What are the other Names for this Condition? (Also known as/Synonyms)

• Birth Defect - Dextrocardia
• Congenital Heart Defect - Dextrocardia
• Cyanotic Heart Defect - Dextrocardia

What is Dextrocardia? (Definition/Background Information)

• 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-rays and echocardiograms can be used to determine which type of Dextrocardia is present

(Source: Dextrocardia; Genetic and Rare Diseases Information Center (GARD) of National Center for Advancing Translational Sciences (NCATS), USA.)

Who gets Dextrocardia? (Age and Sex Distribution)

• Dextrocardia is a rare congenital disorder. The presentation of symptoms may occur at birth
• Both males and females may be affected
• Worldwide, individuals of all racial and ethnic groups may be affected

What are the Risk Factors for Dextrocardia? (Predisposing Factors)

The risk factors for Dextrocardia may include the following:

• A positive family history may be an important risk factor, since Dextrocardia can be inherited
• Use of illegal drugs during pregnancy may increase the risk of giving birth to a baby with the disorder
• Being diabetic before pregnancy is a risk factor for certain types of Dextrocardia

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Dextrocardia? (Etiology)

• The exact underlying cause of Dextrocardia is currently unknown
• However, researchers suspect that there may be genetic and/or environmental factors that contribute to the development of the disorder

What are the Signs and Symptoms of Dextrocardia?

In Dextrocardia without any structural abnormalities of heart or other internal organs, there may be no signs and symptoms present. However, in many cases, Dextrocardia may occur with abnormalities in heart structure, as well as absence, re-arrangement, or structural abnormalities of other internal organs. When this occurs, Dextrocardia may present with the following signs and symptoms, which may vary in type and severity among affected individuals:

• Paleness
• Bluish tinge to skin
• Listlessness
• Labored breathing
• Frequent infections of the respiratory tract and sinuses
• Inability to gain weight
• Jaundice (yellow skin and eyes)

How is Dextrocardia Diagnosed?

Dextrocardia is diagnosed on the basis of the following information:

• Complete physical examination
• Thorough medical history evaluation
• Assessment of signs and symptoms
• Laboratory tests
• Imaging studies
• Biopsy studies, if necessary

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Dextrocardia?

Typically, Dextrocardia may not lead to complications, if it exists as a mirror image of a normal heart. In cases where there are positional as well as structural abnormalities in the heart and other internal organs, the following complications may develop:

• Frequent infections due to absence of spleen
• Pneumonia
• Intestinal malrotation leading to malabsorption
• Infertility in males, if Dextrocardia occurs as part of Kartagener syndrome (a genetic disorder with dysfunctional cilia)
• Heart failure

Complications may occur with or without treatment, and in some cases, due to treatment also.

How is Dextrocardia Treated?

For those cases of Dextrocardia where the heart is a mirror image of a normal heart without structural or functional abnormalities, no treatment may be necessary.

• However, if there are structural defects in the heart and other organs, surgery may be required to repair the same
• In newborns and infants, medications may be necessary before surgery can be attempted. These medicines are typically prescribed to help the baby feed better, gain weight and fight infections, and may include:
  • Antibiotics for infections, or long-term antibiotics prescription when spleen is absent
  • Diuretics
  • Inotropes to modulate the heart’s contractions
  • Angiotensin-converting enzyme (ACE) inhibitors, to reduce blood pressure

How can Dextrocardia be Prevented?

• Dextrocardia may not be preventable, if it forms a part of an inherited condition

• Avoiding all types of illegal drugs during pregnancy may help reduce the risk of giving birth to a child with Dextrocardia
• If a woman has diabetes, seeking medical attention for good control of blood sugar prior to pregnancy may help reduce the risk
• If there is a family history of the condition, then genetic counseling will help assess risks, before planning for a child
• In such cases, genetic testing of the expecting parents (and related family members) and prenatal diagnosis (molecular testing of the fetus during pregnancy) may help in understanding the risks better during pregnancy

• Regular medical screening at periodic intervals with tests and physical examinations are recommended

What is the Prognosis of Dextrocardia? (Outcomes/Resolutions)

The prognosis is good with normal life expectancies, for those cases of Dextrocardia without structural or functional abnormalities of the heart.

• For individuals with symptomatic Dextrocardia with other associated abnormalities and symptoms, the prognosis is dependent upon the severity of the signs and symptoms and associated complications, if any
• Individuals with mild conditions may have a better prognosis than those with severe symptoms and complications
• Typically, the prognosis may be assessed on a case-by-case basis

Additional and Relevant Useful Information for Dextrocardia:

The following DoveMed website link is a useful resource for additional information:

http://www.dovemed.com/diseases-conditions/rare-disorders/