儿童肺动脉高压治疗进展

Advances in treatment of children with pulmonary arterial hypertension

肺动脉高压(pulmonary arterial hypertension,PAH)是由多种诱因引起的肺血管重构、肺小动脉痉挛、血管内皮细胞及平滑肌细胞增殖等,使得肺动脉压(pulmonary arterial pressure,PAP)逐渐升高,终致右心衰竭,甚至死亡。近年由于其发病机制的研究及新治疗药物的研发,PAH患者预后明显改善。PAH的靶向治疗药物主要作用于一氧化氮(nitric oxide,NO)、前列环素(prostaglandin I_(2),PGI_(2))、内皮素(endothelin-1,ET-1)三条经典途径中的不同靶点。其应用及联合应用、手术治疗等方法均大大改善了PAH患者的生存率及生存质量。相对于成人来说,儿童多是先天性心脏病相关PAH(PAH associated with congenital heart disease,PAH-CHD)、特发性PAH(idiopathic PAH,IPAH),发病年龄小,还未出现肺血管病变,因此预后更好。该文就儿童肺动脉高压的治疗新进展作一综述。

Pulmonary arterial hypertension(PAH)is caused by pulmonary vascular remodeling,pulmonary arteriolar spasm,proliferation of vascular endothelial cells and smooth muscle cells,which gradually increase the pulmonary arterial pressure(PAP),eventually leads to right heart failure and even death.In recent years,due to the research of pathogenesis and the development of new drugs,the prognosis of patients with PAH has been significantly improved.The targeted drugs of PAH mainly act on different targets in three classic pathways of nitric oxide(NO),prostacyclin I_(2)(PGI_(2))and endothelin-1(ET-1).Its application,combined application,surgical treatment and other methods have significantly improved the survival rate and life quality of patients with PAH.Compared with adults,children are more likely to have PAH associated with congenital heart disease(PAH-CHD)and idiopathic PAH(IPAH).As the onset age is young and pulmonary vascular lesions have not yet emerged,children can have a better prognosis.This paper reviews new treatment in children with PAH.