Portal hypertension in biliary atresia: a Japanese biliary atresia registry study.

PURPOSE: This study aimed to clarify the incidence of portal hypertension (PH) in biliary atresia (BA), its impact on long-term native liver survival rates (NLSRs), and the role of PH treatment.

METHODS

Data were analyzed from 3,777 patients in the Japanese Biliary Atresia Registry (1989-2021). Incidence and treatment of PH were examined in all patients and in 20-year native liver survivors. A subgroup of 596 jaundice-free native liver survivors was divided into three groups: without PH (A), untreated PH (B), and treated PH (C). Patient characteristics, clinical outcomes, and survival analyses were performed.

RESULTS

PH occurred in 46.4% of all patients, with gastroesophageal varices (35.1%) and hypersplenism (36.8%) being most common. Among 759 20-year native liver survivors, 49.8% had PH, largely diagnosed between 2 and 15 years. Group C patients were older at Kasai portoenterostomy and had more cholangitis. Survival analysis showed best outcomes in group A and worst in group C. Conditional native liver survival analysis revealed group B initially had better NLSRs than group C, but differences diminished beyond 15 years.

CONCLUSION

In native liver survivors without jaundice following KP, PH influenced prognosis, but successful treatment enabled long-term survival comparable to patients with mild or no PH.