Abstract

Catecholaminergic polymorphic ventricular tachycardia (CPVT) characteristically presents with exercise- or emotion-induced syncope during childhood. In 30% of patients, sudden cardiac arrest (SCA) is the first presentation of the disorder [1]. It has been suggested that CPVT is the underlying disease in 56% of cases of unexplained SCA [2]. Diagnosis is based on (family) history of adrenergically induced syncope, response to stress testing and genetic analysis. Delayed diagnosis leads to suboptimal treatment and unnecessary risk of SCA.

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