Introduction: Portal vein stenosis (PVS) represents a significant complication following pediatric liver transplantation (pLT). While PVS can potentially compromise graft function and patient outcomes, its actual impact on survival remains poorly characterized. This study presents the first global registry-based analysis of one-year patient- and graft survival in post-pLT PVS patients.
Methods: The study analyzed data from the Portal vein Obstruction Revascularisation Therapy After Liver transplantation (PORTAL) registry. This registry contains data from 21 centers, across 18 countries, 6 continents over a 20-year cohort. Graft- and patient survival was determined for the first year following PVS-diagnosis using the Kaplan-Meier method and a Cox proportional hazards model.  
Results: We included 239 patients (53% female; median age at pLT, 1.1 years) diagnosed with PVS. Median age at PVS-diagnosis was 2.7 years, and the median pLT-PVS interval was 5.8 months. The one-year graft survival rate was 96%, and the one-year patient survival rate was 99%. Multivariable analysis revealed several independent factors associated with decreased survival outcomes. The presence of a venous jump/interposition graft during pLT was associated with a reduced graft-, as well as patient survival (p=0.003 and p=0.015, respectively). Additionally, early onset diagnosis (≤14 days after pLT, p=0.017), age at pLT less than 1 year (p=0.003) and male gender (p=0.018) emerged as significant patient-related risk factors to the occurrence of graft loss.
Conclusions: Patients with PVS after pLT have excellent graft- and patient survival. Several risk factors which lower patient outcome were identified. Long-term outcomes could be enhanced by targeting these risk factors.