Auditory stimuli as a trigger for arrhythmic events differentiate HERG-related (LQTS2) patients from KVLQT1-related patients (LQTS1)
Arthur A. M. Wilde, MD* ,
Rosalie J. E. Jongbloed ,
Pieter A. Doevendans, MD ||,
Donald R. Düren, MD*,
Richard N. W. Hauer, MD ,
Irene M. van Langen, MD¶,
J. Peter van Tintelen, MD#,
Hubert J. M. Smeets, PhD ,
Henk Meyer, PhD and
Jan L. M. C. Geelen, PhD
* Department of Clinical and Experimental Cardiology, Academic Medical Center, Amsterdam, The Netherlands
Department of Cardiology, Heart Lung Institute, University Hospital, Utrecht, The Netherlands
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)

View larger version (11K):
[in a new window]
|
Figure 1 Extremity leads of a ventricular arrhythmia upon sudden arousal at 3:00 AM by an alarm clock. The HERG mutation in this patient was G604S.
|
|

View larger version (15K):
[in a new window]
|
Figure 2 Ventricular arrhythmia upon sudden arousal at 3.00 A.M. by an alarm clock in a HERG patient (A558P). The leads given are (approximately) V1 and II. See text for discussion.
|
|

View larger version (18K):
[in a new window]
|
Figure 3 ECG recordings of the three sibs III-2, -3 and -4 and their father (II-5). Recordings from lead II, V2 and V5 have been selected. Abnormal, prolonged QT segments are present in patients II-5, III-2 and III-4. Calibrations are standard. The recording in Figure 2 is from patient III-2.
|
|
|