raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:99117

Coronary sinus stenosis

Type
Morphological anomaly
Group
Disorder
Status
active

A rare congenital anomaly of the coronary sinus characterized by its stenosis at the ostium, lumen, or origin, typically leading to dilation of the vessel. Symptoms are variable and can include palpitations, tachypnea, dyspnea, chest pain, fatigue, and cyanosis. The malformation may be associated with other cardiac anomalies, such as coronary artery-coronary sinus fistula, unroofed coronary sinus, atrial septal defect, coronary sinus-left atrium fistula, total anomalous pulmonary venous connection, and ventricular septal defect.

1

Names in Orphadata languages

12 of 12 languages
LangNameSynonyms
enCoronary sinus stenosis
frSténose du sinus coronaire
deKoronarsinusstenose
esEstenosis del seno coronario
itStenosi del seno coronarico
nlCoronaire sinusstenose
ptEstenose do seio coronário
plZwężenie zatoki wieńcowej
csStenóza koronárního sinu
ukСтеноз коронарного синуса
zh冠状静脉窦狭窄
trKoroner sinüs darlığı

Definition in:

2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
ICD-10Q21.1NTBTattributedvalidated
ICD-11LA86.YBTNTindexvalidated
MONDO0020446Evalidated
UMLSC0344684Evalidated

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