BACKGROUND Approaches to risk assessment in pulmonary arterial hypertension (PAH) include the noninvasive French risk assessment approach (number of low-risk criteria based on the European Society of Cardiology and European Respiratory Society guidelines) and Registry to Evaluate Early and Long-term PAH Disease Management (REVEAL) 2.0 risk calculator. The prognostic and predictive value of these methods for morbidity/mortality was evaluated in the predominantly prevalent population of GRIPHON, the largest randomized controlled trial in PAH. METHODS GRIPHON randomized 1,156 patients with PAH to selexipag or placebo. Post-hoc analyses were performed on the primary composite end-point of morbidity/mortality by the number of low-risk criteria (World Health Organization functional class I-II; 6-minute walk distance >440 m; N-terminal pro-brain natriuretic peptide <300 ng/liter) and REVEAL 2.0 risk category. Hazard ratios and 95% confidence intervals were calculated using Cox proportional hazard models. RESULTS Both the number of low-risk criteria and the REVEAL 2.0 risk category were prognostic for morbidity/mortality at baseline and any time-point during the study. Patients with 3 low-risk criteria at baseline had a 94% reduced risk of morbidity/mortality compared to patients with 0 low-risk criteria and were all categorized as low-risk by REVEAL 2.0. The treatment effect of selexipag on morbidity/mortality was consistent irrespective of the number of low-risk criteria or the REVEAL 2.0 risk category at any time-point during the study. Selexipag-treated patients were more likely to increase their number of low-risk criteria from baseline to week 26 than placebo-treated patients (odds ratio 1.69, p = 0.0002); similar results were observed for REVEAL 2.0 risk score. CONCLUSIONS These results support the association between risk profile and long-term outcome and suggest that selexipag treatment may improve risk profile.

中文翻译：

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肺动脉高压的风险评估：GRIPHON 研究的见解。

背景 肺动脉高压 (PAH) 风险评估方法包括无创法国风险评估方法（基于欧洲心脏病学会和欧洲呼吸学会指南的低风险标准数量）和评估早期和长期 PAH 疾病的登记系统管理（REVEAL）2.0风险计算器。这些方法对发病率/死亡率的预后和预测价值在 GRIPHON（最大的 PAH 随机对照试验）的主要患病人群中进行了评估。方法 GRIPHON 将 1,156 名 PAH 患者随机分配至塞来西帕或安慰剂组。根据低风险标准的数量（世界卫生组织功能等级 I-II；6 分钟步行距离 >440 m；N 端亲脑）对发病率/死亡率的主要复合终点进行事后分析利钠肽 <300 纳克/升）和 REVEAL 2.0 风险类别。使用 Cox 比例风险模型计算风险比和 95% 置信区间。结果 低风险标准的数量和 REVEAL 2.0 风险类别均可预测基线和研究期间任何时间点的发病率/死亡率。与具有 0 个低风险标准的患者相比，基线时具有 3 个低风险标准的患者的发病/死亡风险降低了 94%，并且均被 REVEAL 2.0 归类为低风险。无论研究期间任何时间点的低风险标准或 REVEAL 2.0 风险类别的数量如何，selexipag 对发病率/死亡率的治疗效果都是一致的。与安慰剂治疗的患者相比，Selexipag 治疗的患者更有可能从基线到第 26 周增加低风险标准的数量（比值比 1.69，p = 0.0002）；REVEAL 2.0 风险评分也观察到类似的结果。结论 这些结果支持风险状况与长期结果之间的关联，并表明塞来西帕治疗可以改善风险状况。