Maddalena Casale1, Antonella Meloni1, Aldo Filosa1, Liana Cuccia1, Vincenzo Caruso1, Giovanni Palazzi1, Maria Rita Gamberini1, Lorella Pitrolo1, Maria Caterina Putti1, Domenico Giuseppe D'Ascola1, Tommaso Casini1, Antonella Quarta1, Aurelio Maggio1, Maria Giovanna Neri1, Vincenzo Positano1, Cristina Salvatori1, Patrizia Toia1, Gianluca Valeri1, Massimo Midiri1, Alessia Pepe2. 1. From 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.). 2. From 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.). alessia.pepe@ftgm.it.
Abstract
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.
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.
Authors: Tamer H Hassan; Mohamed M Abdel Salam; Marwa Zakaria; Mohamed Shehab; Dina T Sarhan; El Sayed H Zidan; Khaled M El Gerby Journal: Indian J Hematol Blood Transfus Date: 2018-11-08 Impact factor: 0.900