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    Atrioventricular Septal Defect

    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
    7272 Greenville Avenue
    Dallas, TX 75231
    Tel: (214)784-7212
    Fax: (214)784-1307
    Tel: (800)242-8721
    Email: Review.personal.info@heart.org
    Internet: http://www.heart.org

    American Lung Association
    1301 Pennsylvania Ave NW
    Suite 800
    Washington, DC 20004
    USA
    Tel: (202)785-3355
    Fax: (202)452-1805
    Tel: (800)586-4872
    Email: info@lungusa.org
    Internet: http://www.lungusa.org

    NIH/National Heart, Lung and Blood Institute
    P.O. Box 30105
    Bethesda, MD 20892-0105
    Tel: (301)592-8573
    Fax: (301)251-1223
    Email: nhlbiinfo@rover.nhlbi.nih.gov
    Internet: http://www.nhlbi.nih.gov/

    Kids With Heart ~ National Association for Children's Heart Disorders, Inc.
    1578 Careful Dr.
    Green Bay, WI 54304
    Tel: (920)498-0058
    Fax: (920)498-0058
    Tel: (800)538-5390
    Email: michelle@kidswithheart.org
    Internet: http://www.kidswithheart.org

    Little Hearts, Inc.
    P.O. Box 171
    110 Court Street, Suite 3A
    Cromwell, CT 06416
    USA
    Tel: (860)635-0006
    Fax: (860)635-0006
    Tel: (866)435-4673
    Email: info@littlehearts.org
    Internet: http://www.littlehearts.org

    Congenital Heart Information Network (C.H.I.N.)
    P.O. Box 3397
    Margate City, NJ 08402-0397
    Tel: (609)823-4507
    Fax: (609)822-1574
    Email: mb@tchin.org
    Internet: http://www.tchin.org

    Genetic and Rare Diseases (GARD) Information Center
    PO Box 8126
    Gaithersburg, MD 20898-8126
    Tel: (301)251-4925
    Fax: (301)251-4911
    Tel: (888)205-2311
    TDD: (888)205-3223
    Internet: http://rarediseases.info.nih.gov/GARD/

    For a Complete Report:

    This is an abstract of a report from the National Organization for Rare Disorders (NORD). A copy of the complete report can be downloaded free from the NORD website for registered users. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational therapies (if available), and references from medical literature. For a full-text version of this topic, go to www.rarediseases.org and click on Rare Disease Database under "Rare Disease Information".

    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.

    WebMD Medical Reference from the National Organization for Rare Disorders

    Last Updated: May 28, 2015
    This information is not intended to replace the advice of a doctor. Healthwise disclaims any liability for the decisions you make based on this information.

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