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.

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Publication Details

Journal
Liver Transplantation
Published
2026-10-14
DOI
https://doi.org/10.1097/lvt.0000000000001018
Primary Topic
Organ Transplantation Techniques and Outcomes
Type
article
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article

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

Reinoud P.H. Bokkers, Martín de Santibañes, Victoria Ardiles, Julia Minetto et al.
Liver Transplantation
Organ Transplantation Techniques and Outcomes
article

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

Reinoud P.H. Bokkers, Martín de Santibañes, Victoria Ardiles, Julia Minetto, Amar Mukund, Viniyendra Pamecha, Emmanuel M. Gonzales, David P. Duncan, Martin Delle, Jai V. Patel, Catalina Jaramillo, Richard J. Hendrickson, Bader A Alfares, María Mercadal‐Hally, Małgorzata Markiewicz‐Kijewska, Henkjan J. Verkade, Denise Aldrian, Stéphanie Franchi‐Abella, Marumbo Paul Mtegha, Winita Hardikar, Mureo Kasahara, Hubert P. J. van der Doef, Piotr Kaliciński, Adam Koleśnik, Ryan T. Fischer, Florent Guérin, Georg Friedrich Vogel, Valeria Berchtold, Domenico Pinelli, Lorenzo D’Antiga, Helen Evans, Amit A. Shah, Rajeev Khanna, Thomas Casswall, Gerton A. Lunter, Vidyadhar Padmakar Mali, Ekkehard Sturm, Leandra Bitterfeld, Greg Nowak, Guillermo Cervio, Steffen Hartleif, Michael R. Acord, Seisuke Sakamoto, John L McCall, Paolo Marra, Ruben H J de Kleine, Barbara Elisabeth Wildhaber, Rajendra K. Prasad, Hajime Uchida, Marisa Renata Beretta, Phoebe Wood, Jimmy Walker Uño, Lydia Sieben, Arielle Melen, Mauricio Larrarte-King, Gibson C. Ugwu, Marion Aw, Sergio Sierre, Jesús Q. Bernabeu, on behalf of the PORTAL registry investigators, Bhargava Mullapuddi
article en

Abstract

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.

Liver Transplantation
University of Geneva (CH), Hebron University (PS), Royal Children's Hospital (AU), Primary Children's Hospital (US), Children's Mercy Hospital (US), University Medical Center Groningen (NL), Children's Hospital of Philadelphia (US), Innsbruck Medical University (AT), University of Groningen (NL), Universität Innsbruck (AT), University of the Witwatersrand (ZA), University of Utah (US), Université Paris-Saclay (FR), Leeds Teaching Hospitals NHS Trust (GB), Garrahan Hospital (AR), King Faisal Specialist Hospital & Research Centre (SA), Hospital Italiano de Buenos Aires (AR), Karolinska Institutet (SE), Assistance Publique – Hôpitaux de Paris (FR), University Hospital of Geneva (CH), Ospedale Papa Giovanni XXIII (IT), Children's Memorial Health Institute (PL), Wits University Donald Gordon Medical Centre (ZA), Institute of Liver and Biliary Sciences (IN), University Children's Hospital Tübingen (DE), Vall d'Hebron Hospital Universitari (ES), National Center For Child Health and Development (JP), Starship Children's Health (NZ), National University Hospital (SG), Bicêtre Hospital (FR), Laboratoire d'Imagerie Biomédicale Multimodale Paris-Saclay (FR), University of Milano-Bicocca (IT), University of Tübingen (DE)
Openalex Percentile: Top 10%
Organ Transplantation Techniques and Outcomes
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