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Start free with EleplanCongenitally corrected transposition of the great arteries
ORPHA:216694Morphological anomaly
Also called Congenitally corrected transposition of the great vessels · Discordant ventriculoarterial and atrioventricular connections · Double discordance
What it is
A rare cardiac malformation characterized by the combination of discordant atrioventricular (AV) and ventriculo-arterial (VA) connections so that the morphologically right atrium connects to the morphologically left ventricle, the morphologically left atrium connects to the morphologically right ventricle, the morphologically right ventricle connects to the aorta, and the morphologically left ventricle connects to the pulmonary trunk. It is usually accompanied by other cardiovascular malformations which determine the clinical picture.
Key facts
- Prevalence
- 1-9 / 100 000 (at birth)
- Age of onset
- All ages
- Inheritance
- Not applicable
- Classified as
- Morphological anomaly
Prevalence figures describe populations, not individuals, and are often estimates from a single study. Where the figure is an incidence or a birth prevalence, it says so.
Signs and symptoms
Very common80–99%
6Common30–79%
8These ranges are how often a sign appears across everyone diagnosed, not a prediction for one person — most people have some of these and not others. Orphanet also records findings that are specifically absent in this disease; those are in the data but not listed here.
ICD-10 codes
Related health topics
Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.
Cross-references
Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.
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