TY - JOUR T1 - Haemodynamic effects of riociguat in CTEPH and PAH: a 10-year observational study JF - ERJ Open Research JO - erjor DO - 10.1183/23120541.00082-2021 VL - 7 IS - 3 SP - 00082-2021 AU - Suqiao Yang AU - Yuanhua Yang AU - Yixiao Zhang AU - Tuguang Kuang AU - Juanni Gong AU - Jifeng Li AU - Yidan Li AU - Jianfeng Wang AU - Xiaojuan Guo AU - Ran Miao Y1 - 2021/07/01 UR - http://openres.ersjournals.com/content/7/3/00082-2021.abstract N2 - Background Long-term treatment with riociguat has been shown to enhance exercise capacity in patients with 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 with PAH and inoperable CTEPH.Methods During this single-centre 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, mortality, clinical worsening events, 6-min walk distance (6MWD) 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 years were 0.97/0.86/0.72/0.61, without significant differences 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), cardiac index increased (1.7±0.4 L·min−1·m−2 versus 2.4±0.5 L·min−1·m−2, p<0.001), 6MWD 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 cardiac index for up to 8 years, but not PAP. WHO FC may have certain predictive value for the long-term prognosis.In patients with PAH and inoperable CTEPH, riociguat improved pulmonary vascular resistance and cardiac index for 8 years, but not pulmonary arterial pressure. World Health Organization functional class may have predictive value for long-term prognosis. https://bit.ly/3dTf4ft ER -