2021 年第 7 期 第 16 卷

静脉或皮下应用曲前列尼尔在儿童肺动脉高压患者中的研究进展

Research progress on intravenous or subcutaneous treprostinil in children with pulmonary arterial hypertension

作者：彭锦莉李强强顾虹

英文作者：Peng Jinli Li Qiangqiang Gu Hong

单位：首都医科大学附属北京安贞医院小儿心脏中心北京市心肺血管疾病研究所100029

英文单位：Pediatric Heart Center Beijing Anzhen Hospital Capital Medical University Beijing Institute of Heart Lung and Blood Vessel Diseases Beijing 100029 China

关键词：肺动脉高压；曲前列尼尔；儿童

英文关键词：Pulmonaryarterialhypertension;Treprostinil;Children 

• 摘要：

• 肺动脉高压(PAH)是一组以肺小动脉管壁进行性增厚、管腔狭窄和肺血管阻力升高为特征的临床病理综合征，未经治疗的儿童PAH患者的生存率极低，预后极差，对于高危或快速进展性PAH患儿，均建议持续静脉/皮下应用前列环素类似物基础上进行联合治疗。曲前列尼尔是一种稳定的前列环素类似物，是我国目前临床上唯一的静脉/皮下用PAH靶向药物。本文就曲前列尼尔对于儿童PAH患者的安全性及有效性作一阐述。

• Pulmonary arterial hypertension (PAH) is a group of clinicopathological syndrome characterized by pulmonary arteriolar wall progressively thickening, vessel stenosis and pulmonary vascular resistance elevating. PAH children without treatment have extremely poor survival and poor prognosis. In severe or rapidly progressive PAH children, combination therapy based on continuous intravenous/subcutaneous prostacyclin analogue is recommended. Treprostinil, a kind of prostacyclin analogue, is the only intravenous/subcutaneous drug for the treatment of PAH in China. This review elaborates the safety and efficacy of treprostinil in children with PAH.