Vasodilator therapy for primary pulmonary hypertension in children
Vasodilator therapy for primary pulmonary hypertension in children
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DOI：
10.1161/01.cir.99.9.1197
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发表时间：
1999-03-09
期刊：
影响因子：
37.8
通讯作者：
Fishman, AP
中科院分区：
文献类型：
--
作者：
Barst, RJ;Maislin, G;Fishman, AP
背景-本研究介绍了13年来血管扩张剂治疗儿童原发性肺动脉高压（PPH）的经验。涉及两个时代：在1982年和1987年之间，口服钙通道阻滞剂是唯一可用于长期治疗的药物;在1987年之后，前列环素（PGI（2））可用于长期静脉内使用。方法和结果-74名儿童接受了静脉内PGI（2）的短期血管扩张剂试验。那些表现出肺血管舒张的患者（“急性反应者”）接受口服钙通道阻滞剂治疗。直到1987年，“急性无反应者”只要没有严重的副作用，就以同样的方式治疗。当PGI（2）可用于长期给药时，所有无应答者以及钙通道阻滞剂未能改善临床和血流动力学的患者均接受长期PGI（2）治疗。在31名应答者中，钙通道阻滞剂与43名无应答者相比改善了生存率（P=0.0002）。与22例PGI（2）不可用的无应答者（P=0.0005）以及所有常规治疗失败的儿童（n=31; P=0.002）相比，24例PGI（2）治疗无应答者的生存率也更好。结论-长期血管扩张剂治疗可提高PPH儿童的生存率。在急性反应者中，口服钙通道阻滞剂通常足够。在短期试验无应答者和钙通道阻滞剂治疗无效的应答者中，持续静脉输注PGI（2）可提高生存率。
Background-This repels presents 13 years of experience with vasodilator therapy for primary pulmonary hypertension (PPH) in children. Two eras were involved: between 1982 and 1987, oral calcium channel blockers were the only agents available for long-term therapy; after 1987, prostacyclin (PGI(2)) has been available for long-term intravenous use.Methods and Results-Seventy-four children underwent short-term vasodilator testing with intravenous PGI(2). Those who manifested pulmonary vasodilation ("acute responders") were treated with oral calcium channel blockers. Until 1987, "acute nonresponders" were treated in the same way as long as they had no serious side effects. When PGI(2) became available for long-term administration, all nonresponders, as well as those who failed to improve clinically and hemodynamically on calcium channel blockers, were treated with long-term PGI(2). In the 31 responders, calcium channel blockers improved survival compared with the 43 nonresponders (P=0.0002). Survival was also better in 24 PGI(2)-treated nonresponders compared with 22 nonresponders for whom PGI(2) was unavailable (P=0.0005) as well as in all children who failed conventional therapy (n=31; P=0.002),Conclusions-Long-term vasodilator therapy improves survival in children with PPH. In acute responders, oral calcium channel blockers generally suffice. In both nonresponders to short-term testing and responders who fail to improve on calcium channel blockers, continuous intravenous infusion of PGI(2) improves survival.