Right and left ventricular function and myocardial scarring in adult patients with sickle cell disease: a comprehensive magnetic resonance assessment of hepatic and myocardial iron overload

Journal of Cardiovascular Magnetic Resonance

Research

Daniel B P Lima¹  Sidney R Sales¹  Claudio M A O Lima¹  Guilherme M Cunha¹  Flávia P Junqueira²  Tadeu T A Kubo²  Marly Uellendhal³  Edson Marchiori⁴  Viviani L R de Pessoa⁵  Clarisse L C Lobo⁵  Carolina A S Cunha⁵  Juliano L Fernandes⁶ 

[1] Clínica de Diagnóstico Por Imagem (CDPI), Av. das Américas 4666 sala 325,Barra da Tijuca, 22649-900RJ, Rio de Janeiro, Brazil;Clínica de Diagnóstico Por Imagem (CDPI), Av. das Américas 4666 sala 325,Barra da Tijuca, 22649-900RJ, Rio de Janeiro, Brazil;Department of Radiology, Federal University of Rio de Janeiro, Rio de Janeiro, Brazil;Delboni Auriemo Medicina Diagnóstica, São Paulo, Brazil;Department of Radiology, Federal University of Rio de Janeiro, Rio de Janeiro, Brazil;HEMORIO, Rio de Janeiro, Brazil;Universidade Estadual de Campinas, Campinas, Brazil;

关键词: Cardiovascular Magnetic Resonance;    Sickle Cell Disease;    Right Ventricle;    Late Gadolinium Enhancement;    Liver Iron Concentration;   

DOI  :  10.1186/1532-429X-15-83

 received in 2013-05-24, accepted in 2013-09-11,  发布年份 2013

来源: Springer

【 摘 要 】

BackgroundPatients with Sickle cell disease (SCD) who receive regular transfusions are at risk for developing cardiac toxicity from iron overload. The aim of this study was to assess right and left cardiac volumes and function, late gadolinium enhancement (LGE) and iron deposits in patients with SCD using CMR, correlating these values with transfusion burden, ferritin and hemoglobin levels.MethodsThirty patients with SCD older than 20 years of age were studied in a 1.5 T scanner and compared to age- and sex-matched normal controls. Patients underwent analysis of biventricular volumes and function, LGE and T2* assessment of the liver and heart.ResultsWhen compared to controls, patients with SCD presented higher left ventricular (LV) volumes with decreased ejection fraction (EF) with an increase in stroke volume (SV) and LV hypertrophy. The right ventricle (RV) also presented with a decreased EF and hypertrophy, with an increased end-systolic volume. Although twenty-six patients had increased liver iron concentrations (median liver iron concentration value was 11.83 ± 9.66 mg/g), only one patient demonstrated an abnormal heart T2* < 20 msec. Only four patients (13%) LGE, with only one patient with an ischemic pattern.ConclusionsAbnormal heart iron levels and myocardial scars are not a common finding in SCD despite increased liver iron overload. The significantly different ventricular function seen in SCD compared to normal suggests the changes in RV and LV function may not be due to the anemia alone. Future studies are necessary to confirm this association.

【 授权许可】

CC BY   
© Junqueira et al.; licensee BioMed Central Ltd. 2013