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%.[1]
- 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).[2]
- Nevertheless, almost always mortality is related to portal hypertension and complications of liver disease not HPS and HPS related causes of death.[3]
- 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.[4]
References
- ↑ Porres-Aguilar M, Altamirano JT, Torre-Delgadillo A, Charlton MR, Duarte-Rojo A (2012) Portopulmonary hypertension and hepatopulmonary syndrome: a clinician-oriented overview. Eur Respir Rev 21 (125):223-33. DOI:10.1183/09059180.00007211 PMID: 22941887
- ↑ Fallon MB, Krowka MJ, Brown RS, Trotter JF, Zacks S, Roberts KE et al. (2008) Impact of hepatopulmonary syndrome on quality of life and survival in liver transplant candidates. Gastroenterology 135 (4):1168-75. DOI:10.1053/j.gastro.2008.06.038 PMID: 18644373
- ↑ Schenk P, Schöniger-Hekele M, Fuhrmann V, Madl C, Silberhumer G, Müller C (2003) Prognostic significance of the hepatopulmonary syndrome in patients with cirrhosis. Gastroenterology 125 (4):1042-52. PMID: 14517788
- ↑ 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