• Clément Lejealle, Valérie Paradis, Onorina Bruno, Emmanuelle de Raucourt, Claire Francoz, Olivier Soubrane, Didier Lebrec, Pierre Bedossa, Dominique Valla, Hervé Mal, Valérie Vilgrain, François Durand, and Pierre-Emmanuel Rautou.
• Service d'Hépatologie, DHU Unity, Hôpital Beaujon, Assistance Publique-Hôpitaux de Paris, Clichy, France; INSERM, UMR-1149, Centre de Recherche sur l'Inflammation, Paris-Clichy, France.
• Chest. 2019 Jan 1; 155 (1): 123-136.

BackgroundHepatopulmonary syndrome (HPS) is characterized by an arterial oxygenation defect, defined by an increased alveolar-arterial oxygen gradient, induced by pulmonary vascular dilatations in the context of liver disease. The pathogenesis of HPS is poorly understood. Morphologic changes associated with HPS are unknown. This study aimed at describing imaging and pathology changes associated with HPS.MethodsWe performed a case-control study in candidates for transplant with suspicion of cirrhosis. Each patient with HPS (Pao2 ≤ 70 mm Hg) was matched to three control subjects for age, cause, and liver disease severity. Pretransplant thoracic and abdominal imaging and explanted livers were reviewed.ResultsCT scans and Doppler ultrasounds from 21 patients with HPS were compared with those from 63 control subjects. HPS was associated with a two- to threefold higher prevalence of obstructed intrahepatic portal branches, of slowed or hepatofugal portal blood flow, and of large abdominal portosystemic shunts. Hepatic artery diameter was also larger in patients with HPS. Explanted livers from 19 patients with HPS were compared with those from 57 control subjects. HPS was associated with a fourfold higher prevalence of portal venule thrombosis and a ninefold higher prevalence of extensive vascular proliferation within fibrous septa. Obstruction of centrilobular venules, sinusoidal dilatation, and liver parenchymal extinction were also more common in patients with HPS.ConclusionsHPS is associated with intrahepatic vascular changes and with features suggesting severe portal hypertension. These results raise the hypothesis that intrahepatic vascular changes precipitate the development of HPS, opening new therapeutic perspectives for HPS.Copyright © 2018 American College of Chest Physicians. Published by Elsevier Inc. All rights reserved.

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