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. The heart involvement in SSc 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. During the course of SSc, any structure of the heart may be affected leading to myocardial ischemia/fibrosis, pericarditis, myocarditis, conduction system defects, arrhythmias, valvular disease and eventually heart failure. While the proportion of deaths in SSc that are related to renal involvement and to pulmonary causes has substantially declined, cardiac related deaths remained unchanged over the last three decades. It is estimated today to be at approximately 15% of all SSc deaths and unfortunately, the early signs of cardiac involvement are usually non-specific and can be missed by the routine cardiac evaluation. 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.