Class 1. Pulmonary Hypertension Associated with Liver Disease

Congenital portosystemic shunts: diagnosis and treatment

Stéphanie Franchi-Abella, Emmanuel Gonzales, Oanez Ackermann, Sophie Banchereau, Daniéle Pariente, Florent Guérin, International Registry of Congenital Poertosystemic Shunt MembersHôpital Bicêtre, Hôpitaux Universitaire Paris-Sud and Assistance Publique Hôpitaux de Paris. National Reference Centre for Rare Pediatric Liver Diseases and Filfoie. University Paris -Sud. France Abdominal RadiologyAbd Radiol 2018; 43: 2023-2036DOI: 10.1007/s00261-018-1619-8 AbstractCongenital portosystemic shunts (CPSS) are rare

Congenital portosystemic shunts: diagnosis and treatment Read More »

Recurrence of Primary Sclerosing Cholangitis After Pediatric Liver Transplantation: A Single-Center, Retrospective Study in Japan

Athaya Vorasittha, Seisuke Sakamoto, Yusuke Yanagi, Kazuki Degawa, Hirotaka Kato, Tasuku Kodama, Ryuji Komine, Masaki Yamada, Hajime Uchida, Akinari Fukuda, Chiduko Haga, Takako Yoshioka, Mureo KasaharaNational Center for Child Health and Development. Japan Pediatric TransplantationPediatr Transplant 2025; 29: DOI: 10.1111/petr.70078 AbstractAim: Liver transplantation (LT) is a well-accepted treatment for primary sclerosing cholangitis (PSC) with generally good outcomes,

Recurrence of Primary Sclerosing Cholangitis After Pediatric Liver Transplantation: A Single-Center, Retrospective Study in Japan Read More »

Prevalence and Predictors of Pulmonary Hypertension in Children with Portal Hypertension: A Single Center Study

Nehal El Koofy, Sawsan Hassan Okasha, Hala Mounir Agha, Noha Ali, Ahmed Said Behairy, Hanan Mina Fouad, Rehab Hamdy ZawamCairo University, Helwan University.Egypt Pediatric Gastroenterology Hepatology and NutritionPediatr Gastroenterol Hepatol Nutr 2025; 28: 101-111DOI: 10.5223/pghn.2025.28.2.101 AbstractPurpose: This study aimed to estimate the prevalence and predictors of portopulmonary hypertension (POPH) in children with portal hypertension.Methods: We recruited children of

Prevalence and Predictors of Pulmonary Hypertension in Children with Portal Hypertension: A Single Center Study Read More »

Pulmonary vascular complications of cirrhosis: hepatopulmonary syndrome and portopulmonary hypertension

Maïté Verstraeten, Sander Lefere, Sarah RaevensGhent University Hospital and Ghent University.Belgium Acta Clinica BelgicaActa Clin Belg 2025; DOI: 10.1080/17843286.2025.2456697 AbstractHepatopulmonary syndrome (HPS) and portopulmonary hypertension (POPH) are two distinct pulmonary vascular complications seen in patients with liver disease and/or portal hypertension. HPS is characterized by disturbed gas exchange and hypoxemia because of intrapulmonary vascular dilatations. POPH

Pulmonary vascular complications of cirrhosis: hepatopulmonary syndrome and portopulmonary hypertension Read More »

Novel Staged Device Occlusion of Portosystemic Shunt With Atrial Flow Regulator in IVC Stent Platform

Seán T. Kelleher, Kevin P. Walsh, Damien Kenny, Emer Fitzpatrick, Elchanan Bruckheimer, S. Murthy Chennapragada, Pradeep Govender, Philip A. RobertsChildren’s Health Ireland at Crumlin. Schneider Children’s Medical Centre of Israel. Children’s Hospital at Westmead. Tallaght University Hospital.Ireland, Israel and Australia Journal of the American Colege of Cardiology Case ReportsJACC Case Rep 2024; 29: DOI: 10.1016/j.jaccas.2024.102340 AbstractWe

