Primary superior vena cava aneurysm
Synonyms: SVCA
- Type
- Malformation syndrome
- Group
- Disorder
- Inheritance
- Not applicable
- Status
- active
A rare primary vascular anomaly characterized by mostly fusiform, rarely saccular aneurysm of the superior vena cava. Patients can be asymptomatic or manifest mostly with dyspnea, chest pain, pain in the retrosternal region, hypotension, acute upper or lower respiratory tract infection symptoms and left arm paresthesias. It is usually incidentally detected due to the presence of mediastinal shadow in X-ray.
Names in Orphadata languages
10 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Primary superior vena cava aneurysm | SVCA |
| fr | Anévrisme primitif de la veine cave supérieure | Anévrisme de la VCS |
| de | Primäres Aneurysma der Vena cava superior | Primäres Aneurysma der oberen Hohlvene |
| es | Aneurisma primario de la vena cava superior | = en |
| it | Aneurisma primitivo della vena cava superiore | = en |
| nl | Primair aneurysma van vena cava superior | Primair aneurysma van bovenste holle ader · SVCA |
| pt | Aneurisma primário de veia cava superior | = en |
| pl | Pierwotny tętniak żyły głównej górnej | = en |
| cs | Primární aneuryzma vena cava superior | = en |
| uk | Первинна аневризма верхньої порожнистої вени | = en |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | Q26.8 | NTBT | attributed | validated |
| MONDO | 0958097 | E | validated | |
| MONDO | 958097 | E | validated | |
| UMLS | C5925121 | 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:95498Congenital anomaly of superior vena cava
rare developmental anomalies during embryogenesis; rare cardiac malformations; rare circulatory system diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
0 recordsNo prevalence record in this release.
HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/652668
- This record as JSON: /data/d/652668.json