Hepatopulmonary (hep-uh-toe-POOL-moe-nar-ee) syndrome is an uncommon condition that occurs in people with an existing liver disease that has advanced to the point it affects their lungs. People with hepatopulmonary syndrome have low blood oxygen levels (hypoxemia) caused by widespread dilation (expansion) of the lung blood vessels, both in size and number. The dilated vessels make it hard for the lungs to deliver an adequate supply of oxygen to the body. Liver transplant is the only cure for the syndrome.

  • Experience. Mayo Clinic is one of the nation's leading treatment and research centers for liver diseases, and Mayo Clinic doctors are experienced in treating hepatopulmonary syndrome.
  • Good results. People with hepatopulmonary syndrome who receive a liver transplant at Mayo can expect long-term outcomes similar to other people who receive liver transplants — a greater than 70 percent survival rate after five years.
  • Teamwork. Liver, lung and transplant specialists work closely together to diagnose and treat hepatopulmonary syndrome.

Mayo Clinic in Rochester, Minn., ranks No. 1 for respiratory disorders in the U.S. News & World Report Best Hospitals rankings. Mayo Clinic in Jacksonville, Fla., and Mayo Clinic in Scottsdale, Ariz., are ranked high performing for respiratory disorders by U.S. News & World Report. Mayo Clinic also ranks among the Best Children's Hospitals for respiratory disorders.

Mayo Clinic works with hundreds of insurance companies and is an in-network provider for millions of people. In most cases, Mayo Clinic doesn't require a physician referral. Some insurers require referrals or may have additional requirements for certain medical care. All appointments are prioritized on the basis of medical need.

Specialists in liver diseases, lung diseases and transplantation care for people who have hepatopulmonary syndrome.

For appointments or more information, call the Central Appointment Office at 800-446-2279 (toll-free) 8 a.m. to 5 p.m. Mountain Standard Time, Monday through Friday or complete an online appointment request form.

Specialists in liver diseases, lung diseases and transplantation care for people who have hepatopulmonary syndrome.

For appointments or more information, call the Central Appointment Office at 904-953-0853 8 a.m. to 5 p.m. Eastern time, Monday through Friday or complete an online appointment request form.

Specialists in liver diseases, lung diseases and transplantation care for people who have hepatopulmonary syndrome.

For appointments or more information, call the Central Appointment Office at 507-538-3270 7 a.m. to 6 p.m. Central time, Monday through Friday or complete an online appointment request form.

See information on patient services at the three Mayo Clinic locations, including transportation options and lodging.

Most people who are diagnosed with hepatopulmonary syndrome have been previously diagnosed with an underlying liver condition, usually cirrhosis. People with liver disease who exhibit shortness of breath (dyspnea) or symptoms of low oxygen levels in the blood (hypoxemia) are tested for the marker of hepatopulmonary syndrome — abnormal blood vessels in the lungs. The two main tests used are:

  • Echocardiogram
  • Nuclear medicine lung scan. You are given a radioactive agent that can be visualized in the lungs and the brain to quantify how much of the agent passes through dilated lung blood vessels.

Learn more about echocardiograms.

Replacing your damaged liver with a new liver via a liver transplant is the only effective treatment for hepatopulmonary syndrome in both children and adults. Oxygen therapy to boost low blood oxygen levels can help people feel more comfortable, but it doesn't affect the syndrome itself. Mayo Clinic is testing some experimental drugs to help symptoms of respiratory distress.

Liver transplant offers the best chance for long-term survival for people with hepatopulmonary syndrome. Many people with hepatopulmonary syndrome are referred to Mayo Clinic because of Mayo Clinic's extensive experience treating liver disease and performing liver transplants. More than 5,200 liver transplants have been performed at Mayo Clinic since 1985.

Mayo Clinic doctors looked back at results for all people treated for hepatopulmonary syndrome at Mayo over a 17-year period. They found that 76 percent of those treated with a liver transplant (24 people) were alive five years after their transplant. These results compare favorably to national survival statistics for anyone receiving a liver transplant (a little over 70 percent five-year survival). In contrast, five-year survival for people with hepatopulmonary syndrome who did not receive a liver transplant was 23 percent.

Mayo Clinic is testing inhaled prostacyclin to treat symptoms of respiratory distress. Like oxygen supplements, this treatment is intended to make people more comfortable but does not treat the underlying hepatopulmonary syndrome.

Publications

See a list of publications on hepatopulmonary syndrome by Mayo Clinic doctors on PubMed, a service of the National Library of Medicine.

Nov. 19, 2012