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Atrioventricular Septal Defect

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Synonyms of Atrioventricular Septal Defect
  • Atrioventricular Canal Defects
  • AVSD
  • Common Atrioventricular Canal (CAVC) Defect
  • Endocardial Cushion Defects

Disorder Subdivisions

  • Complete Atrioventricular Septal Defect
  • Incomplete Atrioventricular Septal Defect
  • Partial Atrioventricular Septal Defect
  • Transitional 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.
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Organizations related to Atrioventricular Septal Defect
  • American Heart Association
    7272 Greenville Avenue
    Dallas TX 75231-4596
    Phone #: 214-373-6300
    800 #: 800-242-8721
    e-mail: Review.personal.info@heart.org
    Home page: http://www.americanheart.org
  • American Lung Association
    1301 Pennsylvania Ave. NW
    Washington DC 20004
    Phone #: 202-785-3355
    800 #: 800-586-4872
    e-mail: info@lungusa.org
    Home page: http://www.lungusa.org
  • Congenital Heart Information Network (C.H.I.N.)
    101 N Washington Ave, Suite 1A 101 N Washington Ave, Suite 1A
    Margate City NJ 08402-1195
    Phone #: 609-822-1572
    800 #: --
    e-mail: mb@tchin.org
    Home page: http://www.tchin.org
  • Genetic and Rare Diseases (GARD) Information Center
    PO Box 8126
    Gaithersburg MD 20898-8126
    Phone #: 301-251-4925
    800 #: 888-205-2311
    e-mail: http://rarediseases.info.nih.gov/GARD/EmailForm.aspx
    Home page: http://rarediseases.info.nih.gov/GARD
  • Kids With Heart National Association for Children's Heart Disorders, Inc.
    P.O Box 12504
    Green Bay WI 54307-2504
    Phone #: 920-498-0058
    800 #: 800-538-5390
    e-mail: michelle@kidswithheart.org
    Home page: http://www.kidswithheart.org
  • Little Hearts, Inc.
    P.O. Box 171
    110 Court Street, Suite 3A
    Cromwell CT 06416
    Phone #: 860-635-0006
    800 #: 866-435-4673
    e-mail: info@littlehearts.org
    Home page: http://www.littlehearts.org
  • MUMS National Parent-to-Parent Network
    150 Custer Court
    Green Bay WI 54301-1243
    Phone #: 920-336-5333
    800 #: 877-336-5333
    e-mail: mums@netnet.net
    Home page: http://www.netnet.net/mums/
  • NIH/National Heart, Lung and Blood Institute Information Center
    P.O. Box 30105
    Bethesda MD 20824-0105
    Phone #: 301-592-8573
    800 #: --
    e-mail: nhlbiinfo@rover.nhlbi.nih.gov
    Home page: N/A

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Last modified Wednesday, November 26, 2008