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

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.