Hepatopulmonary syndrome

Date Updated: 01/06/2022

Overview

Hepatopulmonary (hep-uh-toe-POOL-moe-nar-e) syndrome is an uncommon condition that affects the lungs of people with advanced liver disease. Hepatopulmonary syndrome is caused by blood vessels in the lungs expanding (dilating) and increasing in number, making it hard for red blood cells to properly absorb oxygen. This leaves the lungs unable to deliver adequate amounts of oxygen to the body, which leads to low oxygen levels (hypoxemia).

Exactly how the liver disease is linked to the lung condition isn't yet known. A liver transplant is the only cure for hepatopulmonary syndrome.

Symptoms

Most people with hepatopulmonary syndrome have no symptoms. If symptoms occur, they may include:

Shortness of breath, especially when sitting or standing
Clubbing of the fingers, in which the fingertips spread out and become rounder than normal
Broken blood vessels under the skin (spider angioma)
Bluish tinge of the lips and skin (cyanosis)

Causes

Hepatopulmonary syndrome is caused when blood vessels in and around the lungs widen (dilate), which affects the amount of oxygen that moves from the lungs into the bloodstream. What causes this abnormality remains unclear, and it's unknown why some people with liver disease develop hepatopulmonary syndrome while others do not.

Diagnosis

These tests can help determine if you have hepatopulmonary syndrome:

Pulse oximetry. In pulse oximetry, a sensor attached to your finger or ear uses light to determine how much oxygen is in your blood.
Chest imaging. X-rays, computerized tomography (CT) scanning or echocardiogram imaging can help rule out other heart or lungs conditions.

Treatment

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.