TY - JOUR T1 - Everolimus improves exercise capacity and pulmonary vascular resistance in patients with advanced pulmonary hypertension – A pilot study JF - European Respiratory Journal JO - Eur Respir J VL - 38 IS - Suppl 55 SP - p2295 AU - Hans-Juergen Seyfarth AU - Stefan Hammerschmidt AU - Michael Halank AU - Petra Neuhaus AU - Hubert Wirtz Y1 - 2011/09/01 UR - http://erj.ersjournals.com/content/38/Suppl_55/p2295.abstract N2 - Background: In recent years, pulmonary arterial hypertension (PAH) has been recognized to be a predominantly proliferative process. The inhibitor of the mammalian target of rapamycin (mTOR) everolimus inhibits cellular protein synthesis and growth in cells of the vascular wall.Methods: Ten patients with PAH (n=8) or chronic thromboembolic pulmonary hypertension (CTEPH) and progressive disease despite therapy with at least two vasodilating drugs were included in a prospective open label pilot study. All patients were treated additionally with everolimus. Safety and tolerability were observed. Pulmonary vascular resistance (PVR) and 6-minute walking distance (6MWD) were considered as primary endpoints.Results: In two patients study medication was stopped prematurely due to an adverse event. The remaining 8 patients exhibited a significant improvement in PVR (1049±438 vs. 689±235 dyn*sec*cm-5; p=0.004) and 6MWD (246±105 vs. 313±127m; p=0.04) after 6 months of therapy with everolimus.Conclusion: Antiproliferative with everolimus therapy was tolerated in ten patients in this pilot study. The observed improvements in PVR and in 6MWD may stimulate further consideration of mTOR inhibition in pulmonary hypertension. ER -