PT - JOURNAL ARTICLE AU - Gérald Simonneau AU - Andrea M. D’Armini AU - Hossein-Ardeschir Ghofrani AU - Friedrich Grimminger AU - Marius M. Hoeper AU - Pavel Jansa AU - Nick H. Kim AU - Chen Wang AU - Martin R. Wilkins AU - Arno Fritsch AU - Neil Davie AU - Pablo Colorado AU - Eckhard Mayer TI - Riociguat for the treatment of chronic thromboembolic pulmonary hypertension: a long-term extension study (CHEST-2) AID - 10.1183/09031936.00087114 DP - 2015 May 01 TA - European Respiratory Journal PG - 1293--1302 VI - 45 IP - 5 4099 - http://erj.ersjournals.com/content/45/5/1293.short 4100 - http://erj.ersjournals.com/content/45/5/1293.full SO - Eur Respir J2015 May 01; 45 AB - Riociguat is a soluble guanylate cyclase stimulator approved for the treatment of inoperable and persistent/recurrent chronic thromboembolic pulmonary hypertension (CTEPH). In the 16-week CHEST-1 study, riociguat showed a favourable benefit–risk profile and improved several clinically relevant end-points in patients with CTEPH. The CHEST-2 open-label extension evaluated the long-term safety and efficacy of riociguat. Eligible patients from CHEST-1 received riociguat individually adjusted up to a maximum dose of 2.5 mg three times daily. The primary objective was the safety and tolerability of riociguat; exploratory efficacy end-points included 6-min walking distance (6MWD) and World Health Organization (WHO) functional class (FC). Overall, 237 patients entered CHEST-2 and 211 (89%) were ongoing at this interim analysis (March 2013). The safety profile of riociguat in CHEST-2 was similar to CHEST-1, with no new safety signals. Improvements in 6MWD and WHO FC observed in CHEST-1 persisted for up to 1 year in CHEST-2. In the observed population at 1 year, mean±sd 6MWD had changed by +51±62 m (n=172) versus CHEST-1 baseline (n=237), and WHO FC had improved/stabilised/worsened in 47/50/3% of patients (n=176) versus CHEST-1 baseline (n=236). Long-term riociguat had a favourable benefit–risk profile and apparently showed sustained benefits in exercise and functional capacity for up to 1 year. Riociguat shows favourable benefit–risk profile in CTEPH patients, with benefits in 6MWD and WHO FC for up to 1 year http://ow.ly/Co04L