Congenital left ventricular aneurysm
- Type
- Malformation syndrome
- Group
- Disorder
- Age of onset
- Antenatal, Neonatal
- Status
- active
A rare congenital non-syndromic heart malformation characterized by a bulging of the left ventricular wall, connected to the left ventricle by a wide neck (with a ratio of the connection to the body of the anomaly >1). The dimensions of aneurysms have been described as small as 0.5 cm in diameter and as big as 8x9 cm in size. Most frequent locations are the left ventricular apex and the perivalvular area. Aneurysms can be a- or dyskinetic or show almost normal contractility. Patients may remain asymptomatic or present with systemic embolization, congestive heart failure, valvular regurgitation, ventricular wall rupture, ventricular tachycardia, or sudden cardiac death.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Congenital left ventricular aneurysm | |
| fr | Anévrysme congénital ventriculaire gauche | |
| de | Fetales Aneurysma des linken Ventrikels | |
| es | Aneurisma congénito ventricular izquierdo | |
| it | Aneurisma congenito del ventricolo destro | |
| nl | Congenitaal linkerventrikelaneurysma | |
| pt | Aneurisma ventricular esquerdo congénito | |
| pl | Tętniak lewej komory serca płodu | |
| cs | Fatální aneuryzma levé komory srdeční | |
| uk | Вроджена аневризма лівого шлуночка | |
| zh | 先天性左心室室壁瘤 | |
| tr | Doğumsal sol ventrikül anevrizması |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | Q24.8 | NTBT | attributed | validated |
| ICD-11 | LA88.Y | NTBT | index | validated |
| MONDO | 0021690 | E | validated | |
| UMLS | C0344915 | E | validated | |
| GARD | 2305 | 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:88991Rare congenital heart disease
rare developmental anomalies during embryogenesis; rare cardiac malformations
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
2 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | Unknown | — | Worldwide | Class only | Validated | ORPHANET |
| Cases/families | 809 cases | Worldwide | Case(s) | Validated | 25782048[PMID] |
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
6 annotations · validated 2019-06-17| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Obligate (100%) | ||
| HP:0001711 | Abnormal left ventricle morphology | |
| Frequent (79-30%) | ||
| HP:0001635 | Congestive heart failure | |
| Occasional (29-5%) | ||
| HP:0012249 | Abnormal ST segment | |
| HP:0005135 | Abnormal T-wave | |
| HP:0002104 | Apnea | |
| HP:0011675 | Arrhythmia | |
Run the differential on the 1 obligate / very frequent terms
Annotation source: 1870816[PMID]_11231449[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/1055
- This record as JSON: /data/d/1055.json