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

Heather N. Anderson, Beth A. Medford, Michael John Ackerman

Research output: Contribution to journalArticle

Abstract

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.

Original languageEnglish (US)
Pages (from-to)376-379
Number of pages4
JournalCardiology in the Young
Volume25
Issue number2
DOIs
StatePublished - Nov 6 2013

Fingerprint

Romano-Ward Syndrome
Long QT Syndrome
Emotions
KCNQ1 Potassium Channel
Frameshift Mutation
Psychological Stress
Cardiac Arrhythmias
Heart Rate
Exercise

Keywords

  • emotion
  • KCNQ1
  • Long QT syndrome
  • prolonged QT
  • stress

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine
  • Pediatrics, Perinatology, and Child Health

Cite this

Marked, transient, emotion-triggered QT accentuation in an adolescent female with type 1 long QT syndrome. / Anderson, Heather N.; Medford, Beth A.; Ackerman, Michael John.

In: Cardiology in the Young, Vol. 25, No. 2, 06.11.2013, p. 376-379.

Research output: Contribution to journalArticle

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