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$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.