儿童肺高压诊治进展

Advances in the diagnosis and treatment of pediatric pulmonary hypertension

儿童肺动脉高IN,（PAH）与多种诱因有关，发病率和病死率均较高，随着新型PAH药物的发现和循证依据充分指南的制定，成人PAH生存率得到明显改善。但儿童PAH在病理生理、潜在因素、临床表现及最终预后方面都有明显不同。为儿童PAH制定目标导向性治疗，对改变儿童PAH不良预后具有重要意义。本文就儿童肺高压目前的诊治进展进行综述。

Pulmonary arterial hypertension （PAH） in children is associated with various underlying diseases and unknown causes and leads to significant morbidity and mortality. With the discovery of novel drugs and the formulation of evidence-based guidelines, the survival of adult PAH has improved significantly. However, children and adults vary greatly in pathophysiological causes, precipitating factors, clinical manifestations and ultimate outcomes. For achieving a better prognosis in children, offering goal-oriented treatments are essential. This review provides an overview of recent advances of diagnosing and treating PAH in children.