Torsade-de-pointes syndrome with short coupling interval
- Type
- Disease
- Group
- Disorder
- Age of onset
- Adult
- Status
- active
A rare variant of Torsade de pointes, a polymorphic ventricular tachycardia, which is characterized by a short coupling interval of the first TdP beat on electrocardiogram in the absence of any structural heart disease. It manifests in early adulthood with syncope, often results in ventricular fibrillation and shows a high risk of sudden cardiac death.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Torsade-de-pointes syndrome with short coupling interval | |
| fr | Syndrome des torsades de pointes à couplage court | |
| de | Torsade-de-Pointes-Tachykardie mit kurzem Kopplungsintervall | |
| es | Síndrome de torsade-de-pointes con intervalo de acoplamiento corto | |
| it | Sindrome da torsione della punta ad intervallo di accoppiamento breve | |
| nl | Torsade-de-pointes-syndroom met kort koppelingsinterval | |
| pt | Síndrome de torsade-de-pointes com intervalo de acoplamento curto | |
| pl | Zespół Torsade-de-pointes o krótkim czasie sprzężenia | |
| cs | Syndrom Torsade-de-pointes (TdP) s krátkým spojovacím intervalem | |
| uk | Синдром torsade-de-pointes з коротким інтервалом зчеплення | |
| zh | 短联律间期尖端扭转型室性心动过速综合征 | |
| tr | Kısa bağlantı aralıklı Torsade-de-pointes sendromu |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| OMIM | 613600 | E | validated | |
| ICD-10 | I47.2 | NTBT | attributed | validated |
| ICD-11 | BC71.01 | NTBT | index | validated |
| MONDO | 0013317 | E | validated | |
| UMLS | C3150851 | 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:101934Genetic cardiac rhythm disease
rare cardiac diseases; rare genetic diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
2 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Cases/families | 14 cases | Worldwide | Case(s) | Validated | 8281648[PMID] | |
| Point prevalence | <1 / 1 000 000 | — | Worldwide | Class only | Validated | 8281648[PMID]_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/51084
- This record as JSON: /data/d/51084.json