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 results in low blood oxygen levels (hypoxemia) caused by blood vessels expanding (dilating) in size and number, which makes it hard for the lungs to deliver adequate amounts 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 85 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 Scottsdale, Ariz. is ranked among the Best Hospitals for respiratory disorders by U.S. News & World Report. Mayo Clinic in Jacksonville, Fla., is 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.

At Mayo Clinic, we assemble a team of specialists who take the time to listen and thoroughly understand your health issues and concerns. We tailor the care you receive to your personal health care needs. You can trust our specialists to collaborate and offer you the best possible outcomes, safety and service.

Mayo Clinic is a not-for-profit medical institution that reinvests all earnings into improving medical practice, research and education. We're constantly involved in innovation and medical research, finding solutions to improve your care and quality of life. Your doctor or someone on your medical team is likely involved in research related to your condition.

Our patients tell us that the quality of their interactions, our attention to detail and the efficiency of their visits mean health care — and trusted answers — like they've never experienced.

Why Choose Mayo Clinic

What Sets Mayo Clinic Apart

Most people who are diagnosed with hepatopulmonary syndrome have already been diagnosed with a liver condition, usually cirrhosis. People with liver disease who have 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:

  • Contrast-enhanced echocardiogram. You're given a solution (agitated saline) through an arm vein that creates harmless, tiny bubbles. The bubbles can be seen in all chambers of the heart only if they pass through dilated lung blood vessels.
  • 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 in the hope of improving oxygen levels.

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 who had liver transplants as a result of hepatopulmonary syndrome at Mayo over a 24-year period. Of the 49 patients receiving transplants, 88 percent were alive after five years. 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 was only 23 percent for people with hepatopulmonary syndrome who did not receive a liver transplant.

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 at Mayo Clinic.

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 at Mayo Clinic.

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 at Mayo Clinic.

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.

Mayo Clinic is part of a multicenter research group, directed out of the University of Pennsylvania, studying the pulmonary vascular complications of liver disease. New treatment options for hepatopulmonary syndrome are being evaluated.

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

Jan. 10, 2015