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Pulmonary arteriovenous fistula

Arteriovenous malformation - pulmonary

 

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.

Causes

 

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.

 

Symptoms

 

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

  • Bloody sputum
  • Difficulty breathing
  • Difficulty exercising
  • Nosebleeds
  • Shortness of breath with exertion
  • Chest pain  
  • Blue skin ( cyanosis )
  • Clubbing of the fingers

 

Exams and Tests

 

The health care provider will examine you. The exam may show:

  • Abnormal blood vessels ( telangiectasias ) on the skin or mucus membranes
  • Abnormal sound, called a murmur when a stethoscope is placed over the abnormal blood vessel
  • Low oxygen when measure with a pulse oximeter

 Tests that may be done include:

  • Arterial blood gas , with and without oxygen
  • Complete blood count (CBC)
  • Chest x-ray
  • Chest CT scan
  • Echocardiogram  to check the function of the heart
  • Lung function tests
  • Perfusion radionuclide lung scan  to measure breathing and circulation (perfusion) in all areas of the lungs
  • Pulmonary arteriogram  to view the lung arteries

 

Treatment

 

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 .

 

Outlook (Prognosis)

 

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.

 

Possible Complications

 

Complications may include:

  • Bleeding in the lung
  • Stroke due to blood clot that travels from the lungs to the arms, legs, or brain (paradoxical venous embolism)
  • Infection in the brain or heart valve, especially in patients with HHT

 

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.

 

Prevention

 

Because HHT is often genetic, prevention is not usually possible. Genetic counseling may help in some cases.

 

 

Open References

References

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.

 

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                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.

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