Mortality Determinants in Children with Biliary Atresia Awaiting Liver Transplantation

Abstract

To determine risk factors for waitlist mortality in children with biliary atresia listed for liver transplantation. There were 2704 children with biliary atresia (<12years of age) listed for a first liver transplant (2002-2018) in the United Network for Organ Sharing database. Fine-Gray regression models for competing risks analysis (main risk=waitlist mortality/delisting owing to too sick; competing risk=liver transplantation) were implemented to identify risk factors for waitlist mortality. The median waitlist time was 83days (IQR, 34-191). The cumulative incidence of waitlist mortality was 5.2%. In multivariable analysis (n=2253), increasing bilirubin level (P<.001), portal vein thrombosis (P=.03), and ventilator dependence (P<.001) at listing were associated with a higher risk, whereas weight ≥10kg at listing (P=.009) was associated with a lower risk of waitlist mortality. When ascites at listing was included in multivariable analysis (n=1376), it was associated with a higher risk for the composite outcome (P=.03). Encephalopathy at listing was not associated with waitlist mortality (n=1376; P=.15). These parameters can be used to more objectively prioritize children with biliary atresia awaiting liver transplantation and identify children with biliary atresia-related end-stage liver disease at high-risk of mortality.