Hepatopulmonary Syndrome: Symptoms, Causes & Treatment Guide

6 days ago

6 min read

Updated: 2 days ago

Medically reviewed by Dr. Baraa Alnahhal, MD · Last reviewed: September 2026

TL;DR

Hepatopulmonary syndrome (HPS) is a rare condition in which blood vessels in and around the lungs widen (dilate) and multiply in people who have advanced liver disease. These changes make it hard for red blood cells to absorb oxygen, leading to low blood oxygen (hypoxemia). Often there are no symptoms at all. When symptoms do appear, they can include breathlessness that worsens when sitting or standing and improves when lying down, rounded fingertips (clubbing), spider-like broken blood vessels under the skin (spider angioma), and a bluish or gray tinge to the lips and skin (cyanosis). Doctors diagnose HPS with three main tests — pulse oximetry, arterial blood gas, and chest imaging. Supplemental oxygen therapy is the main treatment for breathlessness, and a liver transplant is the only cure.

Quick Answer

What it is: A condition in which lung blood vessels dilate and multiply, lowering blood oxygen, occurring only in people with advanced liver disease.
Why it happens: Not fully understood; the link between liver disease and the lung changes is not yet known.
Key symptom: Shortness of breath that is worse when sitting or standing and better when lying down.
Other signs: Finger clubbing, spider angioma, cyanosis (bluish or gray lips and skin).
Diagnosis: Pulse oximetry, arterial blood gas, and chest imaging (X-ray, CT, or saline contrast echocardiogram).
Main treatment: Supplemental oxygen therapy for low blood oxygen.
Only cure: A liver transplant.

What Is Hepatopulmonary Syndrome?

Hepatopulmonary (hep-uh-toe-POOL-moe-nar-e) syndrome is caused by blood vessels in the lungs expanding — also called dilating — and increasing in number. This condition affects the lungs of people who have advanced liver disease.

These changes in the lungs make it hard for red blood cells to take in oxygen. The lungs then can't send enough oxygen to the body. This leads to low oxygen levels in the blood, also called hypoxemia (hye-pok-SEE-mee-uh).

Key point: How liver disease is linked to the lung condition isn't yet known. It's also not known why some people with liver disease develop hepatopulmonary syndrome while others do not.

Infographic showing how advanced liver disease leads to dilated, multiplying lung blood vessels, red blood cells absorbing less oxygen, and low blood oxygen (hypoxemia); notes that there are often no symptoms and that liver transplant is the only cure. — What is hepatopulmonary syndrome — anatomy overview

What Are the Symptoms?

Often, there are no symptoms of hepatopulmonary syndrome. If there are symptoms, they can include the four listed below.

Symptom	What It Looks or Feels Like
Shortness of breath	Worse when sitting or standing; gets better when lying down
Clubbing of the fingers	Fingertips spread out and become rounder than usual
Spider angioma	Broken blood vessels under the skin
Cyanosis	Bluish tinge of the lips and skin in white people; in Black and brown people, the lips or tongue might look light gray

The breathlessness pattern is distinctive. Unlike many lung conditions that worsen when lying flat, hepatopulmonary syndrome often feels worse when upright and better when lying down. If you have liver disease and notice this pattern, it's worth mentioning to your care team.

Infographic illustrating the four signs of hepatopulmonary syndrome: shortness of breath that is worse sitting or standing and better lying down, clubbing of the fingers, spider angioma, and cyanosis, with a note that the condition only affects people with advanced liver disease. — The four signs of hepatopulmonary syndrome

What Causes Hepatopulmonary Syndrome?

Hepatopulmonary syndrome is caused when blood vessels in and around the lungs widen — also called dilate. This affects the amount of oxygen that moves from the lungs into the bloodstream.

Question	What Is Known
What triggers the blood vessel changes?	Not clear
Why do only some people with liver disease develop it?	Not known
Can it be prevented?	No known prevention (cause is not yet understood)

The condition is classified as a rare disease by rare-disease medical organizations. Because the underlying mechanism is still being studied, there is currently no way to prevent it — the focus is on early detection in people who already have advanced liver disease.

Who should be alert: Anyone with advanced liver disease (such as cirrhosis) should report new or unusual breathlessness, fingertip changes, skin color changes, or spider-like skin markings to their doctor.

How Is It Diagnosed?

