儿童肺动脉高压治疗进展  

The Progress of Treatment in Pediatric Patients with Pulmonary Arterial Hypertension

作　　者：李强强[1] 顾虹[1] LI Qiang-qiang;GU Hong(Department of Pediatric Cardiology,Beijing Anzhen Hospital,Capital Medical University,Beijing100029,China)

机构地区：[1]首都医科大学附属北京安贞医院小儿心脏中心,北京100029

出　　处：《中国合理用药探索》2022年第5期1-6,共6页Chinese Journal of Rational Drug Use

基　　金：国家自然科学基金资助项目(82070243)。

摘　　要：动脉性肺动脉高压(PAH)是一类进展性肺血管疾病,会导致患者右心衰竭而死亡。我国儿童PAH以特发性PAH和与先天性心脏病相关PAH为主。儿童PAH的治疗包括常规治疗、PAH相关药物治疗(如内皮素受体拮抗剂、磷酸二酯酶Ⅴ型抑制剂和前列环素类似物等)、姑息性治疗和肺移植。本文旨在总结儿童PAH的治疗现状并阐述新的治疗理念。Pulmonary arterial hypertension(PAH)is a progressive pulmonary vascular disease,eventually leading to right heart failure and death.Children with PAH have a greater predominance of idiopathic PAH and PAH associated with congenital heart disease in China.Treatment for children with PAH comprises the conventional therapies,PAH therapies(eg.endothelin receptor antagonist,phosphodiesterase-Ⅴinhibitor and prostacyclin analogue),the palliative therapy and lung transplantation.In this review,we summarized the present status of pediatric PAH treatment and explained new treatment concepts.

关 键 词：动脉性肺动脉高压 儿童 药物治疗 先天性心脏病 预后 

分 类 号：R725.4[医药卫生—儿科]