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Updated: Feb 8, 2026

Dual-Dye Optical Mapping of Hearts from RyR2R2474S Knock-In Mice of Catecholaminergic Polymorphic Ventricular Tachycardia
Published on: December 22, 2023
Three cases of catecholaminergic polymorphic ventricular tachycardia with prolonged QT intervals including two cases
Aki Saito1, Seiko Ohno2, Norihito Nuruki3
1Department of Pediatrics National Hospital Organization Kagoshima Medical Center Kagoshima Japan.
Abstract:
Catecholaminergic polymorphic ventricular tachycardia (CPVT) is one of the leading causes of sudden arrhythmic death in the young. The QT interval in CPVT patients is typically within the normal range. However, those with prolonged QT interval have often been diagnosed with mutation-negative long QT syndrome (LQTS). We report three CPVT patients with prolonged QT interval. Case 1 and 2 were diagnosed as LQTS at first. Genetic test using next-generation sequencing (NGS) revealed RyR2 mutations. We should consider genetic test using NGS to identify the genes responsible for CPVT in mutation-negative LQTS.
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