These three tests can help find out whether you have hepatopulmonary syndrome:

Test	How It Works	What It Shows
Pulse oximetry	A sensor attached to the finger or ear uses light to measure how much oxygen is in the blood	Blood oxygen level
Arterial blood gas	Blood is taken from an artery	Oxygen level, carbon dioxide level, and pH balance
Chest imaging	X-rays, CT scanning, or an echocardiogram with a salt solution given through a vein (a saline contrast study)	Helps rule out other heart or lung conditions

Doctors typically start with the simplest test — pulse oximetry — and move to blood gas analysis and imaging when the picture needs to be clearer. Imaging is especially useful because many heart and lung conditions can cause similar symptoms.

Flowchart showing the five-step pathway for hepatopulmonary syndrome from pulse oximetry through arterial blood gas, chest imaging, treatment for breathlessness with supplemental oxygen, and liver transplant as the only cure, alongside common questions to ask a doctor. — From evaluation to treatment: the hepatopulmonary syndrome pathway

How Is Hepatopulmonary Syndrome Treated?

Treatment for hepatopulmonary syndrome focuses on two goals: restoring oxygen levels and addressing the underlying liver disease.

Treatment	Purpose	Key Fact
Supplemental oxygen therapy	Main treatment for shortness of breath caused by low blood oxygen	Oxygen is delivered as a supplement to raise blood oxygen levels
Liver transplant	The only cure for hepatopulmonary syndrome	Addresses the liver disease driving the lung blood vessel changes

Being given oxygen, called supplemental oxygen therapy, is the main treatment for shortness of breath caused by low oxygen levels in the blood. A liver transplant is the only cure for hepatopulmonary syndrome.

Managing Day to Day

If you have hepatopulmonary syndrome, your care team may recommend carrying supplemental oxygen during activities that lower your oxygen levels, such as exertion or air travel. Because the condition is tied to advanced liver disease, ongoing specialist care for both the liver and the lungs is essential.

Conclusion

Hepatopulmonary syndrome is a rare but important complication of advanced liver disease. It often causes no symptoms, which makes awareness of the warning signs — unusual breathlessness that improves when lying down, rounded fingertips, spider-like skin markings, and bluish or gray lips — especially valuable. With pulse oximetry, blood gas testing, and chest imaging, doctors can identify the condition and rule out other causes. Supplemental oxygen therapy eases the breathlessness, and a liver transplant remains the only cure.

If you or a loved one has advanced liver disease and notices any of these signs, talk to your healthcare provider. Bring this guide to your appointment, note when symptoms occur and what makes them better, and ask whether oxygen testing is appropriate for you.

References

Frequently Asked Questions

What is hepatopulmonary syndrome?

Hepatopulmonary syndrome is a condition caused by blood vessels in the lungs widening and multiplying, which makes it hard for red blood cells to take in oxygen. It occurs in people who have advanced liver disease and leads to low blood oxygen (hypoxemia).

What are the first signs of hepatopulmonary syndrome?

Often there are no symptoms at all. When they appear, the first signs can include shortness of breath that's worse when sitting or standing and better when lying down, rounded fingertips (clubbing), spider-like broken blood vessels under the skin, and a bluish or gray tinge to the lips and skin.

Can hepatopulmonary syndrome be cured?

Yes — a liver transplant is the only cure for hepatopulmonary syndrome. Until then, supplemental oxygen therapy is the main treatment for shortness of breath caused by low blood oxygen.

Is hepatopulmonary syndrome the same as liver disease?

No. It is a lung condition that develops in some people who already have advanced liver disease. How liver disease leads to the lung changes is not yet known.

How is hepatopulmonary syndrome diagnosed?

Doctors use three tests: pulse oximetry (a light sensor on the finger or ear), an arterial blood gas test (measuring oxygen, carbon dioxide, and pH from artery blood), and chest imaging such as X-rays, CT scans, or a saline contrast echocardiogram.

Why does breathlessness improve when lying down?

In hepatopulmonary syndrome, the blood vessel changes in the lungs make oxygen uptake harder when upright. Lying down tends to improve breathing — a pattern that differs from many other lung conditions and can be an important clue.

Can hepatopulmonary syndrome be prevented?

Not at this time. The cause of the lung blood vessel changes is not clear, and it is not known why some people with liver disease develop the condition while others do not.

Who is at risk of hepatopulmonary syndrome?

The condition only affects people with advanced liver disease. Since the mechanism isn't fully understood, anyone with advanced liver disease who develops new breathing problems should be evaluated.

Rinnit.com provides general health information for educational purposes. This content is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the guidance of a qualified healthcare provider with any questions about your health.