Survival in primary pulmonary hypertension - The impact of epoprostenol therapy
Survival in primary pulmonary hypertension - The impact of epoprostenol therapy
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DOI：
10.1161/01.cir.0000029100.82385.58
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发表时间：
2002-09-17
期刊：
影响因子：
37.8
通讯作者：
Rich, S
中科院分区：
文献类型：
--
作者：
McLaughlin, VV;Shillington, A;Rich, S
背景-原发性肺动脉高压（PPH）是一种严重的进行性疾病。如果不治疗，中位生存期为 2.8 年，1 年、3 年和 5 年生存率分别为 68%、48% 和 34%。静脉注射依前列醇是美国食品和药物管理局批准的第一个 PPH 治疗方法。依前列醇对 PPH 的长期影响仍有待确定。 方法和结果 - 对 162 名连续诊断为 PPH 并接受依前列醇治疗的患者进行平均 36.3 个月（中位 31 个月）的随访。包括功能等级、运动耐量和血流动力学在内的数据记录在定制数据库中。验证了每位患者的生命状态。依前列醇治疗 1 年、2 年和 3 年观察到的生存率分别为 87.8%、76.3% 和 62.8%，显着高于基于历史数据的预期生存率 58.9%、46.3% 和 35.4%。生存的基线预测因素包括运动耐量、功能等级、右心房压力和对腺苷的血管扩张反应。第一年治疗后生存的预测因素包括功能等级以及运动耐量、心脏指数和平均肺动脉压的改善。结论-静脉注射依前列醇可改善 PPH 的长期生存。
Background-Primary pulmonary hypertension (PPH) is a severe and progressive disease. Without treatment, the median survival is 2.8 years, with survival rates of 68%, 48%, and 34% at 1, 3, and 5 years, respectively. Intravenous epoprostenol was the first Food and Drug Administration-approved therapy for PPH. The long-term impact that epoprostenol has made on PPH remains to be defined.Methods and Results-One hundred sixty-two consecutive patients diagnosed with PPH and treated with epoprostenol were followed for a mean of 36.3 months (median, 31 months). Data including functional class, exercise tolerance, and hemodynamics were recorded in a customized database. Vital status was verified in each patient. Observed survival with epoprostenol therapy at 1, 2, and 3 years was 87.8%, 76.3%, and 62.8% and was significantly greater than the expected survival of 58.9%, 46.3%, and 35.4% based on historical data. Baseline predictors of survival included exercise tolerance, functional class, right atrial pressure, and vasodilator response to adenosine. Predictors of survival after the first year of therapy included functional class and improvement in exercise tolerance, cardiac index, and mean pulmonary artery pressure.Conclusions-Intravenous epoprostenol improves long-term survival in PPH.