Jie Zhang, Gong Chen
Children’s Hospital of Fudan University.
China
World Journal of Pediatric Surgery
World J Pediatr Surg 2025; 8:
DOI: 10.1136/wjps-2025-001067
Abstract
Following Kasai portoenterostomy for biliary atresia (BA), long-term survival in children is associated with chronic liver cirrhosis leading to hepatopulmonary syndrome (HPS) and portopulmonary hypertension (PoPH). This further causes a diverse array of respiratory and circulatory symptoms. The incidence of these complications in children with BA is higher than that seen in other chronic liver diseases. With a faster progression, BA is life-threatening in some severe cases. Moreover, HPS and PoPH may coexist in the same child. Enhancing our understanding of HPS/PoPH, making an early diagnosis, rational selection of medication, and appropriate timing of liver transplantation underscore the need for multidisciplinary collaboration.
Category
Class I. Pulmonary Hypertension Associated with Liver Disease
Pulmonary Arteriovenous Malformations
Symptoms and Findings Associated with Pulmonary Vascular Disease
Review Articles Concerning Pulmonary Vascular Disease
Age Focus: Pediatric Pulmonary Vascular Disease
Fresh or Filed Publication: Fresh (PHresh). Less than 1-2 years since publication
Article Access
Free PDF File or Full Text Article Available Through PubMed or DOI: Yes