儿童肺动脉高压定义和诊断及治疗的研究进展

Research Progress of Definition,Diagnosis and Treatment for Pulmonary Hypertension in Children

肺动脉高压(PAH)是一种以肺动脉压力和肺血管阻力升高为特征的肺血管疾病,常引起临床血流动力学改变,严重者可导致右心衰竭甚至死亡。近年来,随着基因诊断技术及心导管术的发展,儿童PAH的诊断逐步规范,但目前为止,尚无特异性治疗药物可以完全治愈,研究人员对PAH靶向治疗的有效性和安全性也在不断探索及总结,尤其是对西地那非的使用存在诸多争议。本文主要对儿童PAH的定义、诊断及治疗进行综述,以期为儿童PAH规范化诊疗提供借鉴。

Pulmonary hypertension(PAH)is a pulmonary vascular disease characterized by increased pulmonary artery pressure and pulmonary vascular resistance.It often causes changes in clinical hemodynamics,it can also lead to right heart failure and death in severe cases.In recent years,with the development of gene diagnosis technology and cardiac catheterization procedure,the diagnosis of PAH in children has been gradually standardized.But so far,there is no specific treatment can completely cure PAH,healer for targeted therapeutic efficacy and safety of PAH are also constantly explored and summarized,especially the use of sildenafil has more controversy.This paper will review the definition,diagnosis and treatment for PAH in children,in order to provide reference for children PAH standardized diagnosis and treatment.