Abstract

Background. Cardiac involvement in systemic sclerosis ( SSc) is controversial but the primary disorder of microvasculature with diffuse arteriolar and capillary lesions could precede any fibrosis, thus causing ischemic dysfunction. Previous studies of coronary flow velocity reserve (CFVR) have demonstrated that CFVR is impaired in patients with SSc. L-Propionylcarnitine( L-Pc) is a metabolic substance that exerts a beneficial effect on both microcirculation and myocite function. We tested the hypothesis that CFVR can be early impaired in patients with SSc without cardiac involvement and whether CFVR could improve after intravenous administration of L-Pc.
Methods. We studied 11 females patients (6 with diffuse form and 5 with localized form of SSc) without clinical evidence of heart disease, mean age 54±11 years and 26 control group patients, matched for age. We evaluated CFVR in the left anterior descending coronary artery (LAD) with contrast Doppler echocardiography during adenosine infusion (140 µg/Kg/min in 5 minutes), before and after 15 minutes of administration of L-Pc ( 300 mg intravenous in 5 minutes bolus).
Results: In patients with SSc CFVR was impaired in comparison to the control group patients (2.56 ± 0.54 vs 3.29±0.52, p=0.0005). Furthermore, in patients with SSc after administration of L-Pc, CFVR increased from 2.56 ± 0.51 to 3.38±0.9, p=0.01. The peak diastolic velocity in the LAD at rest, before and after L-Pc administration were not different (33.8 ±11 vs 28.5±8.6, p=ns).
Conclusion. CFVR is early impaired in patients with SSc without clinical evidence of heart disease. Acute administration of L-Pc exerts a beneficial effect on microvasculature in patients with systemic sclerosis. Therefore, its potential after chronic administration needs to be evaluated.

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