What Is Dextrocardia with Situs Inversus?
Source: Genetic and Rare Diseases (GARD) Information Center
You can contact us here
Dextrocardia with Situs Inversus
Situs Inversus Totalis
Dextrocardia with situs inversus is a condition that is characterized by abnormal positioning of the heart and other internal organs. It is usually present from birth (congenital).
Dextrocardia
Image by Prashanthns
Situs inversus chest
Image by Nevit
Source: Genetic and Rare Diseases (GARD) Information Center
Dextrocardia
Image by Prashanthns
Dextrocardia is a condition in which the heart is located in the right side of the chest instead of the left. It is usually present from birth (congenital). There are several types of dextrocardia. The simplest type occurs when the shape and structure of the heart is a mirror image of a normal heart. Other types of dextrocardia may involve defects of the walls of the heart, nearby blood vessels, or other organs in the abdomen. Chest X-raxys and echocardiograms can be used to determine which type of dextrocardia is present.
Source: Genetic and Rare Diseases (GARD) Information Center
Situs inversus - Mirrored heart and lungs
Image by Adapted from Gray's Anatomy
Situs inversus is a condition in which the arrangement of the internal organs is a mirror image of normal anatomy. It can occur alone (isolated, with no other abnormalities or conditions) or it can occur as part of a syndrome with various other defects. Congenital heart defects are present in about 5-10% of affected people. The underlying cause and genetics of situs inversus are complex. Familial cases have been reported.
Source: Genetic and Rare Diseases (GARD) Information Center
What is primary ciliary dyskinesia?
Image by National Heart Lung and Blood Institute (NIH)
Dextrocardia with situs inversus is a condition that is characterized by abnormal positioning of the heart and other internal organs. In people affected by dextrocardia, the tip of the heart points towards the right side of the chest instead of the left side. Situs inversus refers to the mirror-image reversal of the organs in the chest and abdominal cavity. Some affected people have no obvious signs or symptoms. However, a small percentage of people also have congenital heart defects, usually transposition of the great vessels. Dextrocardia with situs inversus can also be associated with primary ciliary dyskinesia (also known as Kartagener syndrome). Treatment typically depends on the heart or physical problems the person may have in addition to dextrocardia with situs inversus.
Source: Genetic and Rare Diseases (GARD) Information Center
Dextrocardia - The location of the heart on the right
Image by MrArifnajafov
In most cases of dextrocardia with situs inversus, a specific genetic cause has not been identified and inheritance patterns have not been confirmed. However, approximately 25% of affected people have primary ciliary dyskinesia, which is typically inherited in an autosomal recessive manner. This means that to be affected, a person must have a mutation in both copies of the responsible gene in each cell. The parents of an affected person usually each carry one mutated copy of the gene and are referred to as carriers. Carriers typically do not show signs or symptoms of the condition. When two carriers of an autosomal recessive condition have children, each child has a 25% (1 in 4) risk to have the condition, a 50% (1 in 2) risk to be a carrier like each of the parents, and a 25% chance to not have the condition and not be a carrier.
Source: Genetic and Rare Diseases (GARD) Information Center
34 year-old white male with situs inversus due to his Kartagener's syndrome.
Image by Jer5150
In some cases, a diagnosis of dextrocardia with situs inversus is suspected based on the presence of concerning signs and symptoms; however, it is often discovered by chance when an x-ray or ultrasound is performed to investigate a different condition. Computed tomography (CT) scanning is typically the preferred examination to confirm the diagnosis of dextrocardia with situs inversus. Magnetic resonance imaging may be substituted in cases that are associated with congenital heart defects.
Source: Genetic and Rare Diseases (GARD) Information Center
Surgery
Image by sasint/Pixabay
Treatment typically depends on the heart or physical problems the person may have in addition to dextrocardia with situs inversus. For example, infants born with congenital heart defects or other organ malformations may require surgery.
The management of people affected by Kartagener syndrome typically includes measures to enhance clearance of mucus, prevent respiratory infections, and treat bacterial infections.
Source: Genetic and Rare Diseases (GARD) Information Center
Get free access to in-depth articles and track your personal health.
Send this HealthJournal to your friends or across your social medias.