Department of Pharmacology and Clinical Neuroscience, Umeå University, Umeå, Sweden.
Actelion a Division of Janssen-Cilag AB, Stockholm, Sweden.
Scand Cardiovasc J. 2021 Feb;55(1):43-49. doi: 10.1080/14017431.2020.1783456. Epub 2020 Jun 26.
To investigate if the pulmonary arterial hypertension (PAH) risk assessment tool presented in the 2015 ESC/ERS guidelines is valid for patients with chronic thromboembolic pulmonary hypertension (CTEPH) when taking pulmonary endarterectomy (PEA) into account. Incident CTEPH patients registered in the Swedish PAH Registry (SPAHR) between 2008 and 2016 were included. Risk stratification performed at baseline and follow-up classified the patients as low-, intermediate-, or high-risk using the proposed ESC/ERS risk algorithm. There were 250 CTEPH patients with median age (interquartile range) 70 (14) years, and 53% were male. Thirty-two percent underwent PEA within 5 (6) months. In a multivariable model adjusting for age, sex, and pharmacological treatment, patients with intermediate-risk or high-risk profiles at baseline displayed an increased mortality risk (Hazard Ratio [95% confidence interval]: 1.64 [0.69-3.90] and 5.39 [2.13-13.59], respectively) compared to those with a low-risk profile, whereas PEA was associated with better survival (0.38 [0.18-0.82]). Similar impact of risk profile and PEA was seen at follow-up. The ESC/ERS risk assessment tool identifies CTEPH patients with reduced survival. Furthermore, PEA improves survival markedly independently of risk group and age. The ESC/ERS risk stratification for PAH predicts survival also in CTEPH patients, even when taking PEA into account.
为了探究 2015 年 ESC/ERS 指南中提出的肺动脉高压（PAH）风险评估工具在考虑肺动脉内膜切除术（PEA）的情况下是否适用于慢性血栓栓塞性肺动脉高压（CTEPH）患者。纳入了 2008 年至 2016 年期间在瑞典 PAH 注册处（SPAHR）登记的新发 CTEPH 患者。在基线和随访时进行的风险分层使用提出的 ESC/ERS 风险算法将患者分为低危、中危或高危。共有 250 例 CTEPH 患者，中位年龄（四分位间距）为 70（14）岁，53%为男性。32%的患者在 5（6）个月内行 PEA。在调整年龄、性别和药物治疗的多变量模型中，基线时具有中危或高危特征的患者与低危特征的患者相比，死亡率风险增加（危险比[95%置信区间]：1.64 [0.69-3.90] 和 5.39 [2.13-13.59]），而 PEA 与更好的生存相关（0.38 [0.18-0.82]）。在随访中也观察到风险状况和 PEA 的类似影响。ESC/ERS 风险评估工具确定了生存风险降低的 CTEPH 患者。此外，PEA 独立于风险组和年龄显著改善了生存。即使考虑到 PEA，ESC/ERS 对 PAH 的风险分层也可预测 CTEPH 患者的生存。
