Cardiac involvement

Cardiac involvement in systemic sclerosis (SSc) is common, can present with protean manifestations, and, when symptomatic, portends a poor prognosis [1-4]. SSc 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 [1-4]. Furthermore, cardiac manifestations of SSc can be primary (i.e., direct cardiac involvement due to the SSc disease process) or secondary to pulmonary arterial hypertension, interstitial lung disease, or significant kidney disease, all of which are common in patients with SSc [1]. The purpose of the present chapter is to focus on primary, direct cardiac involvement in SSc, with discussion of: (1) epidemiology of cardiac involvement in SSc; (2) screening for cardiac involvement in SSc; (3) characterization of the pathophysiology, diagnosis, and management of specific cardiac lesions in SSc; (4) noninvasive and invasive tools for the detection and monitoring of cardiac involvement; and (5) areas of uncertainty in the study of cardiac involvement in SSc. The successful evaluation and management of cardiac involvement in patients with SSc requires careful screening, a high index of suspicion given the broad and nonspecific symptoms associated with cardiac manifestations, and a multidisciplinary approach involving rheumatologists, cardiologists, and imaging specialists. With the advent of novel noninvasive tests such as new biomarkers, tissue Doppler imaging, speckle-tracking echocardiography, and diffuse fibrosis imaging using cardiac magnetic resonance imaging (MRI), screening, diagnosis, and research of cardiac manifestations of SSc should expand rapidly in the near future.