Diagnosis at Mayo Clinic
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.
Treatment at Mayo Clinic
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.
Explore Mayo Clinic studies testing new treatments, interventions and tests as a means to prevent, detect, treat or manage this disease.
Hepatopulmonary syndrome care at Mayo Clinic
Jan. 10, 2015
- Lange PA, et al. Hepatopulmonary syndrome: Prevalence, causes, clinical manifestations and diagnosis. http://www.uptodate.com/home. Accessed Nov. 22, 2014.
- Lange PA, et al. Hepatopulmonary syndrome: Natural history, treatment and outcomes. http://www.uptodate.com/home. Accessed Nov. 22, 2014.
- Liver transplant. Mayo Clinic. http://www.mayoclinic.org/liver-transplant/statistics.html. Accessed Nov. 22, 2014.
- Krowka MJ, et al. Pulmonary contraindications, indications and MELD exceptions for liver transplantation: A contemporary view and look forward. Journal of Hepatology. 2013;59:367.
- Ferri FF. Ferri's Clinical Advisor 2015: 5 Books in 1. Philadelphia, Pa.: Mosby Elsevier; 2015. https://www.clinicalkey.com. Accessed Nov. 23, 2014.
- Iyer VN, et al. Hepatopulmonary syndrome: Favorable outcomes in the MELD exception era. Hepatology 2013;57:2427.