ArticleEuropean heart journal. Cardiovascular Imaging2024
Peripheral microvascular function is linked to cardiac involvement on cardiovascular magnetic resonance in systemic sclerosis-related pulmonary arterial hypertension.
Article in European heart journal. Cardiovascular Imaging, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 7 papers.
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Who cites it
7 citing papers in PubMed, 6 citations in OpenAlex.
- Mitochondrial Dysregulation and Molecular Signaling in Systemic Sclerosis Cardiac Disease: An Integrative Echocardiographic, Microvascular, and Biomarker Review.International journal of molecular sciences · 2026Review
- Primary heart involvement assessed by cardiac magnetic resonance is associated with capillaroscopy abnormalities in systemic sclerosis.Advances in rheumatology (London, England) · 2026Article
- Cardiac MRI-derived ventricular-pulmonary arterial coupling predicts outcomes in connective tissue disease-associated pulmonary arterial hypertension patients.European journal of radiology open · 2025Article
- Enhancing the quantification of post-occlusive reactive hyperemia: a multimodal optical approach.Pflugers Archiv : European journal of physiology · 2025Article
- Myocardial Disease in Systemic Sclerosis: Recent Updates and Clinical Implications.Current cardiology reports · 2025Review
- Imaging manifestations of hereditary hemorrhagic telangiectasia with pulmonary arterial hypertension: a case report.Frontiers in cardiovascular medicine · 2025Article
- Systemic sclerosis-associated pulmonary arterial hypertension is characterized by a distinct peripheral T helper cell profile.Rheumatology (Oxford, England) · 2024Article
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Authors and funding
11 authors at 2 institutions in 2 countries.
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Abstract
aimsSystemic sclerosis (SSc) is characterized by vasculopathy, inflammation, and fibrosis, and carries one of the worst prognoses if patients also develop pulmonary arterial hypertension (PAH). Although PAH is a known prognosticator, patients with SSc-PAH demonstrate disproportionately high mortality, presumably due to cardiac involvement. In this cross-sectional study, the relationship between cardiac involvement revealed by cardiovascular magnetic resonance (CMR) and systemic microvascular disease severity measured with nailfold capillaromicroscopy (NCM) in patients with SSc-PAH is evaluated and compared with patients with idiopathic PAH (IPAH). METHODS AND
resultsPatients with SSc-PAH and IPAH underwent CMR, echocardiography, and NCM with post-occlusive reactivity hyperaemia (PORH) testing on the same day. CMR imaging included T2 (oedema), native, and post-contrast T1 mapping to measure the extracellular volume fraction (ECV, fibrosis) and adenosine-stress-perfusion imaging measuring the relative myocardial upslope (microvascular coronary perfusion). Measures of peripheral microvascular function were related to CMR indices of oedema, fibrosis, and myocardial perfusion. SSc-PAH patients (n = 20) had higher T2 values and a trend towards a higher ECV, compared with IPAH patients (n = 5), and a lower nailfold capillary density (NCD) and reduced capillary recruitment after PORH. NCD correlated with ECV and T2 (r = -0.443 and -0.464, respectively, P < 0.05 for both) and with markers of diastolic dysfunction on echocardiography. PORH testing, but not NCD, correlated with the relative myocardial upslope (r = 0.421, P < 0.05).
conclusionSSc-PAH patients showed higher markers of cardiac fibrosis and inflammation, compared with IPAH patients. These markers correlated well with peripheral microvascular dysfunction, suggesting that SSc-driven inflammation and vasculopathy concurrently affect peripheral microcirculation and the heart. This may contribute to the disproportionate high mortality in SSc-PAH.
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