Reference Index - Disease & Conditions

Back to Health Library

Pulmonary arteriovenous fistula

Definition:

Pulmonary arteriovenous fistula is a condition in which an abnormal connection (fistula) develops between an artery and vein in the lungs. As a result, blood passes through the lungs without receiving enough oxygen.



Alternative Names:

Arteriovenous malformation - pulmonary



Causes, incidence, and risk factors:

Pulmonary arteriovenous fistulas are usually the result of a genetic (inherited) disease that causes the blood vessels of the lung to develop abnormally. Fistulas also can be a complication of liver disease.

Patients with Rendu-Osler-Weber disease (ROWD) -- also called hereditary hemorrhagic telangiectasis (HHT) -- often have abnormal blood vessels in many parts of the body.



Symptoms:

Many people have no symptoms. When symptoms occur, they can include:

Other possible symptoms include:



Signs and tests:
  • Abscesses or infections of the heart valves
  • Abnormal blood vessels (telangiectasias ) may be seen on the skin or mucus membranes
  • Murmur heard when a stethoscope is placed over the abnormal blood vessel
  • High red blood cell count

Tests include:



Treatment:

A small number of patients who have no symptoms may not need treatment. For most patients with fistulas, the treatment of choice is to block the fistula during an arteriogram (embolization).

Some patients 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.



Support Groups:



Expectations (prognosis):

The outlook for patients with HHT is not as good as for those without HHT. For patients with HHT, surgery to remove the abnormal vessels usually has a good outcome, and the condition is not likely to return.



Complications:

Major complications after treatment for this condition are unusual. Complications may include:

  • Bleeding in the lung
  • Blood clot that travels from the lungs to the arms, legs, or brain (paradoxical embolism)
  • Infection in the brain or heart valve


Calling your health care provider:

Call your health care provider if you often have nosebleeds or difficulty breathing, especially if you also have a history of HHT.



Prevention:

Because this condition is often genetic, prevention is not usually possible.



References:

Marelli AJ. Congenital heart disease in adults. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier;2007:chap 68.




Review Date: 9/15/2010
Reviewed By: David C. Dugdale, III, MD, Professor of Medicine, Division of General Medicine, Department of Medicine, University of Washington School of Medicine; and Denis Hadijiliadis, MD, Assistant Professor of Medicine, Division of Pulmonary, Allergy and Critical Care, University of Pennsylvania, Philadelphia, PA. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

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.
adam.com


Greater Baltimore Medical Center | 6701 North Charles Street | Baltimore, MD 21204 | (443) 849-2000 | TTY (800) 735-2258
© 2014  GBMC. This website is for informational purposes only and not intended as medical advice or a substitute for a consultation with a professional healthcare provider.