raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:99046

Double outlet right ventricle with non-committed subpulmonary ventricular septal defect

Synonyms: DORV with non-committed subpulmonary VSD

Type
Clinical subtype
Group
Subtype of disorder
Inheritance
Multigenic/multifactorial
Status
active

This disease is described under Double outlet right ventricle

1

Names in Orphadata languages

12 of 12 languages
LangNameSynonyms
enDouble outlet right ventricle with non-committed subpulmonary ventricular septal defectDORV with non-committed subpulmonary VSD
frVentricule droit à double issue avec communication interventriculaire à distance des gros vaisseauxDORV avec communication interventriculaire à distance des gros vaisseaux · VDDI avec communication interventriculaire à distance des gros vaisseaux
deRechter Doppelausstromventrikel mit non-committed subpulmonalem VentrikelseptumdefektDORV mit non-committed subpulmonalem VSD
esDoble salida ventricular derecha con comunicación interventricular subpulmonar no comprometidaDSVD con CIV subpulmonar no comprometido
itVentricolo destro a doppia uscita con difetto del setto ventricolare sottopolmonare non connessoDORV con VSD sottopolmonare non connesso
nlRechterventrikel met dubbele uitstroom met non-committed subpulmonaal ventrikelseptumdefectDORV met non-commited subpulmonaal VSD
ptDupla saída do ventrículo direito com defeito septal ventricular sub-pulmonar não implicado
plDwuodpływowa prawa komora z podpłucnym ubytkiem przegrody międzykomorowej oddalonym od wielkich naczyńDORV z podpłucnym VSD oddalonym od wielkich naczyń
csDvojvýtoková pravá komora s neumístěným subpulmonálním defektem komorového septa= en
ukПодвійне відходження магістральних судин від правого шлуночку з субаортальним чи подвійним дефектом міжшлуночкової перетинки та пульмональним стенозом їDORV з незафіксованим підлегеневим синдромом легеневої дисфункції
zh右心室双出口合并未累及肺动脉瓣下室间隔缺损DORV 伴未累及肺动脉瓣下 VSD
trSubpulmoner ventriküler septal defekt ile bağlantısız çift çıkışlı sağ ventrikülBağlantısız subpulmoner VSD ile seyreden DORV
2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
ICD-10Q20.1NTBTattributedvalidated
ICD-11LA85.21Especificvalidated
MONDO0020388Evalidated
UMLSC1956410Evalidated

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