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Catecholaminergic polymorphic ventricular tachycardia

Disease definition

A rare, severe genetic arrhythmogenic disorder of the structurally normal heart characterized by catecholamine-induced ventricular tachycardia (VT) manifesting as syncope and sudden death in young individuals.


Classification level: Disorder
  • Synonym(s):
    • Bidirectional ventricular tachycardia induced by catecholamine
    • CPVT
    • Malignant paroxysmal ventricular tachycardia
    • Polymorphic ventricular tachycardia induced by catecholamines
  • Prevalence: 1-5 / 10 000
  • Inheritance: Autosomal dominant or Autosomal recessive 
  • Age of onset: Adult, Childhood, Adolescent
  • ICD-10: I47.2
  • ICD-11: BC65.5
  • OMIM: 604772  611938  614021  614916  615441
  • UMLS: C1631597
  • MeSH: -
  • GARD: 4421
  • MedDRA: -

Detailed information


Disease review articles

Genetic Testing

ERN : produced/endorsed by ERN(s)
FSMR : produced/endorsed by FSMR(s)
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