title: Benign premature ventricular complexes from the right ventricular outflow tract triggered polymorphic ventricular tachycardia in a latent type 2 LQTS patient.
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.
authors.affiliation: Division of Cardiology, Niigata University Graduate School of Medical and Dental Sciences, Japan.
authors.forename: Akinori
authors.initials: A
authors.lastname: Sato
authors.forename: Masaomi
authors.initials: M
authors.lastname: Chinushi
authors.forename: Keiko
authors.initials: K
authors.lastname: Sonoda
authors.forename: Akira
authors.initials: A
authors.lastname: Abe
authors.forename: Daisuke
authors.initials: D
authors.lastname: Izumi
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Furushima
mesh_terms: D019943:Amino Acid Substitution; D017115:Catheter Ablation; D000072237:ERG1 Potassium Channel; D004562:Electrocardiography; D004837:Epinephrine; D051638:Ether-A-Go-Go Potassium Channels / Q000235:genetics; D005260:Female; D006801:Humans; D008133:Long QT Syndrome / Q000175:diagnosis / Q000235:genetics / Q000503:physiopathology*; D008875:Middle Aged; D020125:Mutation, Missense; D017180:Tachycardia, Ventricular / Q000235:genetics / Q000503:physiopathology* / Q000628:therapy; D018879:Ventricular Premature Complexes / Q000235:genetics / Q000503:physiopathology* / Q000628:therapy; D000098850:Polymorphic Catecholaminergic Ventricular Tachycardia
chemical_list: D000072237:ERG1 Potassium Channel; D051638:Ether-A-Go-Go Potassium Channels; C000606913:KCNH2 protein, human; D004837:Epinephrine