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Start free with EleplanLeft isomerism
ORPHA:566862Clinical subtype
Also called LAI · Left atrial isomerism
What it is
A rare, congenital, non-syndromic, cardiovascular malformation characterized by the position of the heart being predominantly to the right of the thoracic midline, with the cardiac apex pointing to the right, due to abnormalities of embryologic origin that are intrinsic to the heart itself. Additional cardiac defects (e.g. septal defects, congenitally corrected transposition of the great arteries) can be associated.
Key facts
- Classified as
- Clinical subtype
Recorded for the broader condition
- Prevalence
- 1-5 / 10 000 (at birth, United States)Visceral heterotaxy
- Age of onset
- Antenatal, Infancy, NeonatalVisceral heterotaxy
- Inheritance
- Autosomal dominant, Autosomal recessive, X-linked recessiveVisceral heterotaxy
Orphanet records these for the broader condition rather than for this specific form. They are a starting point, not a figure for this subtype — subtypes often differ, and some are defined by being rarer than the condition they sit under.
ICD-10 codes
ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.
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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