Chapter 13 - Systemic Sclerosis

Abstract

Cardiac involvement in systemic sclerosis (SSc) may be either primary or secondary to the impairment of other organs such as the lungs and kidneys. Primary cardiac involvement, which develops as a direct consequence of the disease, may affect all cardiac tunics, endocardium, myocardium, and pericardium. This may conduct to pericardial effusion, arrhythmias, conduction system defects, myocardial ischemia, myocardial hypertrophy, and heart failure. The prevalence of primary cardiac involvement in SSc varies depending on the sensitivity of the diagnostic tools used, but it is ascertained that overt cardiac manifestations are associated with poor prognosis. Microvascular impairment is a pathognomonic feature of SSc, and together with focal fibrosis may involve all cardiac structures. In contrast, the frequency of atherosclerotic coronary artery disease in SSc seems to be comparable to that of the general population. Because cardiac involvement in SSc can be often clinically obscure, a complete routine evaluation should be adopted in every patient, including standard 12-lead electrocardiogram (ECG) and Doppler echocardiography (especially tissue Doppler imaging), while magnetic resonance imaging and cardiac computed tomography are sensitive techniques for earlier detection of both structural and functional scleroderma-related cardiac pathologies, although they still represent a second level examination, not available everywhere. Additional investigations can be required in suspicion of other manifestations such as conduction and rhythm disorders (24-h Holter monitoring, invasive electrophysiological studies), or myocarditis (cardiac magnetic resonance). Routine screening for subclinical cardiac involvement with more sensitive tools leads to early diagnosis, in order to treat patients in a “window of opportunity” with drugs that may improve myocardial perfusion and function, and possibly limit the progression of the most life-threatening complications.