Abstract：
Upfront combination therapy including intravenous prostaglandin I2 (PGI2-IV) is recognized as the most appropriate treatment for patients with severe pulmonary arterial hypertension (PAH). This retrospective study aimed to determine reasons why this therapy is not used for some patients with severe PAH and describe the hemodynamic and clinical prognoses of patients receiving initial combination treatment with (PGI2-IV+) or without (PGI2-IV-) PGI2-IV.Data for patients with severe PAH (World Health Organization Functional Class III/IV and mean pulmonary arterial pressure [mPAP] ≥ 40 mmHg) were extracted from the Japan Pulmonary Hypertension Registry. Overall, 73 patients were included (PGI2-IV + n = 17; PGI2-IV- n = 56). The PGI2-IV+ cohort was younger than the PGI2-IV- cohort (33.8 ± 10.6 versus 52.6 ± 18.2 years) and had higher mPAP (58.1 ± 12.9 versus 51.8 ± 9.0 mmHg), greater prevalence of idiopathic PAH (88% versus 32%), and less prevalence of connective tissue disease-associated PAH (0% versus 29%). Hemodynamic measures, including mPAP, showed improvement in both cohorts (post-treatment median [interquartile range] 38.5 [17.0-40.0] for the PGI2-IV + cohort and 33.0 [25.0-43.0] mmHg for the PGI2-IV - cohort). Deaths (8/56) and lung transplantation (1/56) occurred only in the PGI2-IV - cohort.These Japanese registry data indicate that older age, lower mPAP, and non-idiopathic PAH may influence clinicians against using upfront combination therapy including PGI2-IV for patients with severe PAH. Early combination therapy including PGI2-IV was associated with improved hemodynamics from baseline, but interpretation is limited by the small sample size.
摘要：
包括静脉内前列腺素I2（PGI2-IV）在内的前期联合治疗被认为是重度肺动脉高压（PAH）患者的最合适的治疗方法。这项回顾性研究旨在确定某些严重PAH患者不使用该疗法的原因，并描述接受（PGI2-IV）或不接受（PGI2-IV-）PGI2-IV初始联合治疗的患者的血液动力学和临床预后。严重PAH（世界卫生组织功能III/IV级和平均肺动脉压[mPAP]≥40mmHg）患者的数据来自日本肺动脉高压注册中心。总的来说,包括73例患者(PGI2-IV+n=17;PGI2-IV-n=56)。PGI2-IV队列比PGI2-IV队列年轻（33.8±10.6比52.6±18.2岁），并且mPAP更高（58.1±12.9比51.8±9.0mmHg），特发性PAH的患病率更高（88%对32%），结缔组织疾病相关PAH的患病率较低(0%对29%)。血流动力学测量,包括mPAP,在两个队列中均显示改善(PGI2-IV+队列的治疗后中位数[四分位数间距]38.5[17.0-40.0]和PGI2-IV-队列的33.0[25.0-43.0]mmHg)。死亡（8/56）和肺移植（1/56）仅发生在PGI2-IV队列中。这些日本登记数据表明年龄较大，降低mPAP,非特发性PAH可能会影响临床医生对重度PAH患者使用包括PGI2-IV在内的前期联合治疗。包括PGI2-IV在内的早期联合治疗与基线血流动力学改善相关。但解释受到样本量小的限制。
