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- Open Access
Characterisation of sub-clinical primary myocardial disease in systemic sclerosis - preliminary findings from a cardiac magnetic resonance study
© Erhayiem et al.; licensee BioMed Central Ltd. 2014
- Published: 16 January 2014
- Cardiac Magnetic Resonance
- Interstitial Lung Disease
- Systemic Sclerosis
- Myocardial Perfusion Reserve
- Cardiac Magnetic Resonance Study
Systemic sclerosis (SSc) is a chronic disease characterised by systemic inflammation, vasculopathy and fibrosis. Primary myocardial disease occurs in both limited (lcSSc) and diffuse (dcSSc) cutaneous subtypes, and carries a poor prognosis. The natural history is poorly understood, with no clear approach to identifying the 'at-risk' patient. CMR studies in SSc have rarely correlated with disease phenotype. Our objective is to determine cardiovascular manifestations of SSc using multi-component CMR.
Eleven patients fulfilling SSc ACR/Le-Roy criteria, without known CV disease or diabetes, underwent CMR at 3.0T (Philips Achieva TX). Data from 10 healthy subjects served as a control group. Standard bSSFP cine images were acquired and LV dimensions calculated. First-pass perfusion imaging in three short-axis LV slices was performed during administration of 0.1 mmol/kg of gadobutrol at 3 minutes of 140 mcg/kg/min adenosine for stress and repeated 15 minutes later at rest. Myocardial perfusion reserve (MPR) was calculated using Fermi deconvolution (PMI v.0.4, [Sourbron, 2009] with basal blood pool providing the arterial input. Native and 15 minute post final contrast T1 maps were generated from mid-LV short axis using a modified 3,3,5 Look-Locker inversion sequence to calculate extra-cellular volume (ECV) fraction.
CMR measurements in eleven patients with systemic sclerosis
End-diastolic volume index (ml/m2)
End-systolic volume index (ml/m2)
Stroke volume index (ml/m2)
LV ejection fraction index (%/m2)
LV mass index (g/m2)
Extra-cellular volume fraction (%)
Myocardial perfusion reserve
CMR demonstrates lower MPR in patients with SSc than in controls. Our preliminary data suggest that quantitative CMR may also be able to detect differences in myocardial involvement in subtypes of the disease, justifying future larger studies.
1. Raynaud's and scleroderma association. 2. IMID cardiovascular outcomes program grant, Schering Plough.
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