Key result
CPVT triggers malignant ventricular and supraventricular arrhythmias, typically presenting in childhood.
Why the study?
CPVT is a rare inherited channelopathy with malignant ventricular tachyarrhythmias and frequent supraventricular arrhythmias, associated with high mortality if untreated.
Case Report
CPVT is a rare inherited channelopathy associated with both malignant ventricular tachyarrhythmias and supraventricular arrhythmias, presenting a high mortality risk if untreated.
Supports consideration of CPVT in exertional pediatric arrhythmias; extends case-based phenotypic descriptions.
Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a rare inherited cardiac channelopathy characterized by malignant ventricular tachyarrhythmias, typically bidirectional or polymorphic VT during high adrenergic states.1 Up to 25% of patients with CPVT also have been documented to be associated with supraventricular arrhythmias, including atrial tachycardia, atrial flutter, and atrial fibrillation.1 CPVT is associated with high mortality if left untreated, and most patients present in childhood with syncope or sudden cardiac arrest during a high catecholaminergic state.
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Goggins et al. (2025) conducted a case report in Catecholaminergic polymorphic ventricular tachycardia (CPVT). Pathogenic TECRL variant was evaluated. Catecholaminergic polymorphic ventricular tachycardia is a rare channelopathy associated with malignant ventricular and supraventricular arrhythmias, often presenting in childhood.
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