RT Journal Article SR Electronic T1 Effects of riociguat in patients with persistent/recurrent chronic thromboembolic pulmonary hypertension (CTEPH) following pulmonary endarterectomy (PEA) JF European Respiratory Journal JO Eur Respir J FD European Respiratory Society SP 3421 VO 44 IS Suppl 58 A1 Eckhard Mayer A1 Andrzej Biederman A1 Andrea M. D'Armini A1 Ekkehard Grünig A1 Pavel Jansa A1 David P. Jenkins A1 Nick H. Kim A1 Walter Klepetko A1 Jaroslav Lindner A1 Michael M. Madani A1 Chen Wang A1 Neil Davie A1 Arno Fritsch A1 Hossein-Ardeschir Ghofrani YR 2014 UL http://erj.ersjournals.com/content/44/Suppl_58/3421.abstract AB BackgroundPEA is the recommended treatment for CTEPH as it is potentially curative in the majority of patients (pts). However, some pts have persistent/recurrent pulmonary hypertension (PH) following PEA.AimsThis subgroup analysis of the CHEST study assessed the long-term effects of riociguat in pts with persistent/recurrent PH following PEA.MethodsIn CHEST-1, 72 pts with persistent/recurrent PH following PEA were randomized to riociguat (up to 2.5 mg tid) or placebo (pbo) for 16 wks. After CHEST-1 completion, eligible pts could enter the long-term extension study CHEST-2, where all pts received riociguat. The results of an interim analysis (censored Mar 2013) are reported.ResultsAt the end of CHEST-1, 6MWD and several secondary endpoints had improved in the riociguat group vs pbo. Of the 72 pts with persistent/recurrent PH following PEA in CHEST-1, 65 entered CHEST-2 (mean age 58±15 yrs, mean time since PEA 3.3 yrs). At censor date, 86% (n=56) of pts were ongoing, 69% (n=45) attended the 1-yr visit, and 14% (n=9) had discontinued, of whom 8% (n=5) had died. Riociguat was well tolerated; 2% (n=1) of pts withdrew due to adverse events. Improvements in 6MWD at Wk 12 of CHEST-2 vs CHEST-1 baseline (ex-riociguat 40±75 m; ex-pbo 44±43 m) were sustained for 1 yr in the overall cohort (44±64 m; n=42). WHO functional class improved/stabilized/worsened in 42/53/4% and 42/58/0% of the ex-riociguat and ex-pbo pts, respectively, at Wk 12 of CHEST-2, and 49/51/0% of pts at 1 yr (n=43).ConclusionsLong-term riociguat was well tolerated in pts with persistent/recurrent PH following PEA and improved 6MWD and secondary endpoints for up to 1 yr.