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Hepatopulmonary Syndrome

What is Hepatopulmonary syndrome?

This is an uncommon medical condition that involves both your lungs and liver. It normally affects the lungs of someone who has advanced liver disease. When someone has advanced liver disease it is thought by researchers that their liver starts to produce an increased amount of vasodilators, which are chemical compounds that are designed to widen your blood vessels. At the same time, in your lungs, these widened blood vessels inhibits gas exchanges, which is a process that allows your body to exchange waste gases for the gases it needs to function and is part of the respiratory process. This in turn limits the amount of oxygen in your blood and can lead to shortness of breath as they struggle for more breath.

Hepatopulmonary syndrome occurs in fifteen to twenty percent of people with cirrhosis of the liver. In adults with a liver disorder there is a five to twenty-nine percent possibility that they will develop this syndrome. In children who have a liver disorder this possibility is point five to twenty percent. If they have acquired childhood cirrhosis of the liver the possibility is two to four percent.


There are two types of hepatopulmonary syndrome, which include:

  • Type one – with this type it involves the right to left shunting of your lung and is the most common form of this syndrome. It involves dilation of the capillaries, which are the smallest blood vessels in your body, during the exchange of oxygen. When this happens there is less oxygen contained in your alveoli, which are tiny air sacs in your lungs, where the gas exchange is the primary role. Here the ventilation process is altered.
  • Type two – this type accounts for approximately ten percent of all cases of hepatopulmonary syndrome. In this type the oxygen exchange is still not normally attained because the arteriovenous shunts, which is a connection between a vein and artery, in your circulatory system has not attained their purpose and causes ineffective oxygen exchanged.

Symptoms and Characterizations

Hepatopulmonary syndrome is characterized by difficult breathing and decreased oxygen levels in your blood. Other symptoms can include:

  • Having periods of anxiety
  • Having cyanosis, which is a bluish discoloration of their skin.
  • Fingertips enlarged referred to as digital clubbing
  • Having a spider-like appearance of the blood vessels that are visible on your skin, referred to as spider naevi
  • Increased levels of Nitric oxide levels
  • Increased heart rate

If a person does not already have an underlying liver disease there are no symptoms.


It is caused by a combination of decreased arterial oxygen concentration, advanced liver disease, and dilation of the blood vessels of your lungs.


If you have liver disease and see your physician with symptoms of shortness of breath and low blood oxygen levels they may suspect that you have hepatopulmonary syndrome so to find out if you do they will recommend that you have an echocardiogram of your heart. This is a test that will look for signs of dilated blood vessels and check on the function of your heart. Another diagnostic test that can check the blood flow in your lungs is called a nuclear medicine scan. You will be injected with a radioactive contrast agent to highlight the blood flow. Both of these diagnostic tests can help to confirm if you have hepatopulmonary syndrome.


The physician will also check your level of oxygen referred to as levels of partial oxygen (PaO2). If this level is less than seventy millimeters Hg it indicates that you have hypoxemia, which is having an abnormally low level of oxygen in your blood. When a person has hepatopulmonary syndrome it is essential that his PaO2 level be checked with they are in an upright position when their PaO2 level decreases more than three millimeters Hg. This decrease is an indication of orthodeoxia, which is a decrease in your arterial blood oxygen.


This is a medical condition that can be managed but the only effective treatment is to have a liver transplant. The treatment team for someone who has hepatopulmonary syndrome includes a physician who specializes in liver care referred to as a hepatologist, along with other surgeons and pulmonary specialists. A pulmonary specialist is one who deals with the care of your lungs.

When it has been confirmed that you have hepatopulmonary syndrome the immediate treatment needed is supplemental oxygen, which will help you get more oxygen into your blood. Sometimes standing or sitting upright can make it the situation worse so your physician may suggest that you change certain body positions to address the symptoms. Many times when a person has liver disease they are already on a transplant list but developing hepatopulmonary syndrome may push them up higher on the transplant list.

If there are other complications present you may need to be hospitalized so you can receive a higher level of care. You may also be able to stay at home while waiting for a liver transplant but using supplemental oxygen. While you are preparing for your lung transplant you will learn about the anti-rejection medications that you will need to take to keep your new liver alive and healthy.


The prognosis varies with a lot depending on the person’s level of health. In general, when a person has hepatopulmonary syndrome the prognosis is poor. When diagnosed with this syndrome it is very important that you have check-ups every two to three years.

Life expectancy

When a person has hepatopulmonary syndrome and undergoes a liver transplant they have a seventy-four percent chance of reaching a survival of five years. On average there is a two to five year survival rate if you have a liver transplant but is much lower if you do not have the transplant.

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