Long-term graft and patient survival of patients with portal vein stenosis after pediatric liver transplantation; results from the multicenter multinational PORTAL registry

Background and aims: Pediatric liver transplantation (pLT) has become a standard treatment, but it is still burdened by complications such as portal vein stenosis (PVS). PVS can result in compromised graft function and portal hypertension if left untreated. We aimed to assess long-term patient and graft survival after PVS diagnosis after pLT. Methods: Our study analyzed data from the Portal vein Obstruction Revascularisation Therapy After Liver transplantation (PORTAL) registry, which contains retrospective data from 21 centers, across 18 countries, over a 20-year period. Graft- and patient survival rates were determined after PVS diagnosis. Results : This study included 239 patients (53% female; median age at pLT 1.1 years) diagnosed with PVS. The median age at diagnosis was 2.7 years. The overall ten-year graft survival was 90%, and the ten-year patient survival was 96%. One-year graft and patient survival was lowest in patients with PVS diagnosed ≤7 days after pLT (80% and 92%, respectively). Initial treatment strategies comprised of mostly endovascular procedures (87%), followed by conservative (11%) and surgical treatment (3%). Patient outcome did not differ between these treatment groups. Within the endovascular group, graft survival in patients treated with stent (n=26) was significantly lower compared to those with percutaneous transluminal angioplasty (n=181) (88% vs 98%, respectively, p =0.01). Conclusions: Our findings indicate excellent graft and patient survival after PVS diagnosis and its treatment. Further research is needed to determine the safety and efficacy of optimal management strategies, especially in early-onset PVS.