Type 1 long QT syndrome is the most common long QT syndrome genetic subtype. Exercise and emotional stress can precipitate sudden cardiac events in patients with type 1 long QT syndrome; however, the precise mechanism remains elusive. We report the case of a teenage girl with type 1 long QT syndrome secondary to a rare frameshift mutation (p. L191fs+90X) in the KCNQ1-encoded Kv7.1 potassium channel. During emotional distress, her continuous QTc recordings precipitously increased, peaking within minutes to 669 ms and then returning to baseline (520 ms) as she calmed without concomitant increase in heart rate. This is the first described case documenting transient, marked accentuation of the QTc interval in a long QT syndrome patient during emotional distress. Such events may be triggered by transient accentuation of the intrinsic perturbation in cardiac repolarisation and increase the risk of degeneration to a ventricular arrhythmia. This case illustrates the need improved understanding of the complex interaction between emotion and cardiac stability in patients with long QT syndrome.
- Long QT syndrome
- prolonged QT
ASJC Scopus subject areas
- Cardiology and Cardiovascular Medicine
- Pediatrics, Perinatology, and Child Health