What is the definition of 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.

What are the alternative names for Pulmonary Arteriovenous Fistula?

Arteriovenous malformation - pulmonary

What are the causes for Pulmonary Arteriovenous Fistula?

Pulmonary arteriovenous fistulas are usually the result of abnormal development of the blood vessels of the lung. Most occur in people with 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 these causes are much less common.

What are the symptoms for Pulmonary Arteriovenous Fistula?

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

What are the current treatments for Pulmonary Arteriovenous Fistula?

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.

What is the outlook (prognosis) for Pulmonary Arteriovenous Fistula?

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.

For people with liver disease as a cause, prognosis depends on liver disease.

What are the possible complications for Pulmonary Arteriovenous Fistula?

Complications may include:

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

When should I contact a medical professional for Pulmonary Arteriovenous Fistula?

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

How do I prevent Pulmonary Arteriovenous Fistula?

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

REFERENCES

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.

Stowell J, Gilman MD, Walker CM. Congenital thoracic malformations. In: Shepard JO, ed. Thoracic Imaging: The Requisites. 3rd ed. Philadelphia, PA: Elsevier; 2019:chap 8.

Webb GD, Smallhorn JF, Therrien J, Redington AN. Congenital heart disease in the adult and pediatric patient. In: Zipes DP, Libby P, Bonow RO, Mann DL, Tomaselli GF, Braunwald E, eds. Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine. 11th ed. Philadelphia, PA: Elsevier; 2019:chap 75.

  • Condition: Hepatocellular Carcinoma
  • Journal: Technology in cancer research & treatment
  • Treatment Used: Transarterial Chemoembolization
  • Number of Patients: 816
  • Published —
The study researched the use of transarterial chemoembolization for hepatocellular carcinoma.
  • Condition: Pulmonary Arteriovenous Malformation (PAVM) and Multifocal Benign Metastasizing Leiomyoma (BML) of Lungs
  • Journal: Clinical imaging
  • Treatment Used: Endovascular Treatment
  • Number of Patients: 1
  • Published —
This case report describes a 50-year-old female diagnosed with pulmonary arteriovenous malformation (PAVM) and multifocal benign metastasizing leiomyoma (fibroids; BML) of lungs who underwent endovascular treatment.
Clinical Trial
  • Status: Not yet recruiting
  • Phase: N/A
  • Intervention Type: Device
  • Participants: 50
  • Start Date: March 22, 2021
The Effectiveness of FRAME FR for ArterioVenous Fistula Repair in High-Flow Reduction, A Prospective, Monocenter Controlled Trial
Clinical Trial
  • Status: Recruiting
  • Phase: N/A
  • Intervention Type: Radiation
  • Participants: 50
  • Start Date: November 28, 2019
Diagnostic Value of Ultra-low Dose Thoracic Scanner for the Pulmonary Arteriovenous Malformation Detection in HHT Patient