Advances in treatment of children with pulmonary arterial hypertension / 国际儿科学杂志

ABSTRACT

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.