Advances in pulmonary vascular complications of congenital portosystemic shunts
10.3760/cma.j.issn.1673-4408.2025.02.003
- VernacularTitle:先天性门体静脉分流的肺血管并发症研究进展
- Author:
Yijia YANG
1
;
Daiji JIANG
;
Yi YAN
;
Xu ZHANG
;
Hao ZHANG
;
Lijun FU
Author Information
1. 国家儿童医学中心 上海交通大学医学院附属上海儿童医学中心心内科 200127
- Keywords:
Congenital portosystemic shunts;
Portopulmonary hypertension;
Hepatopulmonary syndrome;
Pathogenesis;
Diagnosis and treatment
- From:
International Journal of Pediatrics
2025;52(2):83-87
- CountryChina
- Language:Chinese
-
Abstract:
Congenital portosystemic shunts（CPSS）are vascular malformations caused by developmental anomalies that create abnormal shunts between the portal and systemic veins.This anomaly permits partial or complete bypass of portal blood flow from the liver，leading it directly into the systemic circulation without hepatic filtration.If untreated，CPSS can lead to multisystem complications，with pulmonary vascular complications being the most severe outcome.These complications are complex in mechanism，often insidious in onset，and present with nonspecific symptoms，increasing the risk of delayed diagnosis and significantly impacting the quality of children lives.Pulmonary vascular complications associated with CPSS primarily include portopulmonary hypertension，characterized by pulmonary vascular remodeling，and hepatopulmonary syndrome，distinguished by intrapulmonary vascular dilatation and abnormal arterial oxygenation.This article aims to review the anatomical classifications of CPSS，along with the pathophysiology，clinical presentation，diagnosis，and treatment of its pulmonary vascular complications，with the goal of enhancing clinical awareness and providing a reference for diagnosis and management.
