Research ressources

The IRCPSS is active in research to improve the care of patients with CPSS. Active areas of investigation include the pathophysiology of CPSS, the search for the genetic underpinnings, and identification of patients at risk of developing complications.

Expert management of congenital portosystemic shunts and their complications.

McLin VA, Franchi-Abella S, Brütsch T, Bahadori A, Casotti V, de Ville de Goyet J, Dumery G, Gonzales E, Guérin F, Hascoet S, Heaton N, Kuhlmann B, Lador F, Lambert V, Marra P, Plessier DA, Quaglia A, Rougemont AL, Savale L, Sarma MS, Sitbon O, Superina RA, Uchida H, van Albada M, Johannes van der Doef HP, Vilgrain V, Wacker J, Zwaveling-Soonawala N, Debray D, Wildhaber BE.
JHEP Rep. 2023 Oct 20;6(1):100933. doi: 10.1016/j.jhepr.2023.100933. eCollection 2024 Jan.
PMID: 38234409

International registry of congenital porto-systemic shunts: a multi-centre, retrospective and prospective registry of neonates, children and adults with congenital porto-systemic shunts

Simona Korff, Khaled Mostaguir, Maurice Beghetti, Lorenzo D’Antiga, Dominique Debray, Stéphanie Franchi-Abella, Emmanuel Gonzales, Florent Guerin, Anne-Lise Hachulla, Virginie Lambert, Periklis Makrythanasis, Nicolas Roduit, Laurent Savale, Marie-Victoire Senat, Joël Spaltenstein, Frank van Steenbeek, Barbara E Wildhaber, Marcel Zwahlen, Valérie A McLin
Orphanet J Rare Dis. 2022 Jul 19;17(1):284. doi: 10.1186/s13023-022-02412-8.
PMID: 35854389

Presentation of Congenital Portosystemic Shunts in Children.

Bahadori A, Kuhlmann B, Debray D, Franchi-Abella S, Wacker J, Beghetti M, Wildhaber BE, McLin VA, On Behalf Of The Ircpss.
Children (Basel). 2022 Feb 11;9(2):243. doi: 10.3390/children9020243.
PMID: 35204963 Free PMC article. Review.

Current Quandaries in the Management of Congenital Portosystemic Shunts.

McLin V, Beghetti M, D’Antiga L, Franchi-Abella S; International Registry of Congenital Porto-Systemic Shunts (IRCPSS).
Liver Transpl. 2021 Aug;27(8):1212-1213. doi: 10.1002/lt.26055. Epub 2021 Jul 20.
PMID: 33749116 No abstract available.

Expert management of congenital portosystemic shunts and their complications.

Valérie Anne McLin, Stéphanie Franchi-Abella, Timothée Brütsch, Nitash Zwaveling, Dominique Debray, Barbara Elisabeth Wildhaber, et al.
Open Access. Published:October 19, 2023

Reliable Detection of Intrapulmonary Shunts Using Contrast-Enhanced Echocardiography in Children With Portal Hypertension or Portosystemic Shunt.

Rock NM, Beghetti M, Tissot C, Willi JP, Bouhabib M, McLin VA, Maggio ABR.
J Pediatr Gastroenterol Nutr. 2021 Jul 1;73(1):73-79. doi: 10.1097/MPG.0000000000003079.
PMID: 33605662

Cardiovascular disorders in patients with congenital portosystemic shunts: 23 years of experience in a tertiary referral centre.

Lambert V, Ladarre D, Fortas F, Durand P, Hervé P, Gonzales E, Guérin F, Savale L, McLin VA, Ackermann O, Franchi-Abella S; International Registry of Congenital Portosystemic Shunts (IRCPSS).
Arch Cardiovasc Dis. 2021 Mar;114(3):221-231. doi: 10.1016/j.acvd.2020.10.003. Epub 2020 Dec 3.
PMID: 33281106

Outcome of paediatric portopulmonary hypertension in the modern management era: A case report of 6 patients.

Joye R, Lador F, Aggoun Y, Farhat N, Wacker J, Wildhaber BE, Vallée JP, Hachulla AL, McLin VA, Beghetti M.
J Hepatol. 2021 Mar;74(3):742-747. doi: 10.1016/j.jhep.2020.11.039. Epub 2020 Dec 1.
PMID: 33276028

Congenital Portosystemic Shunts: Current Diagnosis and Management.

McLin VA, Franchi Abella S, Debray D, Guérin F, Beghetti M, Savale L, Wildhaber BE, Gonzales E; Members of the International Registry of Congenital Porto-Systemic Shunts.
J Pediatr Gastroenterol Nutr. 2019 May;68(5):615-622. doi: 10.1097/MPG.0000000000002263.
PMID:30628988 Review.

Congenital portosystemic shunts: Vascular liver diseases: Position papers from the francophone network for vascular liver diseases, the French Association for the Study of the Liver (AFEF), and ERN-rare liver.

Guérin F, Franchi Abella S, McLin V, Ackermann O, Girard M, Cervoni JP, Savale L, Hernandez-Gea V, Valla D, Hillaire S, Dutheil D, Bureau C, Gonzales E, Plessier A.
Clin Res Hepatol Gastroenterol. 2020 Sep;44(4):452-459. doi: 10.1016/j.clinre.2020.03.004. Epub 2020 Apr 9.
PMID: 32279979 No abstract available.

Globus pallidus MR signal abnormalities in children with chronic liver disease and/or porto-systemic shunting.

Hanquinet S, Morice C, Courvoisier DS, Cousin V, Anooshiravani M, Merlini L, McLin VA.
Eur Radiol. 2017 Oct;27(10):4064-4071. doi: 10.1007/s00330-017-4808-x. Epub 2017 Apr 6.
PMID: 28386718

The C57BL/6J mouse exhibits sporadic congenital portosystemic shunts.

Cudalbu C, McLin VA, Lei H, Duarte JM, Rougemont AL, Oldani G, Terraz S, Toso C, Gruetter R.
PLoS One. 2013 Jul 23;8(7):e69782. doi: 10.1371/journal.pone.0069782. Print 2013.
PMID: 23936100 Free PMC article.