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. 2017 Nov 20;57(6):905–906. doi: 10.2169/internalmedicine.9372-17

Atrioventricular Block-induced Torsades de Pointes Associated with KCNQ1-G269S

Tadashi Nakajima 1, Takashi Sugawara 1, Yoshiaki Kaneko 1, Masahiko Kurabayashi 1
PMCID: PMC5891537  PMID: 29151524

A 78-year-old woman was referred to our hospital because she had been suffering from recurrent episodes of syncope for the last 3 weeks. She had neither a history of syncope nor any family history of sudden death or long QT syndrome (LQTS). A 12-lead electrocardiogram (ECG) showed complete atrioventricular block (AVB) [heart rate (HR): 45 bpm] with QT prolongation (QTc: 541 ms) (Picture A), although ECG recorded 4 months earlier showed sinus rhythm (HR: 66 bpm) with slight QT prolongation (QTc: 449 ms) (Picture B). Holter ECG documented torsades de pointes (TdP) triggered by a short-long-short interval of ventricular activation during complete AVB (Picture C). Blood tests revealed no electrolytes disturbance, and echocardiogram revealed no structural heart disease. Ventricular pacing eliminated the recurrence of TdP. A genetic analysis of major LQTS-related genes identified a KCNQ1-G269S mutation (Picture D), which has previously been reported to be associated with adrenergic-induced LQTS (1,2). This case demonstrated that AVB could be a precipitating factor for TdP in adrenergic-induced LQTS.

Picture.

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The authors state that they have no Conflict of Interest (COI).

References

  • 1.Chen S, Zhang L, Bryant RM, et al. KCNQ1 mutations in patients with a family history of lethal cardiac arrhythmias and sudden death. Clin Genet 63: 273-282, 2003. [DOI] [PMC free article] [PubMed] [Google Scholar]
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