Primary Heart Involvement in Systemic Sclerosis

Systemic sclerosis (SSc) is a connective tissue disease characterised by widespread vascular lesions and fibrosis of the skin and internal organs. Cardiac involvement is recognised as a poor prognostic factor when clinically evident. Primary myocardial involvement is common in SSc. Increasing evidence strongly suggests that myocardial involvement is related to repeated focal ischaemia leading to myocardial fibrosis with irreversible lesions. This results from microcirculation impairment with abnormal vasoreactivity, with or without associated structural vascular abnormalities. Myocardial perfusion impairment, abnormal systolic and diastolic left ventricular dysfunction and right ventricular dysfunction have been reported in SSc, using conventional methods. Recent methods such as tissue Doppler echocardiography and magnetic resonance imaging have underlined these results. These sensitive and quantitative methods have demonstrated the ability of vasodilators, including calcium channel blockers and angiotensin converting enzyme inhibitors, to improve both perfusion and function abnormalities.