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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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71 news items

Northland's NewsCenter

Northland's NewsCenter
Tue, 24 Nov 2015 15:37:30 -0800

His heart disease is called Atrioventricular canal defect also known as AV canal defect. Just months after Evan's first birthday, he had open heart surgery. That's when he received his blood transfusion, which his mother says let him heal much quicker.

Idaho Press-Tribune

Idaho Press-Tribune
Tue, 27 Oct 2015 23:04:09 -0700

That's because Beckett's parents, Dan and Rachael Gray of Middleton, received word in June that their baby had a heart defect of the atrioventricular canal. Rachel was in her 20th week of the pregnancy when they got the news. The Grays learned more ...

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 ...

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 ...


Thu, 22 Oct 2015 21:18:45 -0700

Twenty weeks into pregnancy, the Grays learned their baby had a heart defect, called an atrioventricular canal defect. Late September, Beckett was born, and deemed healthy enough to go home. Three days in, something scary started to happen. "He was in ...

Shelbyville Times-Gazette

Shelbyville Times-Gazette
Sat, 27 Jun 2015 22:03:45 -0700

Also known as atrioventricular canal defects or endocardial cushion defects, the conditions account for about five percent of all congenital heart disease, and are most common in infants with Down syndrome. According to Cincinnati Children's Hospital ...


Mon, 21 Sep 2015 06:07:30 -0700

In Louisiana, Erika Wigstrom has pleaded guilty to killing her 17-month-old baby by putting hand sanitizer into his feeding tube. Her son, Lucas Ruiz, was born with Down syndrome and an atrioventricular canal defect, which required that he take in ...

Champaign/Urbana News-Gazette

Champaign/Urbana News-Gazette
Sat, 10 Oct 2015 05:00:00 -0700

The cause is unknown. People with the condition are significantly predisposed to certain medical conditions, including congenital heart defects. Gigi was born with atrioventricular canal defect, and underwent a risky surgery to repair it when she was 7 ...

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