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.
Abstract

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