A Patient with Systemic Sclerosis, Dyspnea on Exertion, Atypical Chest Pain, and Arrhythmia

Abstract

Primary myocardial disease is common in patients with systemic sclerosis even in the absence of pulmonary or systemic hypertension and without significant renal or pulmonary disease. When clinically evident, primary scleroderma myocardial disease may be a poor prognostic factor. An increasing body of evidence suggests that myocardial involvement is due, at least in part, to early abnormal vasospasm, with or without associated structural abnormalities, of the small coronary arteries or arterioles.

Myocardial perfusion defects with left and right ventricular dysfunction were reported in systemic sclerosis, using conventional methods. Recent studies using tissue Doppler echocardiography and magnetic resonance imaging have confirmed these results. Vasodilators, such as calcium channel blockers, angiotensin-converting enzyme inhibitors, and an endothelin receptor antagonist, improve both myocardial perfusion and functional abnormalities.