van Thor M C J, Ten Klooster L, Snijder R J, Post M C, Mager J J
Dept of Cardiology, St. Antonius Hospital, Nieuwegein, the Netherlands.
Dept of Pulmonology, St. Antonius Hospital, Nieuwegein, the Netherlands.
Int J Cardiol Heart Vasc. 2019 Feb 28;22:163-168. doi: 10.1016/j.ijcha.2019.02.004. eCollection 2019 Mar.
To improve clinical outcome, patients with inoperable and residual chronic thromboembolic pulmonary hypertension (CTEPH) can be treated with riociguat. The aim of this study is to explore long-term outcomes and to compare our 'real world' data with previous research.
We included all consecutive patients with technical inoperable and residual CTEPH, in whom riociguat therapy was initiated from January 2014 onwards, with patients followed till January 2019. Survival, clinical worsening (CW), functional class (FC), N-terminal pro brain natriuretic peptide (NT-proBNP) and 6-minute walking distance (6MWD) were described yearly after riociguat initiation.
Thirty-six patients (50% female, mean age 64.9 ± 12.1 years, 54% WHO FC III/IV and 6MWD 337 ± 138 m could be included, with a mean follow-up of 2.3 ± 1.2 years. Survival and CW-free survival three years after initiation of riociguat were 94% and 78%, respectively. The 6MWD per 10 m at baseline was a significant predictor (HR 0.90 [0.83-0.97], = 0.009) for CW. At three years follow-up the WHO FC and 6MWD improved and NT-proBNP decreased compared to baseline.
Our study confirms that riociguat is an effective treatment in patients with technical inoperable and residual CTEPH at long-term follow-up. Although our results are consistent with previous studies, more 'real world' research is necessary to confirm long-term results.
为改善临床结局，无法手术及存在残留病变的慢性血栓栓塞性肺动脉高压（CTEPH）患者可使用利奥西呱进行治疗。本研究旨在探索长期结局，并将我们的“真实世界”数据与既往研究进行比较。
我们纳入了所有自2014年1月起开始接受利奥西呱治疗的连续性技术上无法手术及存在残留病变的CTEPH患者，并对患者随访至2019年1月。在开始使用利奥西呱后，每年描述患者的生存率、临床恶化（CW）、功能分级（FC）、N末端脑钠肽前体（NT-proBNP）和6分钟步行距离（6MWD）。
共纳入36例患者（50%为女性，平均年龄64.9±12.1岁，54%为世界卫生组织功能分级III/IV级，6MWD为337±138米），平均随访2.3±1.2年。开始使用利奥西呱三年后的生存率和无临床恶化生存率分别为94%和78%。基线时每10米的6MWD是CW的显著预测指标（风险比0.90[0.83 - 0.97]，P = 0.009）。与基线相比，三年随访时世界卫生组织功能分级改善，6MWD增加，NT-proBNP降低。
我们的研究证实，在长期随访中，利奥西呱对技术上无法手术及存在残留病变的CTEPH患者是一种有效的治疗方法。尽管我们的结果与既往研究一致，但仍需要更多的“真实世界”研究来证实长期结果。
