Schanz Olivia, Criner Gerard J, Rali Parth, Gayen Shameek
Department of Thoracic Medicine and Surgery, Lewis Katz School of Medicine at Temple University Hospital, Philadelphia, PA 19140, USA.
J Cardiovasc Dev Dis. 2024 Mar 8;11(3):89. doi: 10.3390/jcdd11030089.
The mortality benefit of PAH-specific therapy for patients with pulmonary hypertension (PH) associated with lung disease is not clear. Our aim was to determine whether pulmonary arterial hypertension (PAH)-specific therapy is associated with reduced mortality among all patients with PH associated with lung disease and in patients with chronic lung disease and severe PH. This was a retrospective cohort study of patients at our institution with chronic lung disease and PH. Survival analysis was performed by comparing patients who received PAH-specific therapy with patients who did not receive pulmonary vasodilators in the entire cohort and in a subgroup of patients with severe PH defined as PVR > 5 WU. We identified 783 patients with chronic lung disease and PH; 246 patients met the new criteria for severe PH. In the entire cohort, a similar survival probability was seen between the treated and untreated PH groups (logrank = 0.67). In the severe PH subgroup, patients treated with PAH-specific therapy had increased survival probability (logrank = 0.03). PAH-specific therapy was independently and significantly associated with decreased mortality in severe PH (HR 0.31, 95% CI 0.11-0.88, = 0.03). PAH-specific therapy may confer a mortality benefit in patients with chronic lung disease and severe PH, which is now defined as PVR > 5 WU, similarly to those with pulmonary arterial hypertension.
肺动脉高压（PAH）特异性治疗对合并肺部疾病的肺动脉高压（PH）患者的死亡率益处尚不明确。我们的目的是确定PAH特异性治疗是否与所有合并肺部疾病的PH患者以及慢性肺部疾病和重度PH患者的死亡率降低相关。这是一项针对我们机构中患有慢性肺部疾病和PH患者的回顾性队列研究。通过比较在整个队列中接受PAH特异性治疗的患者与未接受肺血管扩张剂治疗的患者，以及在定义为肺血管阻力（PVR）>5伍德单位（WU）的重度PH患者亚组中进行生存分析。我们确定了783例患有慢性肺部疾病和PH的患者；246例患者符合重度PH的新标准。在整个队列中，接受治疗和未接受治疗的PH组之间观察到相似的生存概率（对数秩检验=0.67）。在重度PH亚组中，接受PAH特异性治疗的患者生存概率增加（对数秩检验=0.03）。PAH特异性治疗与重度PH患者死亡率降低独立且显著相关（风险比0.31，95%置信区间0.11-0.88，P=0.03）。PAH特异性治疗可能对患有慢性肺部疾病和重度PH（现定义为PVR>5 WU）的患者有死亡率益处，类似于肺动脉高压患者。
