Yang Suqiao, Yang Yuanhua, Zhang Yixiao, Kuang Tuguang, Gong Juanni, Li Jifeng, Li Yidan, Wang Jianfeng, Guo Xiaojuan, Miao Ran
Dept of Pulmonary and Critical Care Medicine, Beijing Chao-Yang Hospital, Capital Medical University, Beijing, China.
Beijing Institute of Respiratory Medicine, Beijing, China.
ERJ Open Res. 2021 Sep 6;7(3). doi: 10.1183/23120541.00082-2021. eCollection 2021 Jul.
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.
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).
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 835±348 dyn·s·cm, p<0.001), cardiac index increased (1.7±0.4 L·min·m 2.4±0.5 L·min·m, p<0.001), 6MWD increased by 43.1±59.6 m, and WHO FC improved/stabilised/worsened in 40%/35%/25% of patients 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 0.19 (p=0.026).
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.
已证明，用利奥西呱长期治疗可提高肺动脉高压（PAH）患者以及无法手术或持续性/复发性慢性血栓栓塞性肺动脉高压（CTEPH）患者的运动能力。本研究旨在评估利奥西呱对PAH患者和无法手术的CTEPH患者的长期血流动力学影响。
在这项单中心长期观察性研究中，利奥西呱的给药剂量为每日三次，最高可达2.5mg。主要结局为肺血管阻力（PVR）。次要结局包括平均肺动脉压（PAP）、心脏指数、死亡率、临床恶化事件、6分钟步行距离（6MWD）和世界卫生组织功能分级（WHO FC）。
纳入37例患者（CTEPH患者19例；PAH患者18例）。中位随访期为96个月。所有患者在1/3/5/8年时的生存估计值分别为0.97/0.86/0.72/0.61，CTEPH患者和PAH患者之间无显著差异。在最终数据截止时，PVR降低（1232±462dyn·s·cm至835±348dyn·s·cm，p<0.001），心脏指数升高（1.7±0.4L·min·m至2.4±0.5L·min·m，p<0.001），6MWD增加43.1±59.6m，40%/35%/25%的患者WHO FC较基线改善/稳定/恶化。未显示PAP有改善。与基线时WHO FC I/II级和III/IV级的患者相比，8年无临床恶化生存率估计值分别为0.51和0.19（p=0.026）。
利奥西呱可改善PVR和心脏指数长达8年，但不能改善PAP。WHO FC可能对长期预后有一定的预测价值。
