Multiparametric Cardiac Magnetic Resonance Survey in Children With Thalassemia Major A Multicenter Study

作者：Casale Maddalena; Meloni Antonella; Filosa Aldo; Cuccia Liana; Caruso Vincenzo; Palazzi Giovanni; Gamberini Maria Rita; Pitrolo Lorella; Putti Maria Caterina; D'Ascola Domenico Giuseppe; Casini Tommaso; Quarta Antonella; Maggio Aurelio; Neri Maria Giovanna; Positano Vincenzo; Salvatori Cristina; Toia Patrizia; Valeri Gianluca; Midiri Massimo; Pepe Alessia^*

来源：Circulation-Cardiovascular Imaging, 2015, 8(8): e003230.

DOI：10.1161/CIRCIMAGING.115.003230

• children
• ferritins
• magnetic resonance imaging
• thalassemia major

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.

• 出版日期2015-8