Mechanics of early biventricular impairment in systemic sclerosis and the effects of peripheral arterial haemodynamics.

OBJECTIVES: Multiple mechanisms commonly lead to severe cardiac involvement in systemic sclerosis (SSc), an autoimmune disease characterised by microvascular lesions, systemic inflammation and fibrosis. Herein, we examined the mechanics of right and left ventricles (RV, LV) at the early stage of impairment and tested the hypothesis that peripheral vasculopathy influences the possible early compromise of LV. METHODS: Ninety-five SSc patients free of any cardiovascular disease or related symptoms (88% women, 53±14 years) and 54 subjects matched for age, gender, arterial hypertension, dyslipidaemia, and diabetes mellitus underwent echocardiography, including multilayer speckle-tracking, and tonometry-based pulse wave analysis of the peripheral arteries; 66 SSc patients were prospectively assessed after 32±7 months. Indices of ventricular and arterial structure and function, as well as LV-arterial coupling, were calculated. RESULTS: At baseline, patients presented RV diastolic/systolic impairment, as well as LV remodelling and diastolic/systolic impairment in terms of reduced deformation parameters versus controls. No association was found between RV and LV strain within individual patients, whereas both RV and LV abnormalities progressed independently during follow-up. Moreover, in the absence of differences in aortic stiffening and LV-arterial coupling between patients and controls, arterial pressure wave reflections assessing small vessel function and/or microcirculation were abnormal in SSc patients and strongly correlated with impaired indices of LV diastolic function and remodelling. CONCLUSIONS: Speckle-tracking echocardiography demonstrates the mechanics of RV early impairment in SSc that develops and progresses independently from the concomitant LV impairment, which, in turn, may be influenced by peripheral microvascular abnormalities in the absence of macrovascular damage.