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.

Hepatopulmonary syndrome care at Mayo medical institution

Jan. 17, 2020
  1. Kellerman RD, et al. Cirrhosis. In: Conn's Current Therapy 2019. Elsevier; 2019. https://www.clinicalkey.com. Accessed Dec. 9, 2019.
  2. Ferri FF. Hepatopulmonary syndrome. In: Ferri's Clinical Advisor 2020. Elsevier; 2020. https://www.clinicalkey.com. Accessed Dec. 9, 2019.
  3. Hepatopulmonary syndrome. National Organization for Rare Disorders. https://rarediseases.org/rare-diseases/hepatopulmonary-syndrome/. Accessed Dec. 9, 2019.
  4. Hepatopulmonary syndrome. Merck Manual Professional Version. https://www.merckmanuals.com/professional/pulmonary-disorders/pulmonary-hypertension/hepatopulmonary-syndrome#. Accessed Dec. 9, 2019.
  5. Brown AY. Allscripts EPSi. Mayo medical institution. Nov. 14, 2019.

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