Abstract

A 37-year-old Japanese woman experienced aborted sudden cardiac death from ventricular fibrillation and was diagnosed with Andersen-Tawil syndrome by genetic analysis that revealed 2 mutations in the KCNJ2 gene. Although she received an implantation of implantable cardioverter defibrillator and beta-blocker therapy, the frequency of premature ventricular contraction and bidirectional ventricular tachycardia did not decrease. Her ventricular arrhythmias increased after a full stomach test and a neostigmine provocation test, and reduced after cibenzoline administration, which indicates the relation with vagal tone. Moreover, increasing the pacing rate significantly decreased them. These findings indicate that the arrhythmia was bradycardia-dependent in this case.

Links

Publisher Full Text

Authors

Kuramoto Y, Furukawa Y, Yamada T, Okuyama Y, Fukunami M

Institution

Division of Cardiology, Osaka General Medical Center, Osaka, Japan.

Source

The American journal of the medical sciences 344:3 2012 Sep pg 248-50

MeSH

Adult
Andersen Syndrome
Anti-Arrhythmia Agents
Death, Sudden, Cardiac
Defibrillators, Implantable
Female
Humans
Imidazoles
Japan
Mutation
Potassium Channels, Inwardly Rectifying
Tachycardia
Treatment Outcome
Ventricular Premature Complexes

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

22739561