An atrioventricular canal defect (AVCD) is a birth defect of the heart.
The heart normally has 2 upper chambers (atria) and 2 lower chambers (ventricles). The wall of tissue that separates the right and left sides of the heart is called the septum. A normal heart has 2 heart valves that separate the upper and lower chambers. The valves and septum together are called the atrioventricular canal. Normally the left side of the heart pumps blood to the body, and the right side pumps blood to the lungs.
In AVCD, there are holes in the walls between the chambers of the heart, and the valves that control blood flow may not work well. With AVCD, blood flows where it should not and more blood gets pumped into the lungs. The heart has to work harder and it gets bigger.
AVCD is also called atrioventricular septal defect or endocardial cushion defect.
What is the cause?
The cause of this birth defect is not known. It is most common in babies with Down syndrome.
What are the symptoms?
The symptoms may start at any time from birth to several months after birth. Symptoms may include:
Not eating well
Heart pounding or irregular heartbeats
More colds and lung infections
Gray or bluish color to skin
Many defects cause a whooshing sound, called a murmur, as blood moves through the heart. Healthcare providers can hear the murmur with a stethoscope.
How is it diagnosed?
AVCD can be diagnosed before birth, using screening tests to check for birth defects. If the defect is large enough, tests that may show the problem include:
An ultrasound, which uses sound waves to show pictures of the baby while it is inside the womb
An echocardiogram which uses sound waves (ultrasound) to show pictures of the baby’s heart and how well blood is flowing through it after the baby is born
After birth, your childâ€™s healthcare provider will ask about your child’s symptoms and medical history and examine your child. In addition to an echocardiogram done after birth, other tests may include:
A chest X-ray
An ECG (also called an EKG or electrocardiogram), which measures and records your childâ€™s heartbeat
Sometimes heart catheterization may be needed. A catheter is a very thin tube that is passed through a blood vessel into the heart. The pressure in the chambers of the heart is measured and blood samples can be taken. This helps to tell how big the defect is.
How is it treated?
Your child will need surgery to repair the AVCD. Your baby may need to take medicines until he is strong enough to have surgery.
If your baby is very sick, or the defect canâ€™t be fixed all at once, your baby may have a procedure to reduce blood flow to the lungs. Your childâ€™s healthcare provider can put a band around the lung artery to make it narrower. Then the heart will not have to work as hard, and the lungs will be protected from high blood pressure. When your child is older, the band can be removed and open heart surgery done.
How can I take care of my child?
Your child may need to have regular follow-up visits with a specialist in congenital heart disease.
Follow your childâ€™s healthcare provider’s instructions. Ask your provider:
How and when you will hear your childâ€™s test results
How long it will take for your child to recover
If there are activities your child should avoid and when he can return to normal activities
How to take care of your child at home
If your child should take antibiotics to prevent infection before having dental work or procedures that involve the rectum, bladder, or vagina
What symptoms or problems you should watch for and what to do if your child has them
Make sure you know when your child should come back for a checkup. Keep all appointments for provider visits or tests.
Developed by RelayHealth.
Pediatric Advisor 2015.1 published by RelayHealth. Last modified: 2015-01-29 Last reviewed: 2015-01-29
This content is reviewed periodically and is subject to change as new health information becomes available. The information is intended to inform and educate and is not a replacement for medical evaluation, advice, diagnosis or treatment by a healthcare professional.
Atrioventricular Canal Defect: References
Kliegman, R.M., Stanton, B., St. Geme, J., Schor, N., and Behrman, R. (2011). Acyanotic Congenital Heart Disease. Nelson Textbook of Pediatrics, 19th Ed; Ch. 421, 1561-1571. Philadelphia: Elsevier Saunders.
Bonow, RO, Carabello, BA, Chatterjee, K, et al. 2008 Focused update incorporated into the ACC/AHA 2006 guidelines for the management of patients with valvular heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Revise the 1998 Guidelines for the Management of Patients With Valvular Heart Disease): endorsed by the Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons. Circulation 2008; 118:e523.
Warnes, CA, Williams, RG, Bashore, TM, et al. ACC/AHA 2008 Guidelines for the Management of Adults with Congenital Heart Disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (writing committee to develop guidelines on the management of adults with congenital heart disease). Circulation 2008; 118:e714.