Cholestasis after Kasai operation predicts portal hypertension in native liver survivors of biliary atresia – A multicentre study

Purpose This study evaluated portal hypertension (PHT) and its predictors among native liver survivors (NLS) of biliary atresia (BA) after Kasai portoenterostomy (KPE). Methods This was a multicenter study using prospectively collected data. The subjects were patients who remained transplant-free for 5 years after KPE. Their status of PHT was evaluated and variables that predicted PHT were determined by regression analysis and receiver operating characteristic (ROC) curve. Results Six centers from East Asia participated in this study and 320 subjects with KPE between 1980 to 2018 were analyzed. The mean follow-up period was 10.6 +/- 6.2 years. At the 5 ^th year after KPE, PHT was found in 37.8% of the subjects (n=121). Patients with KPE done before day 41 of life had the lowest percentage of PHT compared to operation at older age. At 12 months after KPE, PHT +ve subjects had a higher bilirubin level (27.1 +/- 11.7 vs 12.3+/-7.9 µmol/L, p=0.000) and persistent jaundice conferred a higher risk for PHT (OR=12.9 [9.2 – 15.4], p=0.000). ROC analysis demonstrated that a bilirubin level above 38 µmol/L at 12 months after KPE predicted PHT development (sensitivity: 78%, specificity: 60%, AUROC: 0.75). Conclusions In BA, early KPE protects against the development of PHT among NLSs. Patients with persistent cholestasis at one year after KPE are at a higher risk of this complication. They should receive a more vigilant follow up. Level of evidence : Level III