Current Transplantation Reports

, Volume 5, Issue 4, pp 282–288 | Cite as

Perioperative Management of Patients with Hepatopulmonary Syndrome

  • Andrew I. Gagnon
  • Andre De Wolf
  • Jana Hudcova
  • David A. AxelrodEmail author
Anesthesia and Critical Care in Transplantation (D Axelrod and M Kaufman, Section Editors)
Part of the following topical collections:
  1. Topical Collection on Anesthesia and Critical Care in Transplantation


Purpose of Review

Hepatopulmonary syndrome (HPS) is a syndrome of hypoxemia in patients with advanced liver disease, which results in significant mortality in the absence of liver transplantation. Management of HPS is complex both prior to and following liver transplantation as illustrated by a case presentation. This review considers the pathophysiology, diagnosis, assessment of severity, and treatment of HPS with a particular emphasis on perioperative management.

Recent Findings

Screening for HPS should be performed in all liver transplant candidates with room air saturation less than 97%. Diagnosis of HPS has been standardized with the use of agitated saline-enhanced echocardiography. Treatment modalities for profound hypoxemia include patient positioning, transtracheal oxygenation, nitric oxide therapy, intravenous methylene blue, and extracorporeal membrane oxygenation. Fortunately, patients with HPS who undergo successful liver transplantation will often have complete resolution of the symptoms related to HPS, although this may require weeks to months.


Despite technical challenges, liver transplantation for HPS can be safely performed provided patients are identified and appropriately managed. Physicians caring for these patients should be prepared to use all available interventions including extracorporeal oxygenation to allow time for the HPS to resolve and oxygenation to improve. Further investigation is needed to identify effective medical treatments to mitigate HPS-related hypoxemia.


Hepatopulmonary syndrome Liver transplant ECMO Orthodeoxia 



arterial blood gas


arterial-venous malformation


contrast-enhanced transthoracic echocardiography


computed tomographic angiogram


extracorporeal membrane oxygenation


endothelial nitric oxide synthase


hepatopulmonary syndrome


liver transplantation


nitric oxide


room air


transjugular intrahepatic portosystemic shunt


mixed venous oxygenation saturation


peripheral oxygen saturation


vascular endothelial growth factor


Compliance with Ethical Standards

Conflict of Interest

The authors declare that they have no conflict of interest.

Human and Animal Rights and Informed Consent

This article does not contain any studies with human or animal subjects performed by any of the authors.


Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance

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Copyright information

© Springer Nature Switzerland AG 2018

Authors and Affiliations

  • Andrew I. Gagnon
    • 1
  • Andre De Wolf
    • 2
  • Jana Hudcova
    • 1
  • David A. Axelrod
    • 1
    Email author
  1. 1.Division of Transplantation and Hepatobiliary SurgeryLahey Hospital and Medical Center BurlingtonBurlingtonUSA
  2. 2.Northwestern Memorial HospitalChicagoUSA

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