Double outlet left ventricle
Synonyms: DOLV
- Type
- Morphological anomaly
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Infancy, Neonatal
- Status
- active
Double-outlet left ventricle (DOLV) is an extremely rare congenital cardiac malformation in which both the aorta and the pulmonary artery arise, either exclusively or predominantly, from the morphologic left ventricle.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Double outlet left ventricle | DOLV |
| fr | Ventricule gauche à double issue | VGDI |
| de | Doppelausstromventrikel, linker | = en |
| es | Doble salida ventricular izquierda | = en |
| it | Ventricolo sinistro a doppia uscita | = en |
| nl | Linkerventrikel met dubbele uitstroom | DOLV · Double outlet linkerventrikel |
| pt | Ventrículo esquerdo com dupla saída | |
| pl | Dwuujściowa lewa komora | = en |
| cs | Dvojvýtoková levá komora | = en |
| uk | Подвійне відходження великих артерій від лівого шлуночка | = en |
| zh | 左室双出口 | = en |
| tr | Çift çıkışlı sol ventrikül | = en |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | Q20.2 | E | specific | validated |
| ICD-11 | LA85.3 | E | specific | validated |
| MONDO | 0018090 | E | validated | |
| UMLS | C0265809 | E | validated | |
| GARD | 1907 | E | validated | |
| MedDRA | 10080133 | 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:2445Conotruncal heart malformations
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 |
|---|---|---|---|---|---|---|
| Prevalence at birth | 1-9 / 1 000 000 | 0.5 / 100 000 | Worldwide | Value and class | Validated | 21106011[PMID]_22644696[PMID] |
| Point prevalence | <1 / 1 000 000 | — | Europe | Class only | Not yet 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
17 annotations · validated 2023-02-01| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Obligate (100%) | ||
| HP:0011581 | Double outlet left ventricle | |
| Very frequent (99-80%) | ||
| HP:0033118 | Abnormal right ventricular function | |
| HP:0001643 | Patent ductus arteriosus | |
| HP:0001629 | Ventricular septal defect | |
| Frequent (79-30%) | ||
| HP:0011686 | Abnormal coronary artery course | |
| HP:0001640 | Cardiomegaly | |
| HP:0000961 | Cyanosis | |
| HP:0011662 | Tricuspid atresia | |
| Occasional (29-5%) | ||
| HP:0000028 | Cryptorchidism | |
| HP:0001508 | Failure to thrive | |
| HP:0000316 | Hypertelorism | |
| HP:0000202 | Orofacial cleft | |
| HP:0031664 | Systolic heart murmur | |
| HP:0002789 | Tachypnea | |
| Very rare (<4-1%) | ||
| HP:0005182 | Bicuspid pulmonary valve | |
| HP:0004415 | Pulmonary artery stenosis | |
| HP:0010882 | Pulmonary valve atresia | |
Run the differential on the 4 obligate / very frequent terms
Annotation source: 21106011[PMID]_31468015[PMID]_36451474[PMID]_25050529[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/3427
- This record as JSON: /data/d/3427.json