Cardiac Involvement: Evaluation and Management

Abstract

Cardiac involvement in systemic sclerosis (SSc) is common and can affect virtually any cardiac structure, thereby causing myocardial abnormalities (including myocardial fibrosis, left ventricular [LV] systolic dysfunction, and LV diastolic dysfunction), coronary microvascular ischemia, pericardial disease, conduction abnormalities (including brady- and tachyarrhythmias), and less commonly valvular disease. When present clinically, cardiac involvement is a risk factor for death, and approximately 25 % of deaths due to SSc are attributable to primary cardiac causes (i.e., in the absence of pulmonary arterial hypertension, interstitial lung disease, or significant renal disease). Therefore, understanding the pathophysiology, optimal screening, definitive diagnosis, and management of cardiac manifestations of SSc is essential for those involved in the care and research of SSc. Because of rapid advances in cardiac diagnostic technologies with ever-increasing sensitivity, the ability to detect preclinical cardiac involvement in SSc is now possible. Furthermore, detailed knowledge of these diagnostic techniques, which range from biomarkers to echocardiography to radiologic studies such as cardiac magnetic resonance imaging, is critical in order to differentiate between the range of possible cardiac manifestations in SSc, thereby ensuring a correct diagnosis and optimal treatment plan. Here we review the epidemiology, screening, diagnosis, treatment, and uncertainties of cardiac involvement in SSc. We discuss in detail each specific cardiac manifestation of SSc, reviewing several recent studies that have provided great insight. Finally, we offer a practical guide which can serve as a roadmap for screening, diagnosis, and treatment of cardiac involvement in SSc.