目的　探讨儿童先天性门体静脉分流相关性肺动脉高压（PAH-CPS）的临床特点。方法　回顾分析2012年1 月至2019年1月收治的PAH-CPS患儿的临床资料。结果　共5例患儿，男2例、女3例，初诊年龄0.08~4.4岁。主要表现 为反复肺部感染、肝脾大、肝功能异常、肺动脉高压。超声心动图检查明确患儿均有不同程度肺动脉高压、合并心内畸形。 腹部超声示肝内外门体静脉分流。腹部增强CT和心导管造影可显示肝内外血管的走形和发育。结论　PAH-CPS可表现 为反复肺部感染、肝脾肿大、肝功能异常等；超声检查可提供诊断线索，腹部增强CT扫描、心导管造影可明确诊断。

Objective　To explore the clinical characteristics of pulmonary arterial hypertension associated with congenital portosystemic shunt (PAH-CPS) in children. Method　The clinical data of PAH-CPS in children admitted from January 2012 to January 2019 were retrospectively analyzed. Results　In 5 children (2 males and 3 females)， the age at initial diagnosis was from 0.08 to 4.4 years. The main manifestations of 5 patients were recurrent pulmonary infection, hepatosplenomegaly, abnormal liver function and pulmonary arterial hypertension. Echocardiography confirmed that all the children had different degrees of pulmonary arterial hypertension and intracardiac malformations. Abdominal ultrasonography showed intrahepatic and extrahepatic portosystemic shunts. Abdominal contrast-enhanced CT and cardiac catheterization showed the formation of intrahepatic and extrahepatic vessels. Conclusions　PAH-CPS can be manifested as repeated pulmonary infection, hepatosplenomegaly, abnormal liver function, etc. Ultrasonography can provide diagnostic clues. Abdominal enhanced CT scan and cardiac catheterization can make a definite diagnosis.