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Catecholaminergic polymorphic ventricular tachycardia

ORPHA:3286· ICD-10 I47.2

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.

Prevalence
1-5 / 10 000
Inheritance
Autosomal dominant, Autosomal recessive
Age of onset
Adolescent, Adult, Childhood