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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

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 ...

Finger Lakes Times

Finger Lakes Times
Mon, 22 Jun 2015 14:37:13 -0700

“It was really heartwarming.” Colby, who suffered from atrioventricular canal defect, died on the evening of April 30 — just five hours shy of his 12th birthday — at Golisano Children's Hospital in Rochester. In honor of his birthday and his memory ...


Tue, 26 May 2015 08:56:15 -0700

... confocal and time-lapse imaging, the investigators were able to visualize coronary vessels developing from the endocardium, or the inner lining of the heart - specifically from the atrioventricular canal, the structure that divides the heart into ...
BMC Blogs Network
Wed, 15 Jul 2015 20:11:15 -0700

... cardiogenic role of Pitx2 have been reported in models that express hypomorphic Pitx2, which caused disorganization of the atrial septum, increased deposition of extracellular matrix in the atrial chambers, and elongation of the atrioventricular ...
BMC Blogs Network
Fri, 15 May 2015 07:15:31 -0700

This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original ...
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 ...


Mon, 09 Mar 2015 05:56:15 -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 ...

Chicago Daily Herald

Chicago Daily Herald
Mon, 09 Feb 2015 03:50:51 -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 ...

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