattern of myocardial iron overload was associated with a negative cardiac remodeling and significant higher liver iron concentration (P<0.0001). Myocardial fibrosis by late gadolinium enhancement was detected in 15.8% of the patients (youngest children 13 years old). It was correlated with significant lower heart T2* values (P=0.022) and negative cardiac remodeling indexes. A pathological magnetic resonance imaging liver iron concentration was found in the 77.6% of the patients.
Conclusions:
Cardiac damage detectable by a multiparametric CMR approach can occur early in thalassemia major patients. So, the first T2* CMR assessment should be performed as early as feasible without sedation to tailor the chelation treatment. Conversely, late gadolinium enhancement CMR should be postponed in the teenager age.
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