Andersen-Tawil syndrome associated with aborted sudden cardiac death: atrial pacing was effective for ventricular arrhythmias.

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.