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Coronary artery fistula

Coronary artery fistula is an abnormal connection between one of the coronary arteries and a heart chamber or another blood vessel. The coronary arteries are blood vessels that bring oxygen-rich blood to the heart.

Fistula means abnormal connection.

Causes, incidence, and risk factors

A coronary artery fistula is often congenital, meaning that it is present at birth. It generally occurs when one of the coronary arteries fails to form properly, usually when the baby is developing in the womb. The coronary artery abnormally attaches to one of the chambers of the heart (the atrium or ventricle) or another blood vessel (for example, the pulmonary artery).

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A coronary artery fistula can also develop after birth. It may be caused by:

  • An infection that weakens the wall of the coronary artery and the heart
  • Certain types of heart surgery
  • Injury to the heart

Coronary artery fistula is a rare condition. Infants who are born with it sometimes also have other heart defects.

Symptoms

Infants with this condition usually don’t have any symptoms.

If symptoms do occur, they can include:

  • Chest discomfort or pain
  • Easy fatigue
  • Failure to thrive
  • Fast or irregular heartbeat (palpitations)
  • Shortness of breath (dyspnea)

Signs and tests

This condition is usually not diagnosed until later in life. It is usually diagnosed during tests for other heart diseases. However, the doctor may hear a heart murmur that will lead to the diagnosis with further testing.

Tests to determine the size of the fistula include:

  • An x-ray of the heart using dye to see how and where blood is flowing (angiogram)
  • Passing a thin, flexible tube into the heart to evaluate pressure and flow in the heart and surrounding arteries and veins(cardiac catheterization)
  • Ultrasound exam of the heart (echocardiogram)
  • Using magnets to create images of the heart (MRI)
Coronary angiography

Treatment

A small fistula that is not causing symptoms usually will not need treatment. Some small fistulas will close on their own. Often even if they do not close, they will never cause symptoms or need treatment.

Infants with a larger fistula will need to have surgery to close the abnormal connection. The surgeon closes the site with a patch or stitches.

Another treatment plugs up the opening without surgery, using a special wire (coil) that is inserted into the heart with a long, thin tube called a catheter. After the procedure, the fistula will usually close in children.

Expectations (prognosis)

Children who have surgery usually do well, although a small percentage may need to have surgery again. Most people with this condition have a normal lifespan.

Complications

  • Abnormal heart rhythm (arrhythmia)
  • Heart attack
  • Heart failure
  • Opening (rupture) of the fistula
  • Poor oxygen to the heart

Complications are more common in older patients.

Calling your health care provider

Coronary artery fistula is usually diagnosed during a doctor's exam. Call your health care provider if your infant has symptoms of this condition.

Prevention

There is no known way to prevent this condition.

References

Park MK. Park: Pediatric Cardiology for Practitioners. 5th ed. Philadelphia, Pa: Mosby; 2008.

Updated: 2/7/2012

Neil K. Kaneshiro, MD, MHA, Clinical Assistant Professor of Pediatrics, University of Washington School of Medicine; and Michael A. Chen, MD, PhD, Assistant Professor of Medicine, Division of Cardiology, Harborview Medical Center, University of Washington Medical School, Seattle, Washington. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.


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