Early treatment of borderline pulmonary arterial hypertension associated with systemic sclerosis: a randomized, controlled, double-blind, parallel group, proof-of-concept study

The objective of this single-center and prospective study was to assess the efficacy of ambrisentan monotherapy on hemodynamics and exercise capacity in SSc-patients with BoPAP using right heart catheterization (RHC).38 SSc-patients with BoPAP were randomly assigned to a 10 mg/die ambrisentan monotherapy or placebo treated group for 24 weeks. RHC was performed at baseline and at 24 weeks in all subjects. Further clinical parameters as laboratory measures, WHO-FC, 6MWD, exercise capacity, echocardiographic parameters and quality of life (SF36 questionnaire) were also assessed. During follow-up 4 of the 38 patients developed manifest SSc-APAH. The effect of ambrisentan vs. placebo remains to be analysed once data monitoring has been completed.Initial results suggest a worsening of mean PAP in borderline SSc-APAH patients over the course of 24 weeks. The monotherapeutic effect remains to be analysed within the next weeks.

Source: European Respiratory Journal - November 19, 2018 Category: Respiratory Medicine Authors: Pan, Z., Eichstaedt, C. A., Benjamin, N., Blank, N., Lorenz, H.-M., Fiehn, C., Song, J., Xanthouli, P., Harutyunova, S., Egenlauf, B., Marra, A. M., Fischer, C., Grünig, E. Tags: Pulmonary hypertension Source Type: research