The Systemic Sclerosis Heart

SSc can affect a variety of cardiac structures: it can cause myocardial abnormalities (including myocardial fibrosis, left ventricular [LV] systolic dysfunction, and LV diastolic dysfunction), coronary microvascular dysfunction, pericardial disease, and electrophysiologic abnormalities, including brady- and tachyarrhythmias. Cardiac involvement in SSc is a leading risk factor for death, and approximately 25% of deaths due to SSc are attributable to primary cardiac causes. It is therefore critical that clinicians involved in the care of SSc, including those conducting hematopoietic stem cell transplantation (HSCT) in SSc, understand the epidemiology, pathophysiology, screening, diagnosis, and management of cardiac manifestations of SSc.