Abstract
Background A young adult female presented with syncope and periodic weakness. A 12-lead electrocardiogram showed frequent premature ventricular contractions and prolonged QU interval. Repetitive runs of nonsustained ventricular tachycardia were recorded at night.
Investigations Electromyography, muscle biopsy, MRI, echocardiography, exercise stress testing using Bruce protocol with microvolt T-wave alternans testing, 24 h Holter monitoring, electrophysiological testing and examination of the effects of sleep and sleep stage on the patient's ventricular arrhythmias.
Diagnosis Type 1 Andersen–Tawil syndrome, (also known as type 7 long QT syndrome). Severe ventricular arrhythmia was observed, predominantly during rapid eye movement sleep. We speculate that the autonomic instability present during rapid eye movement sleep precipitates increasing vulnerability to sleep-related ventricular tachycardia.
Management β-blocker therapy alone, subsequently combined with mexiletine treatment.
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Acknowledgements
Dr Somers is supported by NIH grants HL-65176, HL-70302, HL-73211 and M01-RR00585, and Dr Ackerman's research program is supported by the Mayo Clinic Windland Smith Rice Comprehensive Sudden Cardiac Death Program, the Dr Scholl Foundation, the CJ Foundation for SIDS, the Hannah Wernke Memorial Foundation, the AHA (Established Investigator Award), and the NIH (HD42569).
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Garcia-Touchard, A., Somers, V., Kara, T. et al. Ventricular ectopy during REM sleep: implications for nocturnal sudden cardiac death. Nat Rev Cardiol 4, 284–288 (2007). https://doi.org/10.1038/ncpcardio0877
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DOI: https://doi.org/10.1038/ncpcardio0877
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