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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]

Development[edit]

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]

References[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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Jamaica Gleaner (subscription)

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Sat, 01 Aug 2015 22:48:45 -0700

Nicola Irving said her daughter was born at UHWI in 2009 and she was informed shortly after that she has Down's syndrome, patent ductus arteriosus and atrioventricular canal defect (hole in the heart). A few weeks after, doctors there attempted a ...
 
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... as they showed that Wnt2 knockout mice, which have a heart malformation that resembles a complete atrioventricular canal defect in humans, can actually be rescued by the transient pharmacological activation of the Wnt signaling network using LiCl.
 
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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 ...
 
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Sun, 12 Jul 2015 21:18:45 -0700

... Lagos State University Teaching Hospital, (LASUTH) Dr. B. A. Animashaun, the girl's conditions include anorectal malformations, bilateral Talipes Equinovarius, and acyanotic congenital heart disease, which involved complete Atrioventricular canal ...

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Fri, 03 Jul 2015 23:11:15 -0700

He was later treated for atrioventricular canal defect, where a hole between the chambers of the heart causes problems with the valves that regulate blood flow in the heart. Page 2 of 4 - By the age of one, he'd already had three open heart surgeries ...

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PAH – Pulmonary Arterial Hypertension and A-V – Atrioventricular canal defect is 'a complete atrioventricular canal defect which is a 'combination of defects including a large hole in the centre of the heart and a single common valve instead of the ...

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Thu, 09 Jul 2015 02:18:44 -0700

The hospital has conducted from simple cases of heart diseases including Atrial septal defect, ventricular septal defect to complicated congenital heart diseases like tetralogy of Fallot, partial atrioventricular canal defects, transposition of great ...

Shelbyville Times-Gazette

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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 ...
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