title: Andersen-Tawil syndrome associated with aborted sudden cardiac death: atrial pacing was effective for ventricular arrhythmias.
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.
authors: Division of Cardiology, Osaka General Medical Center, Osaka, Japan.
authors: Yuki
authors: Y
authors: Kuramoto
authors: Yoshio
authors: Y
authors: Furukawa
authors: Takahisa
authors: T
authors: Yamada
authors: Yuji
authors: Y
authors: Okuyama
authors: Masatake
authors: M
authors: Fukunami
mesh_terms: D000328:Adult; D050030:Andersen Syndrome / Q000150:complications* / Q000235:genetics / Q000628:therapy; D000889:Anti-Arrhythmia Agents / Q000627:therapeutic use; D016757:Death, Sudden, Cardiac / Q000209:etiology*; D017147:Defibrillators, Implantable; D005260:Female; D006801:Humans; D007093:Imidazoles / Q000627:therapeutic use; D007564:Japan; D009154:Mutation; D024661:Potassium Channels, Inwardly Rectifying / Q000235:genetics; D013610:Tachycardia / Q000209:etiology* / Q000517:prevention & control / Q000628:therapy; D016896:Treatment Outcome; D018879:Ventricular Premature Complexes / Q000209:etiology* / Q000517:prevention & control / Q000628:therapy
chemical_list: D000889:Anti-Arrhythmia Agents; D007093:Imidazoles; C489457:KCNJ2 protein, human; D024661:Potassium Channels, Inwardly Rectifying; C032151:cifenline