Novel Staged Device Occlusion of Portosystemic Shunt With Atrial Flow Regulator in IVC Stent Platform Read More »

Impact of Portal Flow on the Prognosis of Children With Congenital Portosystemic Shunt: A Multicentric Observation Study in Japan

Hajime Uchida, Masato Shinkai, Hiroomi Okuyama, Takehisa Ueno, Mikihiro Inoue, Toshihiro Yasui, Eiso Hiyama, Sho Kurihara, Yasunasru, Sakuma, Yukihiro Sanada, Akinobu, Taketomi, Shohei Honda, Motoshi Wada, Ryo Ando, Jun Fujishiro, Mariko Yoshida, Yohei Yamada, Hiroo Uchida, Takahisa Tainaka, Mureo Kasahara, Japanese Society of Pediatric Splenology PortalvenologyNational Center for Child Health and Development. Kanagawa Children’s Medical

Impact of Portal Flow on the Prognosis of Children With Congenital Portosystemic Shunt: A Multicentric Observation Study in Japan Read More »

The multisystem nature of isomerism: left isomerism complicated by Abernethy malformation and portopulmonary hypertension

Megan L. Ringle, Rohit Loomba, John C. Dykes, Danyal Khan, David Schidlow, Gil WernovskyLucile Packard Children’s Hospital and Stanford University School of Medicine. Advocate Children’s Hospital and Chicago Medical School. Nicklaus Children’s Hospital. Boston Children’s Hospital and Harvard Medical School. Children’s National Hospital.United States Cardiology in the YoungCardiol Young 2021; 31: 532-540DOI: 10.1017/S1047951121000809 AbstractIsomerism, also referred

The multisystem nature of isomerism: left isomerism complicated by Abernethy malformation and portopulmonary hypertension Read More »

Expert management of congenital portosystemic shunts and their complications

Valérie Anne McLin, Stéphanie Franchi-Abella, Timothée Brütsch, Atessa Bahadori, Valeria Casotti, Jeande Villede Goyet, Grégoire Dumery, Emmanuel Gonzales, Florent Guérin, Sebastien Hascoet, Nigel Heaton, Béatrice Kuhlmann, Frédéric Lador, Virginie Lambert, Paolo Marra, Aurélie Plessier, Alberto Quaglia, Anne-Laure Rougemont, Laurent Savale, Moinak Sen Sarma, Olivier Sitbon, Riccardo Antonio Superina, Hajime Uchida, Mirjamvan Albada, Hubert Petrus Johannes

Expert management of congenital portosystemic shunts and their complications Read More »

Trans-catheter closure of an unusual type of Abernethy malformation in a child presenting with severe pulmonary hypertension

Santosh Wadile, Ashishkumar Banpurkar, Snehal KulkarniSri Sathya Sai Sanjeevani Centre for Child Heart Care and Training in Pediatric Cardiac Skills.India Cardiology in the YoungCardiol Young 2023;DOI: 10.1017/S1047951123004006 AbstractAbernethy malformation is a congenital extra-hepatic porto-systemic shunt. This malformation is characterized by an abnormal connection between the portal vein or its branches and one of the systemic veins.

Trans-catheter closure of an unusual type of Abernethy malformation in a child presenting with severe pulmonary hypertension Read More »

Human liver single nuclear RNA sequencing implicates BMPR2, GDF15, arginine, and estrogen in portopulmonary hypertension

Arun Jose, Jean M. Elwing, Steven M. Kawut, Michael W. Pauciulo, Kenneth E. Sherman, William C. Nichols, Michael B. Fallon, Francis X. McCormackUniversity of Cincinnati College of Medicine and Cincinnati Children’s Hospital Medical Center. Perelman School at the University of Pennsylvania. University of Arizona. United States Communications BiologyCommun Biol 2023; 6DOI: 10.1038/s42003-023-05193-3 AbstractPortopulmonary hypertension (PoPH) is

Human liver single nuclear RNA sequencing implicates BMPR2, GDF15, arginine, and estrogen in portopulmonary hypertension Read More »

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