Congenitally-Corrected Transposition of the Great Arteries

Congenitally-Corrected Transposition of the Great Arteries

Article
Healthy Lungs
Heart & Vascular Health
+3
Contributed byMaulik P. Purohit MD MPHApr 19, 2018

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

  • Congenitally-Corrected Transposition of the Great Vessels

What is Congenitally-Corrected Transposition of the Great Arteries? (Definition/Background Information)

  • Congenitally-Corrected Transposition of the Great Arteries is a rare heart defect that occurs when the ventricles and attached valves are switched
  • As a result, the aorta and the pulmonary artery are connected to the wrong lower heart chambers
  • While the oxygen-poor blood still flows to the lungs, and oxygen-rich blood still flows out to nourish the body, other heart problems (such as septal defects, pulmonary stenosis, tricuspid regurgitation, and heart block) are often associated with this defect and require treatment

(Source: Congenitally Corrected Transposition of the Great Arteries; Genetic and Rare Diseases Information Center (GARD) of National Center for Advancing Translational Sciences (NCATS), USA.)

Who gets Congenitally-Corrected Transposition of the Great Arteries? (Age and Sex Distribution)

  • Congenitally Corrected Transposition of the Great Arteries 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 Congenitally-Corrected Transposition of the Great Arteries? (Predisposing Factors)

  • Limited data suggests that air pollutants and hair dye may act as environmental risk factors for Congenitally-Corrected Transposition of the Great Arteries
  • Also, having a family history of this heart defect is a risk factor. It has been estimated that the recurrence risk in siblings is around 3% to 5%

(Source: Congenitally Corrected Transposition of the Great Arteries; Genetic and Rare Diseases Information Center (GARD) of National Center for Advancing Translational Sciences (NCATS), USA.)

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 Congenitally-Corrected Transposition of the Great Arteries? (Etiology)

The exact cause of formation of Congenitally-Corrected Transposition of the Great Arteries is presently not known.

What are the Signs and Symptoms of Congenitally-Corrected Transposition of the Great Arteries?

The signs and symptoms of Congenitally Corrected Transposition of the Great Arteries may include:

  • Septal defects
  • Pulmonary stenosis
  • Tricuspid regurgitation
  • Heart block

(Source: Congenitally Corrected Transposition of the Great Arteries; Genetic and Rare Diseases Information Center (GARD) of National Center for Advancing Translational Sciences (NCATS), USA.)

How is Congenitally-Corrected Transposition of the Great Arteries Diagnosed?

Congenitally Corrected Transposition of the Great Arteries 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 Congenitally-Corrected Transposition of the Great Arteries?

The complications of Congenitally Corrected Transposition of the Great Arteries may include:

  • Severe heart abnormalities
  • Respiratory difficulties

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

How is Congenitally-Corrected Transposition of the Great Arteries Treated?

The treatment for Congenitally-Corrected Transposition of the Great Arteries is usually given to manage the signs and symptoms and any complications that develops.

How can Congenitally-Corrected Transposition of the Great Arteries be Prevented?

Congenitally-Corrected Transposition of the Great Arteries may not be preventable.

  • If there is a family history of the condition, then genetic counseling will help assess risks, before planning for a child
  • Active research is currently being performed to explore the possibilities for treatment and prevention of inherited and acquired genetic disorders

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

What is the Prognosis of Congenitally-Corrected Transposition of the Great Arteries? (Outcomes/Resolutions)

  • The prognosis of Congenitally Corrected Transposition of the Great Arteries is dependent upon the severity of the signs and symptoms and associated complications, if any
  • Individuals with mild conditions have 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 Congenitally-Corrected Transposition of the Great Arteries:

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

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

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Maulik P. Purohit MD MPH picture
Approved by

Maulik P. Purohit MD MPH

Assistant Medical Director, Medical Editorial Board, DoveMed Team

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