Perioperative Management of Patients with Hepatopulmonary Syndrome
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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.
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.
KeywordsHepatopulmonary syndrome Liver transplant ECMO Orthodeoxia
arterial blood gas
contrast-enhanced transthoracic echocardiography
computed tomographic angiogram
extracorporeal membrane oxygenation
endothelial nitric oxide synthase
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.
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