The aim of the study was to describe and investigate the effect of pulmonary arterial hypertension (PAH) therapies in a cohort of patients with severe precapillary pulmonary hypertension (PH) associated with chronic obstructive pulmonary disease (COPD; PH-COPD), and to assess factors predictive of treatment response and mortality. We retrospectively included patients with severe incident PH-COPD who received PAH therapy and underwent RHC at diagnosis and on treatment. From 2015 to 2022, 35 severe PH-COPD patients, with clinical features of pulmonary vascular phenotype, were included. Seventeen (48.5%) patients were treated with combined PAH therapy. PAH therapy led to a significant improvement in hemodynamics (PVR -3.5 Wood Units (−39.3%); p < 0.0001), and in the simplified four-strata risk-assessment score, which improved by at least one category in 21 (60%) patients. This effect was more pronounced in patients on dual therapy. Kaplan-Meier estimated survival rates at 1, 3 and 5 years were 94%, 65% and 42% respectively. Univariate analysis showed a significant reduction in survival in patients with a higher simplified risk score at follow-up (Hazard ratio (HR) 2.88 [1.16–7.15]; p = 0.02). Hypoxemia <50 mmHg was correlated to mortality in multivariate analysis (HR 4.33 [1.08–17.42]; p = 0.04). Our study confirms the poor prognosis of patients with COPD and a pulmonary vascular phenotype and the potential interest of combined PAH therapy in this population, with good tolerability and greater clinical and hemodynamic improvement than monotherapy. Using the simplified risk score during follow-up could be of interest in this population.

中文翻译：

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慢性阻塞性肺疾病和肺血管表型患者的生存率和对肺血管扩张剂治疗的反应

该研究的目的是描述和研究肺动脉高压（PAH）治疗对一组患有慢性阻塞性肺疾病（COPD；PH-COPD）的严重毛细血管前肺动脉高压（PH）患者的效果，并评估预测治疗反应和死亡率的因素。我们回顾性纳入了接受 PAH 治疗并在诊断和治疗时接受 RHC 的严重 PH-COPD 患者。 2015年至2022年，纳入35例具有肺血管表型临床特征的重症PH-COPD患者。 17 名 (48.5%) 患者接受了 PAH 联合治疗。 PAH 治疗导致血流动力学显着改善（PVR -3.5 伍德单位（−39.3%）；p < 0.0001），并且简化的四层风险评估评分显着改善，21 例中至少改善了一个类别（60% ） 患者。这种效果在接受双重治疗的患者中更为明显。 Kaplan-Meier 估计 1 年、3 年和 5 年生存率分别为 94%、65% 和 42%。单变量分析显示，随访时简化风险评分较高的患者生存率显着降低（风险比 (HR) 2.88 [1.16–7.15]；p = 0.02）。多变量分析表明，低氧血症<50 mmHg 与死亡率相关（HR 4.33 [1.08–17.42]；p = 0.04）。我们的研究证实了患有慢性阻塞性肺病（COPD）和肺血管表型的患者的预后不良，以及多环芳烃联合治疗对该人群的潜在兴趣，与单一疗法相比，其具有良好的耐受性和更大的临床和血流动力学改善。在随访期间使用简化的风险评分可能会引起该人群的兴趣。