先天性门体静脉分流相关性肺动脉高压5 例临床分析

doi:10.3969/j.issn.1000-3606.2019.12.017

临床儿科杂志 ›› 2019, Vol. 37 ›› Issue (12): 946-.doi: 10.3969/j.issn.1000-3606.2019.12.017

先天性门体静脉分流相关性肺动脉高压5 例临床分析

肖云彬 1,2，　曾云红 1,2，　肖政辉 3，　李文凤 1,2，　陈智 1，　杨舟 1，　向金星 1，　左超 1，　王丹 1

1.湖南省儿童医院心血管内科（湖南长沙　410007）；2.南华大学儿科学院（湖南长沙　410007）； 3.湖南省儿童医院重症医学科（湖南长沙　410007）

发布日期:2020-02-03
通讯作者: 陈智　电子信箱：eychenzhi@163.com
基金资助:
国家自然科学基金(No.81500041)；湖南省临床医疗技术创新引导项目（No. 2018SK50413）；湖南省重点实验室平台项目（No. 2018TP1028）

Clinical analysis of pulmonary arterial hypertension associated with congenital portosystemic shunt in children

　XIAO Yunbin1,2, ZENG Yunhong1,2, XIAO Zhenghui3, LI Wenfeng1,2, CHEN Zhi1, YANG Zhou1, XIANG Jinxing1, ZUO Chao1, WANG Dan1

1. Department of Cardiology, Hunan Children's Hospital, Changsha 410007, Hunan, China; 2. Academy of Pediatrics, University of South China, Changsha 410007, Hunan, China; 3. Intensive Care Unit, Hunan Children's Hospital, Changsha 410007, Hunan, China

Published:2020-02-03

摘要/Abstract

摘要： 　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。

关键词: 先天性门体静脉分流；　肺动脉高压；　儿童

Abstract: 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.

Key words: congenital portosystemic shunt;　pulmonary arterial hypertension;　child

肖云彬，曾云红，肖政辉，等. 先天性门体静脉分流相关性肺动脉高压5 例临床分析[J]. 临床儿科杂志, 2019, 37(12): 946-.

XIAO Yunbin, ZENG Yunhong, XIAO Zhenghui, et al. Clinical analysis of pulmonary arterial hypertension associated with congenital portosystemic shunt in children[J]. Journal of Clinical Pediatrics, 2019, 37(12): 946-.

先天性门体静脉分流相关性肺动脉高压5 例临床分析

Clinical analysis of pulmonary arterial hypertension associated with congenital portosystemic shunt in children

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