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OBJECTIVE: To evaluate the short-term effects of bosentan on myocardial perfusion and function assessed by cardiac magnetic resonance imaging (MRI) and Tissue-Doppler echography (TDE) respectively in patients with systemic sclerosis (SSc). METHODS: We prospectively evaluated 18 SSc patients without clinical heart failure and with normal pulmonary arterial pressure. MRI perfusion index and systolic and diastolic strain rates (SR) determined by TDE were assessed at baseline for comparison with healthy controls (after a 72-hour vasodilator washout period), and repeated after 4 weeks of bosentan treatment (62.5 mg bid for 2 weeks titrated to 125 mg bid for 2 weeks). RESULTS: Patients with SSc had decreased MRI perfusion index and TDE SR in comparison with healthy controls. Bosentan treatment led to a significant increase in median (range) global MRI perfusion index [from 0.17 (0.09-0.23) at Day 0 to 0.22 (0.13-0.30) after bosentan treatment; p = 0.0004], systolic SR [from 2.1 (1.3-3.1) s-1 to 2.8 (2.1-4.8) s-1; p = 0.0002), and diastolic SR [from 2.6 (1.4-6.7) to 3.6 (2.0-7.6) s-1; p = 0.0003]. CONCLUSION: Short-term treatment with bosentan simultaneously improves myocardial perfusion and function, as evaluated by highly sensitive and quantitative methods, in patients with SSc. Whether additional remodeling effect may be observed after longterm treatment with bosentan remains to be determined.