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, [#]% of patients with hepatopulmonary syndrome may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].

OR

Common complications of hepatopulmonary syndrome include [complication 1], [complication 2], and [complication 3].

OR

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

• The symptoms of (disease name) usually develop in the first/ second/ third decade of life, and start with symptoms such as ___.
• The symptoms of (disease name) typically develop ___ years after exposure to ___.
• If left untreated, [#]% of patients with hepatopulmonary syndrome may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].

Complications

• Common complications of hepatopulmonary syndrome include:
  • [Complication 1]
  • [Complication 2]
  • [Complication 3]

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.