Pulmonary arteriovenous fistula
Pulmonary arteriovenous fistula is an abnormal connection between an artery and vein in the lungs. As a result, blood passes through the lungs without receiving enough oxygen.
Arteriovenous malformation - pulmonary
Pulmonary arteriovenous fistulas are usually the result of abnormal development of the blood vessels of the lung. Most occur in people with Rendu-Osler-Weber disease (ROWD), now commonly called hereditary hemorrhagic telangiectasia (HHT). These people often have abnormal blood vessels in many other parts of the body.
Fistulas also can be a complication of liver disease or lung injury, although this is much less common.
Many people have no symptoms. When symptoms occur, they can include:
Exams and Tests
The health care provider will examine you. The exam may show:
Tests that may be done include:
A small number of people who have no symptoms may not need treatment. For most people with fistulas, the treatment of choice is to block the fistula during an arteriogram (embolization).
Some people may need surgery to remove the abnormal vessels and nearby lung tissue.
When arteriovenous fistulas are caused by liver disease, the treatment is a liver transplant.
The outlook for people with HHT is not as good as for those without HHT. For people without HHT, surgery to remove the abnormal vessels usually has a good outcome, and the condition is not likely to return.
Complications may include:
When to Contact a Medical Professional
Call your provider if you often have nosebleeds or difficulty breathing, especially if you also have a personal or family history of HHT.
Because HHT is often genetic, prevention is not usually possible. Genetic counseling may help in some cases.
Marelli AJ. Congenital heart disease in adults. In: Goldman L, Schafer AI, eds. Goldman-Cecil Medicine. 25th ed. Philadelphia, PA: Elsevier Saunders; 2016:chap 69.
Shovlin CL, Jackson JE. Pulmonary vascular abnormalities. In: Broaddus VC, Mason RJ, Ernst JD, et al, eds. Murray and Nadel's Textbook of Respiratory Medicine. 6th ed. Philadelphia, PA: Elsevier Saunders; 2016:chap 61.
Review Date: 8/21/2016
Reviewed By: Denis Hadjiliadis, MD, MHS, Paul F. Harron, Jr. Associate Professor of Medicine, Pulmonary, Allergy, and Critical Care, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.
The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. © 1997- A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.