Related Experiment Video
Updated: May 16, 2026

Determining the Likelihood of Variant Pathogenicity Using Amino Acid-level Signal-to-Noise Analysis of Genetic Variation
Published on: January 16, 2019
Benign premature ventricular complexes from the right ventricular outflow tract triggered polymorphic ventricular
Akinori Sato1, Masaomi Chinushi, Keiko Sonoda
1Division of Cardiology, Niigata University Graduate School of Medical and Dental Sciences, Japan.
Abstract:
A 57-year-old woman showed frequent premature ventricular complexes (PVCs) originating from the right ventricular outflow tract (RVOT), and some of the PVCs triggered polymorphic ventricular tachycardia (PVT). Structural heart diseases were ruled out by conventional cardiac examinations. Radiofrequency catheter ablation was successful in eliminating the PVCs and subsequent PVT. However, epinephrine infusion unmasked her prolonged QT interval, and a genetic analysis revealed a KCNH2 mutation (R694H) as the cause of latent type-2 long QT syndrome (LQTS). This case suggests that latent LQTS may work as an arrhythmogenic substrate of PVT triggered by a benign form of RVOT-PVCs in patients with a structurally normal heart.
Related Concept Videos
ECG Interpretation of Arrhythmias II: Atrial, Junctional and Ventricular Arrhythmias
Dysrhythmias III: Characteristics of Dysrhythmias
Dysrhythmias II: Classification of Tachyarrhythmias
Dysrhythmias IV: Characteristics of Bradyarrhythmias
Disturbances in Heart Rhythm
Arrhythmias are categorized by their speed, rhythm, and origin. A slow heart...
Mechanism of Cardiac Arrhythmias

