Cor triatriatum sinister
Synonyms: Cor triatriatum sinistrum · Divided left atrium
- Type
- Morphological anomaly
- Group
- Disorder
- Age of onset
- Adult, Childhood, Infancy
- Status
- active
A rare, congenital, non-syndromic, heart malformation characterized by the presence of a thin, fibromuscular membrane subdividing the left atrium into an upper and lower chamber. The upper chamber receives blood from the pulmonary veins and the lower chamber is attached to the left atrial appendage. Therefore, the membrane blocks the orifice of the mitral valve and leads to obstruction of the left ventricular inflow. It may be asymptomatic or present in infancy with tachypnea, dyspnea, hemoptysis, chest pain, syncope, pulmonary edema, pulmonary hypertension, or heart failure, depending on the degree of obstruction. The anomaly may be isolated or associated with other congenital heart anomalies.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Cor triatriatum sinister | Cor triatriatum sinistrum · Divided left atrium |
| fr | Coeur triatrial gauche | Cor triatriatum sinister · Division de l'atrium gauche · Division de l'oreillette gauche |
| de | Cor triatriatum sinister | Geteilter linker Vorhof |
| es | Cor triatriatum sinister | Aurícula izquierda dividida · Cor triatriatum sinistrum |
| it | Cor triatriatum sinister | Atrio sinistro diviso · Cor triatriatum sinistrum |
| nl | Cor triatriatum sinister | Cor triatriatum sinistrum · Verdeeld linkeratrium |
| pt | Cor triatriatum | |
| pl | Serce trójprzedsionkowe lewe | Podzielony lewy przedsionek |
| cs | Cor triatriatum sinister | Cor triatriatum sinistrum · Rozdělená levá síň |
| uk | Трьохпередсердне серце (ліве) | Cor triatriatum sinistrum · Розділене ліве передсердя |
| zh | 左侧三房心 | 左心房三间隔 |
| tr | Kor triatriatum sinister | Bölünmüş sol atriyum · Kor triatriatum sinistrum |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | Q24.2 | NTBT | attributed | validated |
| ICD-11 | LA8G.0 | E | specific | validated |
| MONDO | 0020430 | E | validated | |
| UMLS | C0344712 | E | validated | |
| GARD | 12484 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (1):
- ORPHA:1463Triatrial heart
rare developmental anomalies during embryogenesis; rare cardiac malformations
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | Unknown | — | Worldwide | Class only | Validated | ORPHANET |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/99099
- This record as JSON: /data/d/99099.json