SURVIVAL IN PRIMARY PULMONARY-HYPERTENSION WITH LONG-TERM CONTINUOUS INTRAVENOUS PROSTACYCLIN
SURVIVAL IN PRIMARY PULMONARY-HYPERTENSION WITH LONG-TERM CONTINUOUS INTRAVENOUS PROSTACYCLIN
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DOI：
10.7326/0003-4819-121-6-199409150-00003
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发表时间：
1994-09-15
影响因子：
39.2
通讯作者：
LEVY, PS
中科院分区：
文献类型：
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作者：
BARST, RJ;RUBIN, LJ;LEVY, PS
目的：评价长期静脉滴注前列环素对原发性肺动脉高压患者运动能力、血流动力学和生存率的影响。设计：开放、多中心、非对照试验。设置：4个转诊中心。患者：18例原发性肺动脉高压患者：1例纽约心脏协会(NYHA)II级患者，13例NYHA III级患者，4例NYHA IV级患者。干预：使用便携式输液泵持续静脉注射前列环素。测量与主要结果：使用6min步行评价运动能力，患者在接受前列环素治疗后平均可多走100m(6个月时距离为370+/-119m，基线时为264+/-160m；P<；0.001；18个月时距离为408+/-138m；与基线相比P=0.02)。6个月后血流动力学改善：心脏指数较治疗前增加18%(95%CI，0.1%~36.7%；P=0.02)，平均肺动脉压和总肺阻力分别降低9%(CI，1.4%~15.7%；P=0.03)和26%(CI，6.1%~46.3%；P=0.02)。心指数和总肺阻力持续改善12个月(与治疗前相比分别增加27%[CI，1.3%~51.9%；P=0.05]和32%[CI，9.7%~53.6%；P=0.02])。与接受标准治疗的历史对照组(美国国立卫生研究院初级肺动脉高压登记处，n=31，P=0.045)相比，接受持续前列环素治疗的NYHA III级和IV级患者(n=17；随访期37至69个月)的存活率有所提高。Kaplan-Meier估计接受前列环素治疗的患者的1、2和3年生存率分别为86.9%、72.4%和63.3%，而历史对照组分别为77.4%、51.6%和40.6%(危险比2.9CI1.0至8.0P=0.045)。由药物和给药系统引起的严重并发症包括3例患者中2例死亡和7例非致死性脓毒症。结论：持续静脉注射前列环素可持续改善临床和血流动力学，并可能改善重度原发性肺动脉高压患者的生存。尽管有潜在的严重并发症，但长期服用前列环素可能对等待移植的重病患者特别有帮助。
Objective: To evaluate,the effects of long-term intravenous infusion of prostacyclin on exercise capacity, hemodynamics, and survival in patients with primary pulmonary hypertension.Design: Open, multicenter, uncontrolled trial.Setting: Four referral centers.Patients: 18 patients with primary pulmonary hypertension: 1 New York Heart Association (NYHA) class II patient, 13 NYHA class III patients, and 4 NYHA class IV patients.Interventions: Continuous intravenous prostacyclin administered by portable infusion pumps. All patients were treated with anticoagulant agents.Measurements and Main Results: With the 6-minute walk used to evaluate exercise capacity, patients could walk on average more than 100 meters farther after prostacyclin therapy was initiated (distance at 6 months, 370 +/- 119 meters compared with 264 +/- 160 meters at baseline; P < 0.001; distance at 18 months, 408 +/- 138 meters; P = 0.02 compared with baseline). Hemodynamics were improved at 6 months: The cardiac index increased 18% (95% CI, 0.1% to 36.7%; P = 0.02), and mean pulmonary artery pressure and total pulmonary resistance decreased 9% (CI, 1.4% to 15.7%; P = 0.03) and 26% (CI, 6.1% to 46.3%; P = 0.02), respectively, compared with baseline. The improvements in cardiac index and total pulmonary resistance were maintained at 12 months (27% increase [CI, 1.3% to 51.9%; P = 0.05] and 32% decrease [CI, 9.7% to 53.6%; P = 0.02] compared with baseline, respectively). Survival was improved in NYHA class III and IV patients who received continuous prostacyclin (n = 17; follow-up, 37 to 69 months) when compared with historical controls who received standard therapy (National Institutes of Health Primary Pulmonary Hypertension Registry, n = 31, P = 0.045). Kaplan-Meier estimates of 1-, 2-, and 3-year survival rates for the patients treated with prostacyclin were 86.9%, 72.4%, and 63.3%, respectively, compared with 77.4%, 51.6%, and 40.6% for the historical control group (hazard ratio, 2.9 [CI, 1.0 to 8.0; P = 0.045]). Serious complications attributable to the drug and delivery system included two deaths and seven episodes of nonfatal sepsis in three patients.Conclusions: Continuous intravenous prostacyclin resulted in sustained clinical and hemodynamic improvement and probably in improved survival in patients with severe primary pulmonary hypertension. Despite potentially serious complications, long-term prostacyclin may be especially helpful in seriously ill patients awaiting transplantation.