4.7 Article

Predictors of subclinical systemic sclerosis primary heart involvement characterised by microvasculopathy and myocardial fibrosis

Journal

RHEUMATOLOGY
Volume 60, Issue 6, Pages 2934-2945

Publisher

OXFORD UNIV PRESS
DOI: 10.1093/rheumatology/keaa742

Keywords

SSc primary heart involvement; cardiovascular magnetic resonance; risk stratification

Categories

Funding

  1. Scleroderma and Raynaud's UK
  2. ACORN charity
  3. Charitable Foundation Fellowship, Leeds Teaching Hospital
  4. National Institute of Health Research (NIHR) [ICA-CL-2016-02-017]
  5. NIHR [11/117/27]
  6. British Heart Foundation Personal Chair [CH/16/2/32089]
  7. National Institutes of Health Research (NIHR) [ICA-CL-2016-02-017] Funding Source: National Institutes of Health Research (NIHR)

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The subclinical phenotype of SSc primary heart involvement is characterized by myocardial microvasculopathy, diffuse and focal myocardial fibrosis while retaining preserved myocardial contractile function. This phenotype is associated with high-sensitivity troponin I, N-terminal pro-brain natriuretic peptide, SSc disease severity, and complicated peripheral vasculopathy, providing insights into potential underlying pathophysiological processes and risk factors for SSc-pHI.
Objectives. SSc primary heart involvement (SSc-pHI) is a significant cause of mortality. We aimed to characterize and identify predictors of subclinical SSc-pHI using cardiovascular MRI. Methods. A total of 83 SSc patients with no history of cardiovascular disease or pulmonary arterial hypertension and 44 healthy controls (HCs) underwent 3Tesla contrast-enhanced cardiovascular MRI, including T1 mapping and quantitative stress perfusion. High-sensitivity troponin I and N-terminal pro-brain natriuretic peptide were also measured. Results. Cardiovascular MRI revealed a lower myocardial perfusion reserve in the SSc patients compared with HCs {median (interquartile range (IQR)] 1.9 (1.6-2.4) vs 3 (2-3.6), P < 0.0011. Late gadolinium enhancement, indicating focal fibrosis, was observed in 17/83 patients but in none of the HCs, with significantly higher extracellular volume (ECV), suggestive of diffuse fibrosis, in SSc vs HC [mean (s.D.) 31 (4) vs 25 (2), P < 0.001]. Presence of late gadolinium enhancement and higher ECV was associated with skin score [odds ratio (OR)=1.115, P=0.048; R-2 =0.353, P=0.004], and ECV and myocardial perfusion reserve was associated with the presence of digital ulcers at multivariate analysis (R-2 =0.353, P <0.001; R-2 =0.238, P =0.011). High-sensitivity troponin I was significantly higher in patients with late gadolinium enhancement, and N-terminal pro-brain natriuretic peptide was associated with ECV (P < 0.05). Conclusion. Subclinical SSc-pHI is characterized by myocardial microvasculopathy, diffuse and focal myocardial fibrosis but preserved myocardial contractile function. This subclinical phenotype of SSc-pHI was associated with high-sensitivity troponin I, N-terminal pro-brain natriuretic peptide, SSc disease severity and complicated peripheral vasculopathy. These data provide information regarding the underlying pathophysiological processes and provide a basis for identifying individuals at risk of SSc-pHI.

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