Yamamoto Koji, Takeda Yutaka, Takeda Yasuko, Naniwa Taio, Narita Hitomi, Ohte Nobuyuki
Department of Cardio-Renal Medicine, Nagoya City University Graduate School of Medical Sciences, 1 Kawasumi, Mizuho-cho, Mizuho-ku, Nagoya, Aichi 467-8601, Japan.
BMC Res Notes. 2014 Jun 12;7:359. doi: 10.1186/1756-0500-7-359.
Intravenous epoprostenol is the only drug proved in a randomized study to reduce mortality in patients with idiopathic pulmonary arterial hypertension (PAH). However, administration of this drug has procedural difficulties and a risk of sepsis. Oral drugs provide simple treatment, but their benefit for survival has not been proven. A recovery of patients with PAH to World Health Organization functional class (WHO-FC) I or II may predict favorable survival.
Survival analyses were performed on a historical cohort of 41 patients with PAH. The patients were 43 ± 22 years old, 23 had idiopathic or heritable PAH, and 18 had connective tissue disease-associated PAH. The baseline was defined as the initial visit to a medical facility.
The median duration of follow-up was 1276 days (108 to 5389 days) and 21 patients died during this period. The estimated survival times for patients who received intravenous epoprostenol and did and did not recover to WHO-FC I or II were 4371 ± 577 days and 1172 ± 404 days, respectively. These times for patients who were not treated with intravenous epoprostenol and did and did not recover to WHO-FC I or II were 4717 ± 554 days and 925 ± 230 days, respectively. A Cox proportional hazard analysis gave a hazard ratio for death after recovery to WHO-FC I or II of 0.07 (P < 0.001). In contrast, use of intravenous epoprostenol was not a significant factor affecting survival (P = 0.96).
Patients with PAH who achieve recovery to WHO-FC I or II without use of intravenous epoprostenol have similar survival to those who reach the same WHO-FC with use of intravenous epoprostenol. Benign survival of patients with PAH who have recovered to WHO-FC I or II may extend for several years after onset of the disease.
静脉注射依前列醇是唯一在一项随机研究中被证实可降低特发性肺动脉高压（PAH）患者死亡率的药物。然而，使用这种药物存在操作困难和败血症风险。口服药物提供了简单的治疗方式，但其对生存的益处尚未得到证实。PAH患者恢复到世界卫生组织功能分级（WHO-FC）I级或II级可能预示着良好的生存。
对41例PAH患者的历史队列进行生存分析。患者年龄为43±22岁，23例患有特发性或遗传性PAH，18例患有结缔组织病相关PAH。基线定义为首次就诊于医疗机构。
中位随访时间为1276天（108至5389天），在此期间21例患者死亡。接受静脉注射依前列醇且恢复到WHO-FC I级或II级的患者与未恢复到该级别的患者的估计生存时间分别为4371±577天和1172±404天。未接受静脉注射依前列醇且恢复到WHO-FC I级或II级的患者与未恢复到该级别的患者的生存时间分别为4717±554天和925±230天。Cox比例风险分析得出恢复到WHO-FC I级或II级后死亡的风险比为0.07（P<0.001）。相比之下，使用静脉注射依前列醇不是影响生存的显著因素（P=0.96）。
未使用静脉注射依前列醇而恢复到WHO-FC I级或II级的PAH患者与使用静脉注射依前列醇达到相同WHO-FC级别的患者具有相似的生存率。恢复到WHO-FC I级或II级的PAH患者的良好生存可能在疾病发作后持续数年。
