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Tachycardia, Polymorphic Ventricular, Stress-induced

Synonyms

Catecholaminergic polymorphic ventricular tachycardia; Catecholaminergic bidirectional ventricular tachycardia; Familial polymorphic ventricular tachycardia; Ryanodine receptor mediated ventricular tachycardia

Definition and Characteristics

Stress or exertion induces syncope, premature ventricular contractions, and polymorphic or bidirectional ventricular tachycardia in children and young adults in the absence of structural heart disease (and with a normal QT interval on electrocardiography).

Prevalence

Mutations of the RYR2 gene are rare in a general Italian population. However, a mutation in RYR2 was found in 14 of 30 probands with exertion induced polymorphic ventricular tachycardia in the absence of structural heart disease [1]. To date, more than 40 RyR2 mutations have now been found to be associated with stress-induced ventricular tachycardia [1-4].

Genes

RYR2 gene coding for the cardiac ryanodine receptor,