National Organization for Rare Disorders, Inc.
Important
It is possible that the main title of the report Atrioventricular Septal Defect
is not the name you expected. Please check the synonyms listing to find
the alternate name(s) and disorder subdivision(s) covered by this report.
Synonyms
- AVSD
- Atrioventricular Canal Defects
- Common Atrioventricular Canal (CAVC) Defect
- Endocardial Cushion Defects
Disorder Subdivisions
- Complete Atrioventricular Septal Defect
- Transitional Atrioventricular Septal Defect
- Incomplete Atrioventricular Septal Defect
- Partial Atrioventricular Septal Defect
General Discussion
Atrioventricular septal defect (ASVD) is a general term for a group of rare heart defects that are present at birth (congenital). Infants with ASVDs have improperly developed atrial and ventricular septa and adjoining valves.
The normal heart has four chambers. The two upper chambers, known as atria, are separated from each other by a fibrous partition called the atrial septum. The two lower chambers, known as ventricles, are separated from each other by the ventricular septum. Valves (e.g., mitral and tricuspid) connect the atria (left and right) to their respective ventricles. The valves allow for blood to be pumped through the chambers. Blood travels from the right ventricle through the pulmonary artery to the lungs where it receives oxygen. The blood returns to the heart through pulmonary veins and enters the left ventricle. The left ventricle sends the now oxygen-filled blood into the main artery of the body (aorta). The aorta sends the blood throughout the body.
The parts of the heart described above are formed from an embryonic structure called the endocardial cushions. In individuals with ASVD there is some combination of malformation of these parts of the heart. They may include a hole in the atrial septum, a hole in the ventricular septum, and/or abnormalities of the mitral and triscupid valves. ASVD may be classified as one of three forms: an incomplete (or partial) ASVD (atrial septal defect primum); a transitional form (atrial septal defect and small ventricular septal defect); or a more severe or complete form (large atrial and ventricular defects).
The symptoms of ASVD vary greatly and depend on the severity of the malformations (e.g., valve leakage between ventricles and ventricular size). About half the cases of ASVD occur in children with Down syndrome.
Resources
American Heart Association
National Center
7272 Greenville Avenue
Dallas, TX 75231-4596
Tel: (214)373-6300
Fax: (214)373-0268
Tel: (800)242-8721
Email: inquire@heart.org
Internet: http://www.americanheart.org
Congenital Heart Anomalies, Support, Education, & Resources
2112 North Wilkins Road
Swanton, OH 43558
Tel: (419)825-5575
Fax: (419)825-2880
Email: chaser@compuserve.com
Internet: http://www.csun.edu/~hcmth011/chaser/chaser-news.html
American Lung Association
61 Broadway, 6th Floor
New York, NY 10006
USA
Tel: 2123158700
Fax: 2123158870
Tel: 8005864872
Internet: http://www.lungusa.org
NIH/National Heart, Lung and Blood Institute Information Center
P.O. Box 30105
Bethesda, MD 20824-0105
Tel: (301)592-8573
Fax: (301)251-1223
Email: nhlbiinfo@rover.nhlbi.nih.gov
Kids With Heart National Association for Children's Heart Disorders, Inc.
1578 Careful Dr
Green Bay, WI 54304-2941
Tel: (920)498-0058
Fax: (920)498-0058
Tel: (800)538-5390
Email: kidswithheart@greenbaynet.com
Internet: http://www.kidswithheart.org
Little Hearts, Inc.
P.O. Box 171
Cromwell, CT 06416
USA
Tel: 8606350006
Fax: 8606350006
Tel: 8664354673
Email: info@littlehearts.org
Internet: http://www.littlehearts.org
Congenital Heart Information Network
1561 Clark Dr
Yardley, PA 19067
Tel: (215)493-3068
Fax: (215)493-3068
Email: mb@tchin.org
Internet: http://www.tchin.org
MUMS (Mothers United for Moral Support, Inc) National Parent-to-Parent Network
150 Custer Court
Green Bay, WI 54301-1243
USA
Tel: 9203365333
Fax: 9203390995
Tel: 8773365333
Email: mums@netnet.net
Internet: http://www.netnet.net/mums/
For a Complete Report
This is an abstract of a report from the National Organization for Rare Disorders, Inc. ® (NORD). A copy of the complete report can be obtained for a small fee by visiting the NORD website. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational treatments (if available), and references from medical literature. For a full-text version of this topic, see http://www.rarediseases.org/search/rdblist.html
The information provided in this report is not intended for diagnostic purposes. It is provided for informational purposes only. NORD recommends that affected individuals seek the advice or counsel of their own personal physicians.
It is possible that the title of this topic is not the name you selected. Please check the Synonyms listing to find the alternate name(s) and Disorder Subdivision(s) covered by this report.
This disease entry is based upon medical information available through the date at the end of the topic. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder.
For additional information and assistance about rare disorders, please contact the National Organization for Rare Disorders at P.O. Box 1968, Danbury, CT 06813-1968; phone (203) 744-0100; web site www.rarediseases.org or email orphan@rarediseases.org
Last Updated: 2/11/2008
Copyright 1986, 1994, 2005 National Organization for Rare Disorders, Inc.
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