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Impaired myocardial perfusion is associated with extracellular volume expansion, disease activity and impaired strain and strain rate in systemic sclerosis: a cardiovascular magnetic resonance study
Journal of Cardiovascular Magnetic Resonance volume 17, Article number: Q71 (2015)
Background
Systemic sclerosis (SSc) is characterised by vascular dysfunction and multi-organ fibrosis, with the heart commonly involved. Cardiovascular disease (CVD) in SSc may be direct or indirect, but often remains subclinical. SSc patients with apparent cardiovascular clinical features are at greater risk of deterioration and premature cardiovascular death, often from complications of myocardial ischaemia. CMR first-pass perfusion detects myocardial ischaemia with great accuracy. We hypothesised that CMR first-pass perfusion would be able to differentiate between segmental (indicating epicardial coronary artery disease) and non-segmental subendocardial (indicating microvascular dysfunction) perfusion defects in patients with SSc; and that microvascular dysfunction (relating to chronic myocardial inflammation) was more frequent in SSc.
Methods
17 SSc patients (16 female, mean age 55 ± 9 years) and 17 matched controls (16 female, mean age 54 ± 10 years) were enrolled. All patients with known cardiovascular disease were excluded. Study participants underwent CMR at 1.5T and the assessments included cine, tagging, T1 mapping, T2-weighted, perfusion, late gadolinium imaging (0.15mmol/kg gadoderic acid - Dotarem®) and ECV quantification. Comorbid status, disease activity index (VDAI score) and duration of disease were recorded for each subject.
Results
Myocardial perfusion reserve index was 1.5 ± 0.3 and 2.0 ± 0.4 (p<0.001) in SSc and controls, respectively. Non-segmental (circumferential) subendocardial perfusion defects were seen in 41% of SSc and none (p<0.001) of controls studied. There was no significant difference in LV size, mass and ejection fraction between SSc patients and controls. Peak systolic circumferential strain and peak diastolic strain rate were impaired in patients. Impaired MPRI correlated with peak systolic strain (R -0.91, p<0.001) and peak diastolic strain rate (R 0.56, p<0.001) in SSc. Furthermore, abnormal MPRI correlated with VDAI (R -0.58, p=0.02) and ECV (R -0.56, p=0.04) in SSc.
Conclusions
Myocardial perfusion is impaired in asymptomatic SSc patients with apparently normal hearts. Abnormal perfusion correlates with strain, strain rate, disease activity and ECV in SSc. CMR can detect subclinical cardiovascular involvement in SSc.
Funding
This study was funded by investigator-led grants from Guerbet and GlaxoSmithKline.
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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/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. 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.
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Ntusi, N.A., Sever, E., Lockey, J. et al. Impaired myocardial perfusion is associated with extracellular volume expansion, disease activity and impaired strain and strain rate in systemic sclerosis: a cardiovascular magnetic resonance study. J Cardiovasc Magn Reson 17 (Suppl 1), Q71 (2015). https://doi.org/10.1186/1532-429X-17-S1-Q71
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DOI: https://doi.org/10.1186/1532-429X-17-S1-Q71