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The proper development of the atrioventricular canal into its prospective components (The heart septum and associated valves) to create a clear division between the four compartments of the heart and ensure proper blood movement through the heart, are essential for proper heart function. When this process does not happen correctly, a child will develop atrioventricular canal defect which occurs in 2 out of every 10,000 births.[1] It also has a correlation with Down Syndrome because 20% of children with Down Syndrome suffer from atrioventricular canal disease as well. This is a very serious condition and surgery is necessary within the first six months of life for a child.[2] Half of the children who are untreated with this condition die during their first year due to heart failure or pneumonia.[3]

Atrioventricular canal defect is a combination of abnormalities of the heart and is present at birth. There is a problem when there are holes present in the walls that separate chambers (septa),as well as when valves are incorrectly constructed. There are other names for these heart abnormalities such as endocardial cushion defects or atrioventricular septal defect[4]


A photo of the basic anatomical structures of the heart. During normal heart development all four chambers are separated and the mitral and tricupsid valves are properly developed.

In a normal healthy heart there are four chambers. This consists of right and left atria, and right and left ventricles. The right atrium and right ventricle function to pump blood to the lungs while the left atrium and left ventricle pump blood to the rest of the body. There are valves in place that inhibit back-flow between these chambers.[4]

Clinical relevance[edit]

Atrioventricular canal defect[edit]

This is an echocardiography of "Complete" Atriventricular Canal Disorder. There is a clear absence of lower septum that would separate all four chambers of the heart

This defect is developed because of the improper formation of the endocardial cushions, and their job is to separate the different parts of the heart during development when they fuse. It is strongly associated with Down's syndrome. The structures that develop from the fusion of the endocardial cushions are:

Atrioventricular canal defect may be divided into partial or complete forms. In the partial form, openings between the left and right atria and improper formation of the mitral valve exist. In the complete form, there is free movement in all chambers because there is a large hole where the atria and ventricles meet, and instead of there being two valves there is one common valve.[4]

Surgery is usually conducted in-between the 3rd and 6th month of life, and with this condition the earlier the better. Usually, intracardiac repair involves closing the holes in the septum and the creation of two new atrioventricular valves from the underdeveloped common valve leaflet.[3]

See also[edit]


  1. ^ "Orphanet: Complete atrioventricular canal". www.orpha.net. Retrieved 2015-10-09. 
  2. ^ Brown, David MD "Atrioventricular Canal Defect" Children's Hospital Boston 20120 http://www.childrenshospital.org/az/Site521/mainpageS521P0.html
  3. ^ a b Calabro, Raffaele & Limongelli, Giuseppe "Complete Atrioventricular Canal" 2006 April 5, US National Library of Medicine National Institutes of Health http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1459121/
  4. ^ a b c Mayo Clinic September 12, 2012"Atrioventricular Canal Defect" http://www.mayoclinic.com/health/atrioventricular-canal-defect/DS00745
  5. ^ Cincinnati Children's Hospital "Atrioventricular Septal Defect" April 2010 http://www.cincinnatichildrens.org/health/a/avsd/

Original courtesy of Wikipedia: http://en.wikipedia.org/wiki/Atrioventricular_canal — Please support Wikipedia.
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112 news items

13abc Action News

13abc Action News
Thu, 14 Apr 2016 15:18:45 -0700

Megan says they quickly went from the overwhelming joy of having a new baby to the shock of her diagnosis, "Haven was diagnosed with Atrioventricular Canal Defect. That means there's a hole between the heart's chambers and problems with the valves ...

People Magazine

People Magazine
Wed, 16 Sep 2015 13:00:31 -0700

A Louisiana mother pleaded guilty to killing her 17-month-old son by injecting hand sanitizer into his feeding tube, the Plaquemines Parish District Attorney's Office tells PEOPLE. Lucas Ruiz was born with Down syndrome and an atrioventricular canal ...

Chicago Tribune

Chicago Tribune
Fri, 26 Feb 2016 10:00:00 -0800

The talk of surgery was alarming, she said, but so was the news that Emily's diagnosis of atrioventricular canal defect is a condition that is common in children with Down syndrome. "They kept telling us they were linked but I was in denial ...

East Idaho News

East Idaho News
Mon, 01 Feb 2016 10:56:15 -0800

In June, before Beckett was even born, Beckett's mother, Rachael Gray, found out Beckett had a defect of the atrioventricular canal. This meant he would have to have surgery. Rachael said most doctors won't perform open heart surgery on an infant that ...

Union Democrat

Union Democrat
Thu, 04 Feb 2016 00:26:15 -0800

Common types of heart defects include aortic valve stenosis (AVS), atrial septal defect (ASD), coarctation of the aorta (CoA), complete atrioventricular canal defect (CAVC), d-transposition of the great arteries, Ebstein's anomaly, hypoplastic left ...

Queens Tribune

Queens Tribune
Thu, 25 Feb 2016 08:08:04 -0800

At just six months old, little Nazanin was diagnosed with a complex congenital heart issue called an atrioventricular canal defect. Nazanin's father had a similar heart defect and received life-saving surgery through the Gift of Life program. When his ...
Fri, 18 Mar 2016 11:30:43 -0700

Researchers for a new study have identified six symptoms that are experienced across the spectrum of adult congenital heart disease. These symptoms may be useful for measuring patient-reported outcomes of congenital heart disease.

6 On Your Side

6 On Your Side
Sun, 25 Oct 2015 20:52:37 -0700

Beckett was diagnosed with atrioventricular canal defect, which requires surgery most doctors won't perform until four to six months of age. Doctors predicted Beckett wouldn't show any symptoms of his condition until he was at least two months old, but ...

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