Hepatopulmonary syndrome (patient information)
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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]
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Hepatopulmonary syndrome |
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Hepatopulmonary syndrome On the Web |
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Risk calculators and risk factors for Hepatopulmonary syndrome |
Overview
The hepatopulmonary syndrome (HPS) is an uncommon complication of liver disease in lungs. Occasionally because of the liver dysfunction, blood vessels in the lungs dilate and as a result lungs may lose their ability to effectively exchange oxygen during inspiration. HPS occurs in around 5-32% of patients with cirrhosis. Although HPS is more prevalent in patients with long standing chronic liver disease such as alcoholic liver disease or hepatitis C, it might also present in acute liver disease or in early stages of chronic liver disease. HPS is usually present with severe shortness of breath and decreased blood oxygen levels. Oxygen supplementation is usually needed to manage these symptoms. Liver transplant is the only known approved cure for HPS. Although patients with HPS have a poor prognosis without transplantation, in patients who undergo the liver transplantation, in approximately one year lung functions become normal.
What are the Symptoms of Hepatopulmonary Syndrome?
- HPS is usually present with severe shortness of breath and decreased blood oxygen levels.
- Blue fingers or lips (Cyanosis)
What Causes Hepatopulmonary Syndrome?
- Occasionally because of the liver dysfunction, blood vessels in the lungs dilate and as a result lungs may lose their ability to effectively exchange oxygen during inspiration.
Who is at Highest Risk?
- HPS occurs in around 5-32% of patients with cirrhosis. Although HPS is more prevalent in patients with long standing chronic liver disease such as alcoholic liver disease or hepatitis C, it might also present in acute liver disease or in early stages of chronic liver disease.
Diagnosis
- HPS is diagnosed based on a diagnostic criteria using blood oxygen levels, and sophisticated imaging techniques.
When to Seek Urgent Medical Care?
- Severe shortness of breath
- Decreased consciousness
Treatment Options
- Oxygen supplementation is usually needed to manage these symptoms.
- Liver transplant is the only known approved cure for HPS
Where to find Medical Care for Hepatopulmonary Syndrome?
Medical care for Hepatopulmonary Syndrome can be found here.
Prevention
- There is no known prevention for HPS. Nevertheless, having a healthy life stile, avoiding alcohol, and fatty foods, exercising daily, and practicing safe sex will decrease development of liver diseases and also HPS as a result.
What to Expect (Outlook/Prognosis)?
- Although patients with HPS have a poor prognosis without transplantation, in patients who undergo the liver transplantation, in approximately one year lung functions become normal.
Possible Complications
- Here is a list of possible complications after liver transplantation in HPS patients:
- Severe post-transplant hypoxemia and subsequent prolonged mechanical ventilation
- Post-operative infections
- Anastomotic bile duct leaks due to delayed wound healing because of hypoxemia
- Post-operative portal vein and hepatic artery clots
- Post-transplant HPS recurrence because of graft dysfunction
- Progressive pulmonary hypertension, another known pulmonary complication of liver disease