Pulmonary arteriovenous fistula

Causes

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:

• Bloody sputum
• Difficulty breathing
• Difficulty exercising
• Nosebleeds
• Shortness of breath with exertion

Other possible symptoms include:

• Blue skin (cyanosis)
• Clubbing of the fingers

Exams 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:

• Arterial blood gas
• Complete blood count (CBC)
• Chest x-ray
• Chest CT scan
• Echocardiogram with bubble study
• Liver function tests
• Perfusion radionuclide lung scan
• Pulmonary arteriogram

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.

Outlook (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.

Possible 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

When to Contact a Medical Professional

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.

Alternative Names

Arteriovenous malformation - pulmonary

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.

Update Date: 9/15/2010

Updated 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.