Marked, transient, emotion-triggered QT accentuation in an adolescent female with type 1 long QT syndrome

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...