raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:566862

Left isomerism

Synonyms: LAI · Left atrial isomerism

Type
Clinical subtype
Group
Subtype of disorder
Status
active

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.

1

Names in Orphadata languages

12 of 12 languages
LangNameSynonyms
enLeft isomerismLAI · Left atrial isomerism
frIsomérisme gaucheIsomérisme atrial gauche
deLinksisomerieIsomerie des linken Vorhofanhängsels · LAI · Linksatrialer Isomerismus
esIsomerismo izquierdoIsomerismo auricular izquierdo
itIsomerismo sinistroIsomerismo atriale sinistro · LAI
nlLinker isomerieLAI · Linker atriale isomerie · Linker atriaal isomerisme
ptIsomerismo atrial esquerdo
plIzomeryzm lewostronnyLAI
csLevostranná síňová izomerie= en
ukЛівобічна ізомерія передсердьІзомерія лівого передсердно-шлуночкового вузла · LAI
zh左房异构左心耳异构
trSol atriyal izomerizmLAI · Sol atriyal uzantının izomerizmi

Definition in:

2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
ICD-10Q20.6NTBTattributedvalidated
ICD-11LA8YNTBTindexvalidated
MONDO0035451Evalidated
UMLSC0344694Evalidated

E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.

3

Classification

Preferential parent (Orphanet linearisation):

Parents in the classifications (2):

No children.

4

Genes

0 direct

Orphanet records no gene directly on this code.

5

Prevalence and incidence

0 records

No prevalence record in this release.

6

HPO phenotypes

Orphanet has no HPO annotation for this code in this release.

7

Sources for this card