Hereditary Hemorrhagic Telangiectasia

Hepatopulmonary syndrome is a rare complication of liver disease that causes low oxygen levels in your blood (hypoxemia). “Hepato” means “of the liver,” and “pulmonary” means “of the lungs.” Your liver and lungs are connected by blood vessels, which become dilated (widened) in advanced liver disease. The dilated blood vessels form “shunts,” irregular blood flow patterns that bypass the oxygenation process in your lungs. Dilated blood vessels (vasodilation) in your lungs and shunting lead to hypoxemia.

How does this condition affect me?

Hepatopulmonary syndrome causes shortness of breath, which worsens over time. It may improve when lying flat and worsen in an upright position. It can become so severe that it’s present even at rest. This gradual worsening process is also known as chronic respiratory failure. Everything doesn’t fail at once, but eventually, your organs and tissues become seriously oxygen deprived (hypoxia). This is a dangerous condition that can do permanent damage to your brain and heart. If it goes untreated, it can be fatal.

Who does hepatopulmonary syndrome affect?

Hepatopulmonary syndrome occurs in an estimated 25% of people with chronic liver disease. It can also occur with acute liver failure, but this is less common. Typically, cirrhosis of the liver leads to portal hypertension, which in some cases leads to hepatopulmonary syndrome. Less commonly, portal hypertension by other causes, such as blood clots, can also lead to hepatopulmonary syndrome.