Cardiovascular magnetic resonance in systemic sclerosis: “Pearls and pitfalls”

Abstract Systemic sclerosis (SSc) is an autoimmune disease characterized by vascular dysfunction and excessive fibrosis, involving internal organs including the heart. The estimated prevalence of cardiac involvement in SSc is high and remains subclinical until the late stages. It is either primary, related to myocardial inflammation and fibrosis, or secondary, due to pulmonary arterial hypertension (SSc-PAH) or systemic hypertension, in those patients with renal involvement. Cardiovascular magnetic resonance (CMR) is a useful tool for the early assessment of cardiac involvement in SSc. It is the gold standard technique to assess ventricular volumes,ejection fraction, and in particular is very useful to reliably and non-invasively detect myocardial inflammation, early perfusion defects, and myocardial fibrosis. However, the CMR evaluation in SSc may be problematic, because of cardiac and respiratory artefacts, commonly found in these patients. Therefore, a high level of expertise is necessary for both acquisition and interpretation of CMR images in SSc.