raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:99120

Persistent eustachian valve

Type
Morphological anomaly
Group
Disorder
Status
active

A rare congenital anomaly of the inferior vena cava characterized by the postnatal presence of an eustachian valve remnant, which may be asymptomatic and considered a normal variant or prominent and clinically significant. Clinical presentation is variable and includes obstruction of the inferior vena cava, cyanosis, thrombosis, pulmonary embolism, infective endocarditis, and when combined with persistent foramen ovale, it may generate permanent right-to-left shunt.

1

Names in Orphadata languages

12 of 12 languages
LangNameSynonyms
enPersistent eustachian valve
frValve d'Eustache persistante
deEustachische Klappe, persistierende
esPersistencia de la válvula de Eustaquio
itPersistenza della valvola d'Eustachio
nlPersisterende klep van Eustachius
ptVálvula de eustáquio persistente
plPrzetrwała zastawka Eustachiusza
csPerzistující chlopeň dolní duté žíly
ukПерсистуючий Євстахієв клапан
zh永存下腔静脉瓣
trKalıcı östaki valfi

Definition in:

2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
ICD-10Q26.8NTBTattributedvalidated
ICD-11LA86.YNTBTattributedvalidated
MONDO0020449Evalidated
UMLSC5190784Evalidated

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