TY - JOUR
T1 - Predictors of subclinical systemic sclerosis primary heart involvement characterised by microvasculopathy and myocardial fibrosis
AU - Dumitru, R B
AU - Bissell, L A
AU - Erhayiem, B
AU - Fent, G
AU - Swoboda, P
AU - Abignano, G
AU - Donica, H
AU - Burska, A
AU - Greenwood, J P
AU - Biglands, J
AU - Del Galdo, F
AU - Plein, S
AU - Buch, Maya
PY - 2020/9/26
Y1 - 2020/9/26
N2 - Objectives
Systemic sclerosis primary heart involvement (SSc-pHI) is a significant cause of mortality. We aimed to characterise and identify predictors of subclinical SSc-pHI using cardiovascular magnetic resonance (CMR).
Methods
Eighty-three SSc patients with no history of cardiovascular disease or pulmonary arterial hypertension and 44 healthy controls (HC) underwent 3Tesla contrast-enhanced CMR including T1 mapping and quantitative stress perfusion. High-sensitivity troponin I (Hs-TnI) and N-terminal pro-brain natriuretic peptide (NT-proBNP) were also measured.
Results
CMR revealed lower myocardial perfusion reserve (MPR) compared to HC [median (IQR) 1.9 (1.6, 2.4) vs 3 (2, 3.6), p<0.001]. Late gadolinium enhancement (LGE), indicating focal fibrosis was observed in 17/83 patients but in no HC, with significantly higher extracellular volume (ECV), suggestive of diffuse fibrosis in SSc vs HC [mean (SD) 31 (4) vs 25 (2), p<0.001]. Presence of LGE and higher ECV associated with skin score (OR=1.115, p=0.048; R2=0.353, p=0.004), and ECV and MPR associated with the presence of digital ulcers at multivariate analysis (R2=0.353, p<0.001; R2=0.238, p=0.011). Hs-TnI was significantly higher in patients with LGE, and NT-proBNP associated with ECV (p<0.05).
Conclusion
Subclinical SSc-pHI is characterised by myocardial microvasculopathy, diffuse and focal myocardial fibrosis but preserved myocardial contractile function. This subclinical phenotype of SSc-pHI associates with Hs-TnI, NT-proBNP, SSc disease severity and complicated peripheral vasculopathy. These data inform on the underlying pathophysiological processes and provide a basis to identify individuals at risk of SSc-pHI.
AB - Objectives
Systemic sclerosis primary heart involvement (SSc-pHI) is a significant cause of mortality. We aimed to characterise and identify predictors of subclinical SSc-pHI using cardiovascular magnetic resonance (CMR).
Methods
Eighty-three SSc patients with no history of cardiovascular disease or pulmonary arterial hypertension and 44 healthy controls (HC) underwent 3Tesla contrast-enhanced CMR including T1 mapping and quantitative stress perfusion. High-sensitivity troponin I (Hs-TnI) and N-terminal pro-brain natriuretic peptide (NT-proBNP) were also measured.
Results
CMR revealed lower myocardial perfusion reserve (MPR) compared to HC [median (IQR) 1.9 (1.6, 2.4) vs 3 (2, 3.6), p<0.001]. Late gadolinium enhancement (LGE), indicating focal fibrosis was observed in 17/83 patients but in no HC, with significantly higher extracellular volume (ECV), suggestive of diffuse fibrosis in SSc vs HC [mean (SD) 31 (4) vs 25 (2), p<0.001]. Presence of LGE and higher ECV associated with skin score (OR=1.115, p=0.048; R2=0.353, p=0.004), and ECV and MPR associated with the presence of digital ulcers at multivariate analysis (R2=0.353, p<0.001; R2=0.238, p=0.011). Hs-TnI was significantly higher in patients with LGE, and NT-proBNP associated with ECV (p<0.05).
Conclusion
Subclinical SSc-pHI is characterised by myocardial microvasculopathy, diffuse and focal myocardial fibrosis but preserved myocardial contractile function. This subclinical phenotype of SSc-pHI associates with Hs-TnI, NT-proBNP, SSc disease severity and complicated peripheral vasculopathy. These data inform on the underlying pathophysiological processes and provide a basis to identify individuals at risk of SSc-pHI.
M3 - Article
SN - 1462-0324
JO - Rheumatology (Oxford)
JF - Rheumatology (Oxford)
ER -