raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:99107

Atrial septal aneurysm

Type
Morphological anomaly
Group
Disorder
Status
active

A rare congenital non-syndromic heart malformation characterized by an abnormal protrusion of the interatrial septum into the right or left atrium, or both, during the cardiorespiratory cycle. The defect may be limited to the fossa ovalis or involve the entire septum. It can present as an isolated finding but is more often associated with interatrial shunts, in particular patent foramen ovale. Clinically it increases the risk of peripheral arterial embolism and stroke.

1

Names in Orphadata languages

12 of 12 languages
LangNameSynonyms
enAtrial septal aneurysm
frAnévrisme du septum interauriculaire
deAtrialseptumaneurysma
esAneurisma del septo auricularAneurisma del septo atrial
itAneurisma del setto interatriale
nlAneurysma van atriumseptum
ptAneurisma do septo auricular
plTętniak przegrody międzyprzedsionkowej
csAneuryzma síňového septa
ukАневризма міжпередсердної перегородки
zh房间隔动脉瘤
trAtriyal septal anevrizma

Definition in:

2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
ICD-10Q21.1NTBTattributedvalidated
ICD-11LA8E.YNTBTindexvalidated
MONDO0020438Evalidated
UMLSC0521533Evalidated

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 (1):

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