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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. 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. [1] Half of the children who are untreated with this condition die during their first year due to heart failure or pneumonia.[2]

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[3]


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.[3]

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. [3]

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.[2]

See also[edit]


  1. ^ Brown, David MD "Atrioventricular Canal Defect" Children's Hospital Boston 20120 http://www.childrenshospital.org/az/Site521/mainpageS521P0.html
  2. ^ 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/
  3. ^ a b c Mayo Clinic September 12, 2012"Atrioventricular Canal Defect" http://www.mayoclinic.com/health/atrioventricular-canal-defect/DS00745
  4. ^ 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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61 news items


Mon, 09 Mar 2015 06:06:24 -0700

Huhn was born with an atrioventricular canal defect, which involves holes in the membrane separating the chambers of the heart. It causes extra blood to be pumped to the lungs and forces the heart and lungs to work harder. She also had a defect in the ...
The Sentinel
Thu, 26 Feb 2015 05:05:55 -0800

Hershey stuck relatively close to average mortality numbers in the nine benchmark procedures. It did not report any deaths in arterial switch operations, fontan procedures, truncus repair, complete atrioventricular canal repair or tetralogy of fallout ...
The Advocate
Wed, 01 Apr 2015 09:07:03 -0700

“The first was congenital patent ductus arteriosus and the second was a much more complex defect known as complete atrioventricular canal defect,” said Unglesby. Atrioventricular canal defect is a combination of several closely associated heart ...
Wilkes Barre Times-Leader
Thu, 26 Feb 2015 21:15:00 -0800

Complete Atrioventricular Canal Repair: A large hole between the two sides of the heart, and one large valve developing where two should have developed. Surgery is usually done within three months of birth, closing the hole and separating the large ...

Chicago Daily Herald

Chicago Daily Herald
Mon, 09 Feb 2015 03:48:45 -0800

Their 3-year-old daughter, Charlotte, was born with a complete atrioventricular canal defect -- a large hole in the center of her heart requiring surgical repair. Located in the area of the heart where the upper chambers join the lower chambers, the ...
Wed, 04 Feb 2015 18:02:14 -0800

Brandon and Karen Reilly of Canonsburg received one of the bags when their son Nolan was going into surgery last month to correct a complete atrioventricular canal defect, a large hole in the center of his heart. “It definitely was helpful in a ...

The Republic

The Republic
Wed, 15 Apr 2015 17:56:51 -0700

Kaleena Joy Carothers and Sarah Violet Kuehner display personalities that are similar to other 3- and 4-year-olds. Kaleena is a fun, bubbly girl who loves to give hugs. Sarah is a curious animal lover who loves to fix things. But early in their young ...
Courthouse News Service
Thu, 26 Feb 2015 10:45:00 -0800

Weeks after her daughter, Sophia Cowan, was born with atrioventricular canal defects in Indiana on July 14, 2011, the baby died on Aug. 7, allegedly as a result of the complications. Atkinson sought punitive damages for claims including fraud and ...

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