PT  - JOURNAL ARTICLE
AU  - Eckhard Mayer
AU  - Andrzej Biederman
AU  - Andrea M. D'Armini
AU  - Ekkehard Grünig
AU  - Pavel Jansa
AU  - David P. Jenkins
AU  - Nick H. Kim
AU  - Walter Klepetko
AU  - Jaroslav Lindner
AU  - Michael M. Madani
AU  - Chen Wang
AU  - Neil Davie
AU  - Arno Fritsch
AU  - Hossein-Ardeschir Ghofrani
TI  - Effects of riociguat in patients with persistent/recurrent chronic thromboembolic pulmonary hypertension (CTEPH) following pulmonary endarterectomy (PEA)
DP  - 2014 Sep 01
TA  - European Respiratory Journal
PG  - 3421
VI  - 44
IP  - Suppl 58
4099  - http://erj.ersjournals.com/content/44/Suppl_58/3421.short
4100  - http://erj.ersjournals.com/content/44/Suppl_58/3421.full
SO  - Eur Respir J2014 Sep 01; 44
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.