Coronary artery fistula

Definition

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).

In rare cases, the abnormal coronary artery (usually the right coronary artery) connects with the large artery leaving the heart (aorta), or the vessel that carries blood from the lungs back to the heart (pulmonary vein). The bad connection interrupts normal blood flow to the heart.

However, a coronary artery fistula can 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

Some infants are born with more than one fistula.

Coronary artery fistula is a rare condition. Infants who are born with it sometimes also have other heart defects. Children who have pulmonary atresia and an intact ventricular septum can have coronary artery fistula.

Symptoms

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

If symptoms do occur, they can include:

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

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 in the infant.

Tests to determine the size of the fistula include:

• An x-ray of the heart using dye to see how well blood is flowing (angiogram)
• Passing a thin, flexible tube into the heart to examine the arteries (cardiac catheterization)
• Ultrasound exam of the heart (echocardiogram)
• Using magnets to create images of the heart (MRI)

Treatment

A small fistula that is not causing symptoms may not need treatment. Some small fistulas will close on their own.

Infants with a larger fistula will need to have surgery to close the abnormal connection as soon as possible. The surgeon closes the site with a patch or stitches (suture).

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 often 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.