CausesBy Mayo Clinic staff
Atrioventricular canal defect occurs during fetal growth when your baby's heart is developing. While some factors, such as Down syndrome, may increase the risk of atrioventricular canal defect, in most cases the cause is unknown.
The normal-functioning heart
Your heart is divided into four chambers, two on the right and two on the left. In performing its basic job — pumping blood throughout your body — your heart uses its left and right sides for different tasks. The right side moves blood into vessels that lead to your lungs. In your lungs, oxygen enriches your blood, which circulates to your heart's left side. The left side of your heart pumps blood into a large vessel called the aorta, which circulates blood to the rest of your body. Valves control the flow of blood into and out of the chambers of your heart. These valves open to allow blood to move to the next chamber or to one of the arteries, and they close to keep blood from flowing backward.
What happens in atrioventricular canal defect
In partial atrioventricular canal defect, a hole exists in the wall (septum) that separates the upper chambers (atria) of the heart. Also, the mitral valve between the upper and lower left chambers often has a defect called a cleft that causes it to leak (mitral valve regurgitation).
In complete atrioventricular canal defect, there's a large hole in the center of the heart where the walls between the upper chambers (atria) and lower chambers (ventricles) meet. Instead of two separate valves — one on the right (tricuspid) and one on the left (mitral) — one large common valve exists between the upper and lower chambers. And, this valve may not close tightly.
Oxygen-rich and oxygen-poor blood mix through the hole in the septum, and the abnormal valves leak blood into the heart's lower chambers (ventricles). These problems make the heart work harder, causing it to enlarge.
- Atrioventricular septal defect. The Merck Manuals: The Merck Manual for Healthcare Professionals. http://www.merckmanuals.com/professional/print/pediatrics/congenital_cardiovascular_anomalies/atrioventricular_septal_defect.html. Accessed July 26, 2012.
- Atrioventricular canal defect. American Heart Association. http://www.heart.org/HEARTORG/Conditions/CongenitalHeartDefects/AboutCongenitalHeartDefects/Complete-Atrioventricular-Canal-defect-CAVC_UCM_307023_Article.jsp. Accessed July 26, 2012.
- Atz AM, et al. Surgical management of complete atrioventricular septal defect: Associations with surgical technique, age, and trisomy 21. Journal of Thoracic and Cardiovascular Surgery. 2011;141:1371.
- Miller A, et al. Long term survival of infants with atrioventricular septal defects. Journal of Pediatrics. 2010;156:994.
- Warnes CA, et al. ACC/AHA 2008 guidelines for the management of adults with congenital heart disease. Journal of the American College of Cardiology. 2008;52:e143.
- Living with a congenital heart defect. National Heart, Lung, and Blood Institute. http://www.nhlbi.nih.gov/health/health-topics/topics/chd/livingwith.html. Accessed July 26, 2012.
- Rasiah SV, et al. Outcome following prenatal diagnosis of complete atrioventricular septal defect. Prenatal Diagnosis. 2008;28:95.