Systemic sclerosis and cardiac dysfunction: evolving concepts and diagnostic methodologies

Abstract

Primary myocardial involvement is common in systemic sclerosis. There is strong evidence that this involvement is related to repeat focal ischemic injury causing irreversible myocardial fibrosis. Clinically evident cardiac involvement is recognized to be a poor prognostic factor; thus preclinical identification is highly encouraged. The severity of heart involvement has been confirmed and patients having systemic sclerosis with antitopoisomerase I antibodies seem to be at higher risk. Echocardiography, coupled, if possible, to pulsed tissue Doppler, is the cornerstone of heart assessment. Myocardial perfusion may be assessed by single photon emission computed tomography. When available, cardiac magnetic resonance imaging should be considered as it allows simultaneous measurement of volumes and ventricular function, myocardial perfusion, and assessment of possible inflammation and/or fibrosis. As a result of recent innovations, clinicians have a large panel of methods - some of these possibly reserved for research--whereas the others seem to be widely available and suitable for routine clinical practice. Indeed, conventional echocardiography, pulsed tissue Doppler, and natriuretic peptides may be used for routine assessment.