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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 is completed which is when they close the holes in the septum and they make two new atrioventricular valves that is available 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/

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4 news items

The Times-Picayune - NOLA.com
Fri, 08 Aug 2014 13:35:06 -0700

Lucas Ruiz was born with Down syndrome and atrioventricular canal defect, a condition that left him with holes in his heart, requiring corrective surgery and a feeding tube. In explaining why she poisoned her child on Jan. 23 by injecting the Germ-X ...
Kaufman Herald
Wed, 06 Aug 2014 22:09:06 -0700

Walker was born at Texas Health Presbyterian Hospital Kaufman with a congenital heart defect, which included holes in his septum, coarctation of the aorta and an unbalanced atrioventricular canal, which is all to say “he has a small left ventricle ...

Winston-Salem Chronicle

Winston-Salem Chronicle
Thu, 24 Jul 2014 13:11:01 -0700

Prior to Easton's birth, a heart defect – AV Canal Defect (Atrioventricular Canal) and EA/TEF (Esophageal Atresia/Tracheoesophageal Fistula) – was discovered. Initially, the infant did well, but while in the natal intensive care unit, Easton contracted ...

Nigerian Tribune

Nigerian Tribune
Wed, 30 Jul 2014 16:00:30 -0700

Frontier Lifeline, India, representative, Dr Sanjay Cherian, said one of the children had a hole in the heart, while the others had other complex congenital heart defects (atrioventricular canal defect and tetralogy of fallot). The team, which had ...

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