RT Journal Article SR Electronic T1 Haemodynamic effects of riociguat in CTEPH and PAH: a ten-year observational study JF ERJ Open Research JO erjor FD European Respiratory Society SP 00082-2021 DO 10.1183/23120541.00082-2021 A1 Suqiao Yang A1 Yuanhua Yang A1 Yixiao Zhang A1 Tuguang Kuang A1 Juanni Gong A1 Jifeng Li A1 Yidan Li A1 Jianfeng Wang A1 Xiaojuan Guo A1 Ran Miao YR 2021 UL http://openres.ersjournals.com/content/early/2021/07/08/23120541.00082-2021.abstract AB Background Long-term treatment with riociguat has been shown to enhance exercise capacity in patients of pulmonary arterial hypertension (PAH) and inoperable or persistent/recurrent chronic thromboembolic pulmonary hypertension (CTEPH). This study sought to evaluate the long-term haemodynamic effects of riociguat in patients of PAH and inoperable CTEPH.Methods During this single-center long-term observational study, riociguat was administered at a three-times-daily dose of up to 2.5 mg. The primary outcome was pulmonary vascular resistance (PVR). The secondary outcomes included mean pulmonary arterial pressure (PAP), cardiac index (CI), mortality, clinical worsening events, 6-min walking distance (6 MWD), and World Health Organization functional class (WHO FC).Results 37 patients (CTEPH, n=19; PAH, n=18) were included. The median follow-up period was 96 months. The survival estimates for all the patients at 1/3/5/8 year were 0.97/0.86/0.72/0.61, without significant difference between patients with CTEPH and PAH. At the final data cut-off, PVR decreased (1232±462 dyn·s·cm–5 versus 835±348 dyn·s·cm–5, p<0.001), CI increased (1.7±0.4 L·min−1·m−2 versus 2.4±0.5 L·min−1·m−2, p<0.001), 6 MWD increased by 43.1±59.6 m, and WHO FC improved/stabilised/worsened in 40/35/25% of patients versus baseline. Improvement in PAP was not shown. Compared with patients in WHO FC I/II and III/IV at baseline, the 8-year clinical worsening-free survival estimates were 0.51 versus 0.19 (p=0.026).Conclusions Riociguat improved PVR and CI for up to 8 years, but not PAP. WHO FC may have certain predictive value for the long-term prognosis.FootnotesThis manuscript has recently been accepted for publication in the ERJ Open Research. It is published here in its accepted form prior to copyediting and typesetting by our production team. After these production processes are complete and the authors have approved the resulting proofs, the article will move to the latest issue of the ERJOR online. Please open or download the PDF to view this article.Conflict of interest: Dr. Yang has nothing to disclose.Conflict of interest: Dr. Yang has nothing to disclose.Conflict of interest: Miss. Zhang has nothing to disclose.Conflict of interest: Dr. Kuang has nothing to disclose.Conflict of interest: Dr. Gong has nothing to disclose.Conflict of interest: Dr. Li has nothing to disclose.Conflict of interest: Dr. Li has nothing to disclose.Conflict of interest: Dr. Wang has nothing to disclose.Conflict of interest: Dr. Guo has nothing to disclose.Conflict of interest: Dr. Miao has nothing to disclose.