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%.
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%.
- The presence of hepatic encephalopathy is associated with a particularly /poor prognosis among patients with hepatopulmonary syndrome.
- 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.
- 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.[1]
References
- ↑ Swanson KL, Wiesner RH, Krowka MJ (2005) Natural history of hepatopulmonary syndrome: Impact of liver transplantation. Hepatology 41 (5):1122-9. DOI:10.1002/hep.20658 PMID: 15828054