RT Journal Article
SR Electronic
T1 Haemodynamic effects of riociguat in CTEPH and PAH: a 10-year observational study
JF ERJ Open Research
JO erjor
FD European Respiratory Society
SP 00082-2021
DO 10.1183/23120541.00082-2021
VO 7
IS 3
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/7/3/00082-2021.abstract
AB 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