Serum bile acid levels predict the development of portal hypertension and high-risk esophageal varices following successful Kasai in biliary atresia

Background and Aims: Biliary atresia can lead to portal hypertension (PH), high-risk esophageal varices (HRV), and liver transplantation, despite a successful Kasai procedure (KP). Serum bile acids (sBA) can reflect residual cholestasis in patients with successful KP. We investigated the predictive value of sBA measured during the first year after KP on the development of PH and HRV within 5 years after KP. Approach and Results: In this retrospective monocentric observational study, we included patients with biliary atresia who underwent a successful KP, defined as a total serum bilirubin level of ≤25 μmol/L within 6 months after KP, and in whom sBA were measured during the first year after KP (n=60). We collected clinical, biological, and digestive endoscopic data up to 5 years after KP to assess the occurrence of PH and HRV. Predictive values of sBA were analyzed using receiver operating characteristic curves. sBA measured at a median time of 6 months (range: 4.5–9) and of 11 months (range: 9–12) after KP, predicted PH at 3 and 5 years after KP, with AUCs between 0.89 and 0.93 ( p <0.0003), and the occurrence of HRV within 5 years after KP with AUCs between 0.74 and 0.75 ( p <0.04). sBA thresholds of 56 and 30 μmol/L at a median time of 6 and 11 months after KP, respectively, predicted HRV within 5 years after KP with a sensitivity of 100%. Conclusions: In patients with biliary atresia with successful KP, sBA is an early biomarker predicting the development of PH and HRV within 5 years after KP.