Hepatopulmonary syndrome natural history, complications and prognosis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Soroush Seifirad, M.D.[2]

Overview

If left untreated, prognosis is generally poor, and the 2.5 year mortalityl rate of patients with hepatopulmonary syndrome is approximately 40% to 60%. With liver transplantation, the 5 year survival rate is 74%, which is comparable to patients who undergo liver transplants who do not suffer from hepatopulmonary syndrome.

Natural History, Complications, and Prognosis

Natural History

• Although development of hepatopulmonary syndrome is not directly related to the Age of the patients, the symptoms of hepatopulmonary syndrome usually develop in the 6th decade of life, and start with symptoms such as dyspnea, and particularly platypnea.
• The symptoms of hepatopulmonary syndrome may develop in acute liver disease nevertheless maajority of patients have years of known liver disorder or cirrhosis, particularly candides of liver transplantation are whom with the highest rate of hepatopulmonary syndrome.
• If left untreated, prognosis is generally poor, and the 2.5 year mortalityl rate of patients with hepatopulmonary syndrome is approximately 40% to 60%. With liver transplantation, the 5 year survival rate is 74%, which is comparable to patients who undergo liver transplants who do not suffer from hepatopulmonary syndrome

Complications

• Common complications of hepatopulmonary syndrome include:
  • Hypoxemia
  • Impaired cognitive function, increase risk of developing hepatic encephalopathy.
  • Increased asterixis
  • Stroke possibly from paradoxical embolism

Prognosis

• Prognosis is generally poor, and the 2.5 year mortalityl rate of patients with hepatopulmonary syndrome is approximately 40% to 60%.
• An increased mortality rate has been observed in patients with HPS.
• Hypoxaemia development and progression is not related to the liver function.
• Unfortunately it has been observed that between 40 to 60 percent of patients with HPS will dye in 2.5 years.
• After adjustment for Model of End-stage Liver Disease (MELD) score and liver transplantation setting, mortality risk has been observed to be more than twice that of non-HPS patients (hazard ratio 2.41, 95% CI 1.31–4.42).
• Nevertheless, almost always mortality is related to portal hypertension and complications of liver disease not HPS and HPS related causes of death.
• But, the degree of hypoxaemia has been associated with a higher mortality.
• HPS decrease quality of life the patients.
• It is reasonable to anticipate that hypoxaemia impair cognition and contribute as a risk factor for hepatic encephalopathy.
• A higher frequency of asterixis has been observed in HPS versus non-HPS cirrhotics.
• The presence of hepatic encephalopathy is associated with a particularly poor prognosis among patients with hepatopulmonary syndrome.
• Coexistence of hepatic encephalopathy could further worsen the prognosis of patients with HPS.
• With liver transplantation, the 5 year survival rate is 74%, which is comparable to patients who undergo liver transplants who do not suffer from hepatopulmonary syndrome.