$Unique_ID{BRK03661} $Pretitle{} $Title{Dextrocardia with Situs Inversus} $Subject{Dextrocardia with Situs Inversus Mirror-Image Dextrocardia Heart, Dextroversion Heart, Dextroposition Isolated Dextrocardia Kartagener Syndrome (Kartagener Triad; Sinusitis-Bronchiectasis-Situs Inversus Syndrome; Dextrocardia-Bronchiectasis-Sinusitis Syndrome)} $Volume{} $Log{} Copyright (C) 1987, 1990 National Organization for Rare Disorders, Inc. 466: Dextrocardia with Situs Inversus ** IMPORTANT ** It is possible the main title of the article (Dextrocardia with Situs Inversus) is not the name you expected. Please check the SYNONYMS listing on the next page to find alternate names, disorder subdivisions, and related disorders covered by this article. Synonyms Mirror-Image Dextrocardia Information on the following disorders can be found in the Related Disorders section of this report: Heart, Dextroversion Heart, Dextroposition Isolated Dextrocardia Kartagener Syndrome (Kartagener Triad; Sinusitis-Bronchiectasis-Situs Inversus Syndrome; Dextrocardia-Bronchiectasis-Sinusitis Syndrome) General Discussion ** REMINDER ** The information contained in the Rare Disease Database is provided for educational purposes only. It should not be used for diagnostic or treatment purposes. If you wish to obtain more information about this disorder, please contact your personal physician and/or the agencies listed in the "Resources" section of this report. Dextrocardia with Situs Inversus is a heart condition characterized by abnormal positioning of the heart. In this disorder, the tip of the heart (apex) is positioned on the right side of the chest. Additionally, the position of the heart chambers as well as the visceral organs such as the liver and spleen is reversed (situs inversus). However, most patients can live a normal life without associated symptoms or disability. Symptoms Dextrocardia with Situs Inversus is characterized by the reversal of the normal position of the heart chambers and abdominal organs such as the liver and spleen. The electrocardiogram shows an inversion of the electrical waves from the heart. The apex of the heart is positioned on the right side of the chest whereas it is normally located on the left. Causes Dextrocardia with Situs Inversus is a genetic disorder present at birth. It is transmitted by autosomal recessive genes. The primitive loop in the embryo moves into the reverse direction of its normal position during fetal development, causing displacement of organs. (Human traits including the classic genetic diseases, are the product of the interaction of two genes for that condition, one received from the father and one from the mother. In recessive disorders, the condition does not appear unless a person inherits the same defective gene from each parent. If one receives one normal gene and one gene for the disease, the person will be a carrier for the disease, but usually will show no symptoms. The risk of transmitting the disease to the children of a couple, both of whom are carriers for a recessive disorder, is twenty-five percent. Fifty percent of their children will be carriers, but healthy as described above. Twenty-five percent of their children will receive both normal genes, one from each parent and will be genetically normal.) Affected Population Dextrocardia with Situs Inversus is present at birth. The condition affects males and females in equal numbers. Related Disorders Dextroversion of the heart means that the location of the heart is abnormally positioned in the right half of the chest. The left ventricle remains on the left, but lies in front of the right ventricle. The heart is rotated to the right. The waves representing the heart beat on the electrocardiogram will indicate an abnormality. Dextroposition of the heart is a displacement of the heart to the right. It is usually caused by acquired disease of the lungs, the membrane around the lungs (pleura), or diaphragm. The electrocardiogram is usually normal. Kartagener syndrome is a combination of Dextrocardia with chronic dilatation of the bronchi, difficulty breathing, recurrent respiratory infection (bronchiectasis) and infection of the sinuses (sinusitis). Clubbed fingers and bluish discoloration of the skin (cyanosis) may also be present. Therapies: Standard Treatment of Dextrocardia with Situs Inversus is symptomatic and supportive when needed. In most cases patients can live a normal life without any symptoms or discomfort. If the condition is associated with other more serious heart malformations, the prognosis and treatment will vary. Genetic counseling may be helpful for affected families. Therapies: Investigational This disease entry is based upon medical information available through March 1990. 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. Resources For more information on Dextrocardia with Situs Inversus, please contact: National Organization for Rare Disorders (NORD) P.O. Box 8923 New Fairfield, CT 06812-1783 (203) 746-6518 American Heart Association 7320 Greenville Ave. Dallas, TX 75231 (214) 750-5300 NIH/National Heart, Lung and Blood Institute (NHLBI) 9000 Rockville Pike Bethesda, MD 20892 (301) 496-4236 For information on genetics and genetic counseling referrals, please contact: March of Dimes Birth Defects Foundation 1275 Mamaroneck Avenue White Plains, NY 10605 (914) 428-7100 Alliance of Genetic Support Groups 35 Wisconsin Circle, Suite 440 Chevy Chase, MD 20815 (800) 336-GENE (301) 652-5553 References CONGENITALLY CORRECTED TRANSPOSITION IN THE ADULT: DETECTION BY RADIONUCLIDE ANGIOCARDIOGRAPHY: G.L. Guit, et al.; Radiology (November 1985: issue 157(2)). Pp. 521-527. A POSSIBLE INCREASE IN THE INCIDENCE OF CONGENITAL HEART DEFECTS AMONG THE OFFSPRING OF AFFECTED PARENTS: V. Rose, et al.; Journal Am Coll Cardiol (August 1985: issue 6(2)). Pp. 376-382. INTERNAL MEDICINE, 2nd ed.: Jay H. Stein, et al., eds; Little, Brown, 1987. P. 525.