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Definition Return to top
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 Return to top
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:
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 Return to top
Infants with this condition usually don’t have any symptoms.
If symptoms do occur, they can include:
Exams and Tests Return to top
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:
Treatment Return to top
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.
Outlook (Prognosis) Return to top
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.
Possible Complications Return to top
Complications are more common in older patients.
When to Contact a Medical Professional Return to top
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 Return to top
There is no known way to prevent this condition.
References Return to top
Park MK. Park: Pediatric Cardiology for Practitioners. 5th ed. Philadelphia, Pa: Mosby; 2008.
Update Date: 2/29/2008 Updated by: Mark A Fogel, MD, FACC, FAAP, Associate Professor of Pediatrics and Radiology and Director of Cardiac MR, The Children's Hospital of Philadelphia.
