Ventricular ectopy during REM sleep: implications for nocturnal sudden cardiac death.

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: Beta-blocker therapy alone, subsequently combined with mexiletine treatment.