Mortality Determinants in Children with Biliary Atresia Awaiting Liver Transplantation

Objective To determine risk factors for waitlist mortality in children with biliary atresia listed for liver transplantation. Study design There were 2704 children with biliary atresia (<12 years 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. Results The median waitlist time was 83 days (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 >= 10 kg 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). Conclusions 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.

Automated summary

The study identified factors such as increasing bilirubin level, portal vein thrombosis, and ventilator dependence at listing associated with higher risk of waitlist mortality, while weight >= 10 kg at listing associated with lower risk. Ascites at listing was also found to be associated with a higher risk for the composite outcome.