Abstract: Congenital pulmonary vascular disease are a group of diseases，which are caused by deformity of the anatomical morphology and structure of pulmonary arteries，pulmonary veins，and pulmonary vascular plexus during the embryonic development. In recent years，there have been increasing reports of such diseases. However，there is no comprehensive and systematic classification of congenital pulmonary vascular disease，which leads to confusion in the clinical diagnosis and treatment. This article summarizes the classification，clinical manifestations，diagnostic methods and treatment strategies of congenital pulmonary vascular disease.

Key words: congenital pulmonary vascular abnormality, classification, clinical manifestation, diagnosis, therapy

摘要： 先天性肺血管病指在人体胚胎发育过程中，肺动脉、肺静脉和肺血管丛形成或连接异常而引起的结构畸形。近年来，关于先天性肺血管病的病例报告日益增多，但尚无统一分类，影响了该类疾病的临床诊疗。该文介绍了先天性肺血管病的分类、临床表现、诊断方法和治疗进展。

关键词: 先天性肺血管异常, 分类, 临床表现, 诊断, 治疗