Abstract

Objectives

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. Design. 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. Results. 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. Conclusion. The ESC/ERS risk assessment tool identifies CTEPH patients with reduced survival. Furthermore, PEA improves survival markedly independently of risk group and age.

Take home message: The ESC/ERS risk stratification for PAH predicts survival also in CTEPH patients, even when taking PEA into account.

中文翻译：

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慢性血栓栓塞性肺动脉高压的风险分层预测生存

摘要

目标

研究 2015 ESC/ERS 指南中提出的肺动脉高压 (PAH) 风险评估工具是否适用于考虑肺动脉内膜切除术 (PEA) 的慢性血栓栓塞性肺动脉高压 (CTEPH) 患者。设计。包括 2008 年至 2016 年在瑞典 PAH 登记处 (SPAHR) 注册的 CTEPH 患者。在基线和随访时进行的风险分层使用建议的 ESC/ERS 风险算法将患者分类为低、中或高风险。结果。有 250 名 CTEPH 患者的中位年龄（四分位距）为 70 (14) 岁，其中 53% 为男性。32% 的患者在 5 (6) 个月内接受了 PEA。在对年龄、性别和药物治疗进行调整的多变量模型中，基线时具有中等风险或高风险特征的患者显示出更高的死亡风险（危险比 [95% 置信区间]：1.64 [0.69–3.90] 和 5.39 [ 2.13-13.59]）与低风险患者相比，而 PEA 与更好的生存相关（0.38 [0.18-0.82]）。在随访中观察到风险状况和 PEA 的类似影响。结论。ESC/ERS 风险评估工具可识别存活率降低的 CTEPH 患者。此外，PEA 显着提高了生存率，而不受风险组和年龄的影响。

重要信息： PAH 的 ESC/ERS 风险分层也可以预测 CTEPH 患者的生存率，即使考虑到 PEA。