Chen Ju-Yin, Chang Mei-Hwei, Ho Ming-Chih, Peng Shinn-Forng, Hsu Wen-Ming, Lin Wen-Hsi, Wu Jia-Feng
Department of Pediatrics, Taipei City Hospital Women and Children Campus, Taipei, Taiwan; Departments of Pediatrics, National Taiwan University Hospital, Taipei, Taiwan.
Departments of Pediatrics, National Taiwan University Hospital, Taipei, Taiwan.
Pediatr Neonatol. 2025 Jul;66(4):363-367. doi: 10.1016/j.pedneo.2024.06.012. Epub 2024 Nov 14.
Hepatopulmonary syndrome (HPS) is a complication in biliary atresia (BA) children following hepatoportoenterostomy. Liver transplantation (LT) was the definitive treatment of HPS. However, little was known about the risk factors between HPS and mortalities. We aimed to evaluate the role of HPS and the predictors of complications after LT in BA children.
One hundred and twenty (54 males and 66 females) children with BA receiving LT were retrospectively enrolled. The primary outcome was postoperative biliary/vascular complication rates and the secondary outcome was post-LT mortality rates.
Among 120 BA children receiving LT, six (5%) children were diagnosed with HPS before LT. The overall survival rate of LT in BA children was 80% and the overall survival rate of LT in BA children with and without HPS was 17% and 83%, respectively. Vascular complications and HPS were predictors for poor overall survival rates both in univariate logistic regression analyses (hazard ratio [HR], 11.63 and 5.96; P < 0.0001 and P = 0.001, respectively) and multivariate logistic regression analyses (HR, 10.02 and 4.16; P < 0.0001 and P = 0.007, respectively). Kaplan-Meier analysis indicated the predictive role of HPS on poor overall survival rates (P < 0.0001), higher risks of biliary complications (P < 0.0001), and higher risks of jaundice (P < 0.01) post-LT.
The present study comprising children over long-term follow-up revealed that the development of pre-LT HPS had a poor impact on overall survival rates and higher risks of biliary complications in BA children receiving LT.
