P406Effect of 6 months bosentan therapy on right ventricular systolic function in patients with Eisenmenger syndrome


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Abstract

Objectives: The aim of the study was to investigate the effect of dual endothelin receptor antagonist bosentan on right ventricular (RV) function in patients with Eisenmenger syndrome (ES).Methods: Twenty four ES patients in WHO functional class III-IV (age 20-59y, mean 33,8) were enrolled into the study. The diagnoses included VSD - 16, CAVSD - 5, PDA-2, ASD II-1. Eight pts with Down syndrome. Six minute walk test (6MWT) and echocardiography were performed at baseline and after six months of bosentan therapy. RV longitudinal systolic function Doppler tissue imaging (DTI), speckle-tracking (STE), tricuspid annular plane systolic excursion (TAPSE)) as well as RV Tei index and RVOT muscle systolic thickening were recorded with respect to EAE guidelines.Results: Mean 6MWT increased from 321.8m to 378.2m; (p<0.05). RV systolic function was not significantly depressed comparing with normal values. RV systolic longitudinal function did not change statistically during 6 month follow-up. Pulsed DTI peak S wave was 10.7 +/-3,0cm/s vs 11.1+/-2.4cm/s. Maximal strain of RV segments (-21.1+/-7,6% vs -20.16+/-6.6% for basal, -19.58+/-6% vs -19.7%+/-5% for mid and -19.53+/-5.8% vs -20.7+/-5.4% for apical segments).Pulsed tissue Doppler Tei index (0.58+/-0.3 vs 0.58+/-0.3).We found significant increase of TAPSE (18+/- 4 vs 20.5+/-4.5; p - 0,00001) and systolic thickening of RVOT muscle (0.62 +/-0.28 vs 0.88 +/-0.33; p = 0.001)Conclusion: Although RV function in ES pts assessed by echocardiography is fairly well preserved, 6 month bosentan therapy resulted in significant increase of TAPSE and thickening of RVOT muscle which is parallel with improved functional capacity assessed by 6MWT.

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