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The hepatopulmonary syndrome.

Lucas Souto Nacif1, Wellington Andraus1, Rafael Soares Pinheiro1

  • 1Department of Gastroenterology, Liver and Gastrointestinal Transplant Division, University of São Paulo, São Paulo, SP, Brazil.

Arquivos Brasileiros De Cirurgia Digestiva : ABCD = Brazilian Archives of Digestive Surgery
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Hepatopulmonary syndrome, a lung complication of cirrhosis, causes hypoxemia due to intrapulmonary vascular dilatations. Liver transplantation is the only effective treatment for this condition.

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Area of Science:

  • Hepatology
  • Pulmonology
  • Vascular Medicine

Background:

  • Hepatopulmonary syndrome (HPS) is a significant vascular complication in cirrhosis patients with portal hypertension.
  • It manifests as hypoxemia caused by intrapulmonary vascular dilatations, affecting 4-32% of cirrhosis patients.
  • HPS increases mortality in cirrhosis and may necessitate continuous oxygen supplementation.

Purpose of the Study:

  • To provide an updated review of the clinical manifestations, diagnostic approaches, and treatment strategies for hepatopulmonary syndrome.
  • To consolidate current knowledge on managing HPS in patients with liver disease.

Main Methods:

  • A comprehensive literature review was conducted focusing on the management of hepatopulmonary syndrome.
  • Searches were performed on Medline-PubMed using keywords: "hepatopulmonary syndrome", "liver transplantation", and "surgery", completed in September 2013.

Main Results:

  • Hepatopulmonary syndrome is characterized by an elevated alveolar-arterial oxygen gradient (AaPO2) due to intrapulmonary vasodilatation in hepatic dysfunction or portal hypertension.
  • Clinical presentations, diagnostic criteria, classification systems, treatment options, and patient outcomes for HPS are diverse.
  • The severity of HPS is a critical predictor of survival and influences the timing and risks associated with liver transplantation.

Conclusions:

  • The severity of hepatopulmonary syndrome is a key factor in predicting survival and guiding decisions regarding liver transplantation.
  • Liver transplantation remains the sole definitive therapeutic option for resolving hepatopulmonary syndrome.