Hepatopulmonary Syndrome

What is hepatopulmonary syndrome?

Hepatopulmonary syndrome is a rare complication of chronic liver disease. It affects the capillaries in your lungs. “Hepato-” means “of the liver,” and “-pulmonary” means “of the lungs.” This condition causes low oxygen levels in your blood. This can make it hard for you to breathe.

This disease happens when changes in your liver make capillaries in your lungs widen or dilate. Your capillaries contain red blood cells that carry oxygen throughout your body. When they dilate, your red blood cells can’t absorb oxygen. Trouble breathing can affect your quality of life. Severe hypoxemia may damage your brain and heart.

What are the symptoms of hepatopulmonary syndrome?

Hepatopulmonary syndrome may not cause symptoms early on. Shortness of breath is often the first symptom people notice. Other symptoms that develop later include:

• Cyanosis: Low blood oxygen levels may make your lips, nail beds or skin take on a blueish tint. This looks gray or pale on dark skin tones.
• Clubbed fingers (digital clubbing): Hypoxemia may make the tips of your fingers or toes bulge. Your fingernails may curve downward and look like they aren’t attached to your fingers.
• Orthopnea: In orthopnea, you have shortness of breath that gets worse when you lie down and better when you sit up.
• Platypnoea: This is shortness of breath that gets worse when you sit up and eases when you lie down.

What causes hepatopulmonary syndrome?

Experts don’t understand the connection between liver disease and widening capillaries in your lungs. One theory is that liver disease keeps your liver from filtering toxins (harmful substances) in your blood. When that happens, toxins may build up in your capillaries, so they widen.

What are the complications of hepatopulmonary syndrome?

Hepatopulmonary syndrome may cause hypoxemic hypoxia. This is low oxygen levels in your tissues and organs. Hypoxemic hypoxia can damage your brain and heart and may be life-threatening.

How is hepatopulmonary syndrome diagnosed?

Most people with hepatopulmonary syndrome already have a liver disease diagnosis. But some people may develop hepatopulmonary syndrome symptoms without that diagnosis. In that case, a healthcare provider will order liver function tests. If tests show you have liver disease, a provider will order tests to measure the oxygen content in your blood. Those tests may include:

• Arterial blood gas: This test measures oxygen and carbon dioxide levels in your blood.
• Contrast-enhanced ultrasound (CEUS): An ultrasound produces images of your heart and pulmonary veins. The test tracks blood flow through your heart.
• Pulse oximetry: This test measures your blood oxygen saturation levels.

What is the triad of hepatopulmonary syndrome?

Healthcare providers look for specific medical conditions to diagnose hepatopulmonary syndrome. They may call these conditions the triad of hepatopulmonary syndrome. The diagnostic criteria for hepatopulmonary syndrome are:

• Hypoxemia
• Portal hypertension
• Vasodilation

How is hepatopulmonary syndrome treated?

Treatment typically involves oxygen therapy to boost your oxygen levels. You may have hyperbaric oxygen therapy or receive oxygen through a mask.

Is there a cure for hepatopulmonary syndrome?

The only cure is a liver transplantation to give you a healthy liver. The transplantation will cure the underlying liver disease and eliminate hepatopulmonary syndrome symptoms. However, not everyone is a candidate for a liver transplantation. Those who qualify have to wait for a matching donor liver.

What can I expect if I have hepatopulmonary syndrome?

That depends on your treatment. A successful liver transplantation will give you a healthy liver. But it may take six months to a year for your capillaries to shrink and your blood oxygen levels to return to normal.

How long can you live with hepatopulmonary syndrome?

Research shows about 76% of people who had a successful liver transplantation are alive five years after their transplantation. That survival rate drops two years for people who don’t have a liver transplantation. But many factors affect survival rate. Your situation may be different from that of another person with the disease. If you have survival rate questions, your healthcare team is your best resource for information.

How can I manage hepatopulmonary syndrome?

Hepatopulmonary syndrome is a complication of chronic liver disease. Treatment may ease some hepatopulmonary symptoms. Taking care of your liver may help. Here are some things you can do for your liver:

• Eat well: Focus on healthy proteins, leafy vegetables, nuts and olive oil.
• Maintain a weight that’s right for you: Your body mass index (BMI) is one way to evaluate your weight. But it’s not the only measure of a healthy weight. If you have questions, ask your healthcare provider for advice.
• Quit smoking: Hepatopulmonary syndrome causes shortness of breath. Smoking can make it even more difficult to breathe. It can make chronic liver disease worsen.
• Avoid alcohol: Consuming beverages that contain alcohol puts stress on your liver.
• Take steps to avoid infections: Practice good hygiene like washing your hands often and avoiding people who have colds or flu.

When should I see my healthcare provider?

Hepatopulmonary syndrome leaves you short of breath. You may have oxygen therapy to help ease your symptoms. Contact your provider if it’s becoming more difficult for you to breathe even though you’re receiving oxygen.

What is hepatopulmonary syndrome shunt?

This is a medical term for when capillaries dilate so your red blood cells can’t absorb oxygen. A healthcare provider may call this intrapulmonary shunting.