Hepatopulmonary syndrome in patients with porto-sinusoidal vascular disorder: Characteristics and outcome
Hepatopulmonary syndrome (HPS) is a rare complication of porto-sinusoidal vascular disorder (PSVD). This multicentric study found that patients with PSVD and HPS had less preserved liver function, and harbored genetic disorders more frequently (especially telomere biology disorders) than patients without HPS. HPS did not negatively impact transplantation-free survival when applying HPS MELD exception policy for liver transplantation through a competitive risk analysis. Our findings suggest that patients with PSVD with respiratory symptoms and/or telomere biology disorders may benefit from systematic screening for HPS.
Authors
- Aurélie Plessier (ORCID: https://orcid.org/0000-0001-5656-1622)
- Dominique Thabut (ORCID: https://orcid.org/0000-0002-9658-0323)
- Shantha Valainathan (ORCID: https://orcid.org/0000-0002-9980-2104)
- Isabelle Hourmand-Ollivier
- Vincent Olivier Mallet (ORCID: https://orcid.org/0000-0003-2219-9201)
- Pauline Houssel‐Debry (ORCID: https://orcid.org/0000-0001-9271-9810)
- Marion Tanguy (ORCID: https://orcid.org/0000-0002-3927-2228)
- Alexandra Heurgué (ORCID: https://orcid.org/0000-0003-0020-0590)
- Charlotte E. Costentin (ORCID: https://orcid.org/0000-0001-6115-8512)
- François Durand (ORCID: https://orcid.org/0000-0002-0357-1090)
- Laure Elkrief (ORCID: https://orcid.org/0000-0003-2843-1710)
- Armelle Poujol‐Robert (ORCID: https://orcid.org/0000-0002-6136-6511)
- Pierre‐Emmanuel Rautou (ORCID: https://orcid.org/0000-0001-9567-1859)
- Sabrina Sidali (ORCID: https://orcid.org/0000-0003-3282-9254)
- Valérie Paradis (ORCID: https://orcid.org/0000-0003-3142-3762)
- Sarah Raevens (ORCID: https://orcid.org/0000-0003-2810-7507)
- Adrien Lannes
- Lucile Moga (ORCID: https://orcid.org/0000-0002-9083-2315)
- Nicolas Drilhon
- Agnès Cachier
- Estelle Marcault
- Nathalie Ganne‐Carrié (ORCID: https://orcid.org/0000-0002-7351-5027)
- Kinan El Husseini (ORCID: https://orcid.org/0000-0001-9188-833X)
- Sophie Hillaire
- Ylang Spaes
- Odile Goria (ORCID: https://orcid.org/0000-0002-8786-3022)
- Jean‐Baptiste Nousbaum
- Anne Gervais
Institutions
- Université de Tours (FR)
- Centre National de la Recherche Scientifique (FR)
- Inserm (FR)
- Université Paris Cité (FR)
- Hôpital Beaujon (FR)
- Centre Hospitalier Universitaire de Grenoble (FR)
- Ghent University Hospital (BE)
- Sorbonne Université (FR)
- Hôpital Louis-Mourier (FR)
- Hôpital Charles-Nicolle (FR)
- Université Sorbonne Paris Nord (FR)
- Hôpital Cochin (FR)
- Centre Hospitalier Universitaire de Tours (FR)
- Assistance Publique – Hôpitaux de Paris (FR)
- Hôpital Saint-Antoine (FR)
- Centre Hospitalier Universitaire de Reims (FR)
- Centre Hospitalier Universitaire d'Angers (FR)
- Centre Hospitalier Universitaire de Caen Normandie (FR)
- Hôpital Nord (FR)
- Pitié-Salpêtrière Hospital (FR)
- Hôpital Avicenne (FR)
- Centre de Recherche sur l'Inflammation (FR)
- Centre Hospitalier Régional Universitaire de Brest (FR)
- Centre de Recherche Saint-Antoine (FR)
- Hôpital Saint-Louis (FR)
- Hôpital Bichat-Claude-Bernard (FR)
- Centre Hospitalier Universitaire de Rennes (FR)
- Hôpital Foch (FR)
- Université Grenoble Alpes (FR)
- Université de Reims Champagne-Ardenne (FR)
Publication Details
- Journal
- JHEP Reports
- Published
- 2024-12-20
- DOI
- https://doi.org/10.1016/j.jhepr.2024.101310
- Citations
- 2
- Primary Topic
- Liver Disease and Transplantation
- Type
- article
- Field-Weighted Citation Impact
- 0.74
Funders
- European Commission
- Agence Nationale de la Recherche
- Institut National de la Santé et de la Recherche Médicale
- Fondation pour la Recherche Médicale
- Fondation ARC pour la Recherche sur le Cancer