Zhang Meng, Guo Xueran, Guo Wei, Wang Yan, Xiao Yao, Han Jiancheng, Li Ye, Man Tingting, Li Jie, Chen Yong, Duan Shengchen, Zhang Wenmei, Li Hui, Yin Ao, Peng Jiafei, An Chunrong, Xie Wanmu, Gao Qian, Zhang Shuai, Zhang Yunxia, Zhai Zhenguo, Wan Jun
Department of Pulmonary and Critical Care Medicine, Beijing Anzhen Hospital, Capital Medical University, Beijing Institute of Heart, Lung and Blood Vessel Diseases, Beijing, 100029, China.
Beijing Key Laboratory of Ophthalmology & Visual Sciences, Beijing Tongren Eye Center, Beijing Tongren Hospital, Capital Medical University, Beijing, 100730, China.
BMC Pulm Med. 2025 Jul 22;25(1):348. doi: 10.1186/s12890-025-03833-4.
Pulmonary arterial hypertension (PAH) patients with cardiopulmonary comorbidities have been prevalent nowadays. However, there was limited data on clinical characteristics and therapeutic responses in these populations.
Patients diagnosed with right heart catheterization (RHC)-confirmed PAH between October 2021 to March 2023 were included in our study. According to whether they had cardiopulmonary diseases or not, they were classified into two groups: comorbidities group and non-comorbidities group. We aimed to compare the clinical data, PAH-targeted strategies, and therapeutic responses between these two PAH groups. We further analyzed the impact of the numbers and categories of comorbidities on therapeutic responses.
Almost half of the patients co-existed with cardiopulmonary diseases. Compared with non-comorbidities group (n = 40), comorbidities group (n = 36) were senior (p = 0.000) and male predominantly (p = 0.005). Comorbidities group also associated inconsistencies between hemodynamics and 6-min walking distance (6MWD), with a shorter 6MWD (p = 0.000), but a lower mean pulmonary artery pressure (mPAP) (p = 0.008). Non-comorbidities group showed an upturn in the WHO-FC (p = 0.010) and risk assessment (p = 0.033), while the improvement of hemodynamics [decreased mPAP (p = 0.009) and pulmonary vascular resistance (PVR) (p = 0.001), increased cardiac index (p = 0.001)] in comorbidities group did not match the change in clinical severity (no significant improvements in WHO-FC, risk stratification and 6MWD). When categorized by the comorbidities counts, it demonstrated that the more comorbidities, the more severe the clinical conditions, and the worse the therapeutic responses.
PAH patients with cardiopulmonary comorbidities represent a unique phenotype, with different clinical manifestation and treatment responses from typical PAH and inconsistencies between hemodynamics and functional status from baseline to follow-up.
如今，合并心肺疾病的肺动脉高压（PAH）患者颇为常见。然而，关于这些人群的临床特征和治疗反应的数据有限。
纳入2021年10月至2023年3月期间经右心导管检查（RHC）确诊为PAH的患者。根据是否患有心肺疾病，将他们分为两组：合并症组和无合并症组。我们旨在比较这两组PAH患者的临床数据、PAH靶向治疗策略及治疗反应。我们进一步分析了合并症的数量和类别对治疗反应的影响。
几乎一半的患者合并心肺疾病。与无合并症组（n = 40）相比，合并症组（n = 36）患者年龄更大（p = 0.000），且以男性为主（p = 0.005）。合并症组还存在血流动力学与6分钟步行距离（6MWD）不一致的情况，6MWD更短（p = 0.000），但平均肺动脉压（mPAP）更低（p = 0.008）。无合并症组患者的世界卫生组织功能分级（WHO-FC）（p = 0.010）和风险评估（p = 0.033）有所改善，而合并症组血流动力学的改善[降低的mPAP（p = 0.009）和肺血管阻力（PVR）（p = 0.001），增加的心指数（p = 0.）]与临床严重程度的变化不匹配（WHO-FC、风险分层和6MWD无显著改善）。按合并症数量分类时，结果显示合并症越多，临床病情越严重，治疗反应越差。
合并心肺疾病的PAH患者代表一种独特的表型，其临床表现和治疗反应与典型PAH不同，且从基线到随访期间血流动力学与功能状态存在不一致。
