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

Published online by Cambridge University Press:  26 March 2014

Heather N. Anderson ,
Beth A. Medford  and
Michael J. Ackerman
Heather N. Anderson
Affiliation:
Department of Pediatric and Adolescent Medicine, Division of Pediatric Cardiology, Mayo Clinic, Rochester, Minnesota, United States of America
Beth A. Medford
Affiliation:
Department of Pediatric and Adolescent Medicine, Division of Pediatric Cardiology, Mayo Clinic, Rochester, Minnesota, United States of America
Michael J. Ackerman*
Affiliation:
Department of Pediatric and Adolescent Medicine, Division of Pediatric Cardiology, Mayo Clinic, Rochester, Minnesota, United States of America Department of Medicine, Division of Cardiovascular Diseases, Mayo Clinic, Rochester, Minnesota, United States of America Department of Molecular Pharmacology & Experimental Therapeutics; Windland Smith Rice Sudden Death Genomics Laboratory, Mayo Clinic, Rochester, Minnesota, United States of America
*
Correspondence to: M. J. Ackerman, Mayo Clinic Windland Smith Rice Sudden Death Genomics Laboratory Guggenheim 501, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, United States of America. Tel: +507 284 0101; Fax: +507 284 3757; E-mail: ackerman.michael@mayo.edu
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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.

Keywords

Long QT syndromeKCNQ1prolonged QTemotionstress
Type
Brief Reports
Information
Cardiology in the Young , Volume 25 , Issue 2 , February 2015 , pp. 376 - 379
Copyright
© Cambridge University Press 2014 

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