Third Department of Cardiology, National and Kapodistrian University of Athens, Sotiria Chest Disease Hospital, Athens, Greece.
First Department of Cardiology, AHEPA University Hospital, Aristotle University of Thessaloniki, Thessaloniki, Greece; National Pulmonary Hypertension Service, Royal Brompton Hospital, Guy's & St. Thomas' NHS Foundation Trust, London, UK.
J Heart Lung Transplant. 2024 Sep;43(9):1383-1394. doi: 10.1016/j.healun.2024.04.066. Epub 2024 May 12.
Contemporary patients with pulmonary arterial hypertension (PAH) are older and exhibit cardiovascular or/and lung comorbidities. Such patients have typically been excluded from major PAH drug trials. This systematic review compares baseline characteristics, hemodynamic parameters, and mortality rate between PAH patients with significant number of comorbidities compared to those with fewer or no comorbidities. ΜETHODS: A systematic literature search in PubMed, Web of Science, and Cochrane databases was conducted searching for studies comparing PAH patients with more than 2 cardiovascular comorbidities or/and at least a lung comorbidity against those with fewer comorbidities.
Seven observational studies were included. PAH patients with comorbidities were older, with an almost equal female-to-male ratio, shorter 6-minute walk distance, higher N-terminal pro-brain natriuretic peptide levels, and lower lung diffusion for carbon monoxide. In terms of hemodynamics, they had higher mean right atrial pressure and pulmonary artery wedge pressure, lower mean pulmonary arterial pressure, pulmonary vascular resistance and mixed venous oxygen saturation. Pooled analysis of 6 studies demonstrated a higher mortality risk for PAH patients with comorbidities compared to those without (HR 1.86, 95% CI 1.20 to 2.89, p < 0.001, I²=92%), with the subgroup of PAH patients with lung comorbidities having an even higher mortality risk (test for subgroup differences: p < 0.001). Combination drug therapy for PAH was less frequently used in patients with comorbidities.
Cardiovascular and lung comorbidities impact the clinical characteristics and outcomes of PAH patients, highlighting the need for optimal phenotyping and tailored management for this high-risk population.
当代肺动脉高压（PAH）患者年龄较大，并伴有心血管或/和肺部合并症。这些患者通常被排除在主要的 PAH 药物试验之外。本系统评价比较了具有大量合并症的 PAH 患者与具有较少或无合并症的患者之间的基线特征、血流动力学参数和死亡率。
在 PubMed、Web of Science 和 Cochrane 数据库中进行系统文献检索，以寻找比较具有 2 种以上心血管合并症或/和至少一种肺部合并症的 PAH 患者与具有较少合并症的患者的研究。
纳入了 7 项观察性研究。患有合并症的 PAH 患者年龄较大，男女比例几乎相等，6 分钟步行距离较短，N 端脑利钠肽前体水平较高，一氧化碳肺弥散量较低。在血流动力学方面，他们的平均右心房压和肺动脉楔压较高，平均肺动脉压、肺血管阻力和混合静脉血氧饱和度较低。6 项研究的汇总分析显示，患有合并症的 PAH 患者的死亡率高于无合并症的患者（HR 1.86，95%CI 1.20 至 2.89，p<0.001，I²=92%），患有肺部合并症的 PAH 患者亚组的死亡率甚至更高（检验亚组差异：p<0.001）。合并症患者较少使用联合药物治疗 PAH。
心血管和肺部合并症影响 PAH 患者的临床特征和结局，突出了对这一高风险人群进行最佳表型分析和针对性管理的必要性。
