raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:3283

His bundle tachycardia

Synonyms: JET · Junctional ectopic tachycardia

Type
Disease
Group
Disorder
Inheritance
Unknown
Age of onset
Infancy, Neonatal
Status
active

His bundle tachycardia is a very rare congenital genetic tachyarrhythmia characterized by incessant tachycardia and high morbidity and mortality.

1

Names in Orphadata languages

12 of 12 languages
LangNameSynonyms
enHis bundle tachycardiaJET · Junctional ectopic tachycardia
frTachycardie hisienneTachycardie jonctionnelle ectopique
deHis-Bündel-TachykardieJET · Tachykardie, ektopische junktionale
esTaquicardia del haz de HisJET · Taquicardia ectópica juntural
itTachicardia del nodo di HisJET · Tachicardia ectopica giunzionale
nlTachycardie van bundel van HisJET · Junctionele ectopische tachycardie
ptTaquicardia do feixe de His
plTachykardia pęczka HisaCzęstoskurcz ogniskowy węzłowy · JET
csTachykardie z Hisova svazkuJET · Junkční ektopická tachykardie
ukПучкова тахікардіяJET · Сумісна ектопічна тахікардія
zh希氏束心动过速异位性交界性心动过速
trDemet taşikardisiJET · Nodel ektopik taşikardi

Definition in:

2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
ICD-10I47.1NTBTinclusionvalidated
ICD-11BC81.1Especificvalidated
MONDO0017989Evalidated
MeSHD013613Evalidated
UMLSC0039235Evalidated
GARD2706Evalidated
MedDRA10074640Evalidated

E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.

3

Classification

Preferential parent (Orphanet linearisation):

Parents in the classifications (1):

No children.

4

Genes

0 direct

Orphanet records no gene directly on this code.

5

Prevalence and incidence

1 record
TypeClassValueAreaQualificationValidationSource
Point prevalenceUnknown—WorldwideClass onlyNot yet validatedORPHANET

Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.

6

HPO phenotypes

4 annotations · validated 2016-06-01
HPOTerm (as annotated by Orphanet)Diagnostic
Very frequent (99-80%)
HP:0011675Arrhythmia
HP:0011716Junctional ectopic tachycardia
Frequent (79-30%)
HP:0001638Cardiomyopathy
Occasional (29-5%)
HP:0100544Neoplasm of the heart

Run the differential on the 2 obligate / very frequent terms

7

Sources for this card