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J Am Coll Cardiol, 1999; 33:327-332
© 1999 by the American College of Cardiology Foundation
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CLINICAL STUDIES

Auditory stimuli as a trigger for arrhythmic events differentiate HERG-related (LQTS2) patients from KVLQT1-related patients (LQTS1)

Arthur A. M. Wilde, MD* {dagger}, Rosalie J. E. Jongbloed{ddagger} §, Pieter A. Doevendans, MD§ ||, Donald R. Düren, MD*, Richard N. W. Hauer, MD{dagger}, Irene M. van Langen, MD, J. Peter van Tintelen, MD#, Hubert J. M. Smeets, PhD{ddagger}, Henk Meyer, PhD{ddagger} and Jan L. M. C. Geelen, PhD{ddagger}

* Department of Clinical and Experimental Cardiology, Academic Medical Center, Amsterdam, The Netherlands
{dagger} Department of Cardiology, Heart Lung Institute, University Hospital, Utrecht, The Netherlands
{ddagger} Division of Genetics, University Maastricht, Maastricht, The Netherlands
§ Cardiovascular Research Institute Maastricht (CARIM), University Maastricht, Maastricht, The Netherlands
|| Department of Cardiology, University Maastricht, Maastricht, The Netherlands
Department of Clinical Genetics, Academic Medical Center, Amsterdam, The Netherlands
# Clinical Genetics Center Utrecht, Utrecht, and the Interuniversity Cardiology Institute of the Netherlands, Utrecht, The Netherlands. Dr. Wilde is a clinical investigator for the Dutch Heart Foundation (NHS, grant D95/014)

Manuscript received May 11, 1998; revised manuscript received September 1, 1998, accepted October 2, 1998.

Reprint requests and correspondence: Dr. A.A.M. Wilde, Department of Clinical and Experimental Cardiology, Academic Medical Center, University of Amsterdam, P.O. Box 22700, 1100 DE Amsterdam, The Netherlands

Objective

This study was performed to identify a possible relationship between genotype and phenotype in the congenital familial long QT syndrome (cLQTS).

Background

The cLQTS, which occurs as an autosomal dominant or recessive trait, is characterized by QT-interval prolongation on the electrocardiogram and torsade de pointes arrhythmias, which may give rise to recurrent syncope or sudden cardiac death. Precipitators for cardiac events are exercise or emotion and occasionally acoustic stimuli.

Methods

The trigger for cardiac events (syncope, documented cardiac arrhythmias, sudden cardiac death) was analyzed in 11 families with a familial LQTS and a determined genotype.

Results

The families were subdivided in KVLQT1-related families (LQTS1, n = 5) and HERG (human ether-a-gogo-related gene)-related families (LQTS2, n = 6) based on single-strand conformation polymorphism analysis and sequencing. Whereas exercise-related cardiac events dominate the clinical picture of LQTS1 patients, auditory stimuli as a trigger for arrhythmic events were only seen in LQTS2 patients.

Conclusions

Arrhythmic events triggered by auditory stimuli may differentiate LQTS2 from LQTS1 patients.

Abbreviations and Acronyms
  cLQTS = congenital long QT syndrome
  EAD = early afterdepolarization
  ECG = electrocardiogram
  HERG = human ether-a-gogo-related gene
  PCR = polymerase chain reaction
  SCD = sudden cardiac death
  SSCP = single-strand conformation polymorphism




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