{"orpha":3283,"name":"His bundle tachycardia","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["JET","Junctional ectopic tachycardia"],"definition":"His bundle tachycardia is a very rare congenital genetic tachyarrhythmia characterized by incessant tachycardia and high morbidity and mortality.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-3283/","orphanet_url":"https://www.orpha.net/en/disease/detail/3283","names":{"fr":{"name":"Tachycardie hisienne","synonyms":["Tachycardie jonctionnelle ectopique"],"definition":"Une tachyarythmie génétique congénitale très rare caractérisée par une tachycardie incessante associée à une morbidité et une mortalité élevées."},"de":{"name":"His-Bündel-Tachykardie","synonyms":["JET","Tachykardie, ektopische junktionale"]},"es":{"name":"Taquicardia del haz de His","synonyms":["JET","Taquicardia ectópica juntural"],"definition":"Es una taquicardia, congénita y muy poco frecuente, caracterizada por ser incesante y elevada morbimortalidad."},"it":{"name":"Tachicardia del nodo di His","synonyms":["JET","Tachicardia ectopica giunzionale"],"definition":"La tachicardia del nodo di His è una tachiaritmia congenita molto rara di origine genetica, caratterizzata da tachicardia incessante associata ad elevata morbilità e mortalità."},"nl":{"name":"Tachycardie van bundel van His","synonyms":["JET","Junctionele ectopische tachycardie"],"definition":"His-bundeltachycardie is een zeer zeldzame, congenitale, genetische tachyaritmie die gekarakteriseerd wordt door onophoudelijke tachycardie en hoge morbiditeit en mortaliteit."},"pt":{"name":"Taquicardia do feixe de His"},"pl":{"name":"Tachykardia pęczka Hisa","synonyms":["Częstoskurcz ogniskowy węzłowy","JET"]},"cs":{"name":"Tachykardie z Hisova svazku","synonyms":["JET","Junkční ektopická tachykardie"]},"uk":{"name":"Пучкова тахікардія","synonyms":["JET","Сумісна ектопічна тахікардія"],"definition":"His bundle tachycardia is a very rare congenital genetic tachyarrhythmia characterized by incessant tachycardia and high morbidity and mortality."},"zh":{"name":"希氏束心动过速","synonyms":["异位性交界性心动过速"]},"tr":{"name":"Demet taşikardisi","synonyms":["JET","Nodel ektopik taşikardi"]}},"xrefs":[{"source":"ICD-10","code":"I47.1","qualifier":"NTBT","icd_relation":"inclusion","validated":true},{"source":"ICD-11","code":"BC81.1","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"962827983"},{"source":"MONDO","code":"0017989","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D013613","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0039235","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"2706","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10074640","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":97929,"name":"Rare cardiac disease"},"parents":[{"orpha":101934,"name":"Genetic cardiac rhythm disease","classifications":[146,156]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Unknown"],"onset":["Infancy","Neonatal"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Not yet validated","source":"ORPHANET"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2016-06-01","source":null,"terms":[{"hpo":"HP:0011675","label":"Arrhythmia","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0011716","label":"Junctional ectopic tachycardia","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0001638","label":"Cardiomyopathy","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0100544","label":"Neoplasm of the heart","frequency":"Occasional (29-5%)","criteria":null}]}}