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Transplantation. 2016 Jul;100(7):1440-52. doi: 10.1097/TP.0000000000001229.

International Liver Transplant Society Practice Guidelines: Diagnosis and Management of Hepatopulmonary Syndrome and Portopulmonary Hypertension.

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1 Pulmonary and Gastroenterology/Hepatology Division, Mayo Clinic, Rochester, MN. 2 Gastroenterology and Hepatology, University of Texas, Houston, TX. 3 Pulmonary Division, University of Pennsylvania, Philadelphia, PA. 4 University Medical Center Hamburg-Eppendorf, Hamburg, Germany. 5 Transplant Surgery, Mayo Clinic, Rochester, MN. 6 Department of Anesthesia, Baylor University Medical Center, Dallas, TX. 7 Pulmonary Division, Hôpital Bicêtre, University of Paris-Sud, Le Kremlin-Bicêtre, France. 8 Pediatric Gastroenterology, Hepatology and Nutrition Section, University of Colorado School of Medicine, Anschutz Medical Campus, Aurora, CO.


Two distinct pulmonary vascular disorders, hepatopulmonary syndrome (HPS) and portopulmonary hypertension (POPH) may occur as a consequence of hepatic parenchymal or vascular abnormalities. HPS and POPH have major clinical implications for liver transplantation. A European Respiratory Society Task Force on Pulmonary-Hepatic Disorders convened in 2002 to standardize the diagnosis and guide management of these disorders. These International Liver Transplant Society diagnostic and management guidelines are based on that task force consensus and should continue to evolve as clinical experience dictates. Based on a review of over 1000 published HPS and POPH articles identified via a MEDLINE search (1985-2015), clinical guidelines were based on, selected single care reports, small series, registries, databases, and expert opinion. The paucity of randomized, controlled trials in either of these disorders was noted. Guidelines are presented in 5 parts; I. Definitions/Diagnostic criteria; II. Hepatopulmonary syndrome; III. Portopulmonary hypertension; IV. Implications for liver transplantation; and V. Suggestions for future clinical research.

[Indexed for MEDLINE]

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