Subclinical myocardial inflammation and diffuse fibrosis are common in systemic sclerosis – a clinical study using myocardial T1-mapping and extracellular volume quantification

Journal of Cardiovascular Magnetic Resonance

Research

Paul M Matthews¹  James Moon²  Paul B Wordsworth³  Stefan Neubauer⁴  Vanessa M Ferreira⁴  Jane M Francis⁴  Aitzaz BS Rai⁴  Ntobeko AB Ntusi⁴  Theodoros D Karamitsos⁴  Stefan K Piechnik⁴  Matthew D Robson⁴ 

[1] GlaxoSmithKline Clinical Imaging Centre, London, UK;Division of Brain Sciences, Department of Medicine, Imperial College, London, UK;Institute of Cardiovascular Science, University College London & Heart Hospital, London, UK;Nuffield Department of Orthopaedics & NIHR Oxford Musculoskeletal Biomedical Research Unit, Rheumatology and Musculoskeletal Sciences, University of Oxford, Nuffield Orthopaedic Centre and John Radcliffe Hospital, Oxford, UK;Radcliffe Department of Medicine, Division of Cardiovascular Medicine, Level 0, John Radcliffe Hospital, University of Oxford Centre for Clinical Magnetic Resonance Research, OX3 9DU, Oxford, United Kingdom;

关键词: Scleroderma;    Cardiovascular magnetic resonance;    T1 mapping;    Extracellular volume estimation;    Gadolinium;   

DOI  :  10.1186/1532-429X-16-21

 received in 2013-11-11, accepted in 2014-02-17,  发布年份 2014

来源: Springer

【 摘 要 】

BackgroundSystemic sclerosis (SSc) is characterised by multi-organ tissue fibrosis including the myocardium. Diffuse myocardial fibrosis can be detected non-invasively by T1 and extracellular volume (ECV) quantification, while focal myocardial inflammation and fibrosis may be detected by T2-weighted and late gadolinium enhancement (LGE), respectively, using cardiovascular magnetic resonance (CMR). We hypothesised that multiparametric CMR can detect subclinical myocardial involvement in patients with SSc.Methods19 SSc patients (18 female, mean age 55 ± 10 years) and 20 controls (19 female, mean age 56 ± 8 years) without overt cardiovascular disease underwent CMR at 1.5T, including cine, tagging, T1-mapping, T2-weighted, LGE imaging and ECV quantification.ResultsFocal fibrosis on LGE was found in 10 SSc patients (53%) but none of controls. SSc patients also had areas of myocardial oedema on T2-weighted imaging (median 13 vs. 0% in controls). SSc patients had significantly higher native myocardial T1 values (1007 ± 29 vs. 958 ± 20 ms, p < 0.001), larger areas of myocardial involvement by native T1 >990 ms (median 52 vs. 3% in controls) and expansion of ECV (35.4 ± 4.8 vs. 27.6 ± 2.5%, p < 0.001), likely representing a combination of low-grade inflammation and diffuse myocardial fibrosis. Regardless of any regional fibrosis, native T1 and ECV were significantly elevated in SSc and correlated with disease activity and severity. Although biventricular size and global function were preserved, there was impairment in the peak systolic circumferential strain (-16.8 ± 1.6 vs. -18.6 ± 1.0, p < 0.001) and peak diastolic strain rate (83 ± 26 vs. 114 ± 16 s-1, p < 0.001) in SSc, which inversely correlated with diffuse myocardial fibrosis indices.ConclusionsCardiac involvement is common in SSc even in the absence of cardiac symptoms, and includes chronic myocardial inflammation as well as focal and diffuse myocardial fibrosis. Myocardial abnormalities detected on CMR were associated with impaired strain parameters, as well as disease activity and severity in SSc patients. CMR may be useful in future in the study of treatments aimed at preventing or reducing adverse myocardial processes in SSc.

【 授权许可】

Unknown   
© Ntusi et al.; licensee BioMed Central Ltd. 2014. This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly credited. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.