Orphanet: Catecholaminergic polymorphic ventricular tachycardia

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Catecholaminergic polymorphic ventricular tachycardia

Disease definition

Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a severe genetic arrhythmogenic disorder characterized by adrenergically induced ventricular tachycardia (VT) manifesting as syncope and sudden death.


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

Detailed information

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