Advertisement

Submit Manuscripts
Author Information
Subscribe/Renew
Order Reprints
Email Alerts
Feedback
RSS Feed
CME

About the Journal
Editorial Board & Staff

JACC Imaging Homepage JACC Interventions Homepage
Still not a subscriber to JACC Imaging or JACC Interventions?
Click here
to sign up now!

ACC.org
Cardiosmart
Cardiosource
CVN
Imaging Library
JACC Imaging
JACC Interventions

Click here for more guidelines.
ACCF/ASNC/ACR/
AHA/ASE/SCCT/
SCMR/SNM 2009 Appropriate Use Criteria for Cardiac Radionuclide Imaging
SCIENCE ADVISORY: Percutaneous Device Closure of Patent Foramen Ovale for Secondary Stroke Prevention
ACCF/AHA 2009 Expert Consensus Document on Pulmonary Hypertension
CME Topic Collections Past Issues Search Current Issue Home
     

J Am Coll Cardiol, 1999; 33:327-332
© 1999 by the American College of Cardiology Foundation
This Article
Right arrow Figures Only
Right arrow Full Text
Right arrow Full Text (PDF)
Right arrow Alert me when this article is cited
Right arrow Alert me if a correction is posted
Services
Right arrow Email this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Download to citation manager
Citing Articles
Right arrow Citing Articles via HighWire
Right arrow Citing Articles via Google Scholar
Google Scholar
Right arrow Articles by Wilde, A. A. M.
Right arrow Articles by Geelen, J. L. M. C.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Wilde, A. A. M.
Right arrow Articles by Geelen, J. L. M. C.

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




This article has been cited by other articles:


Home page
 EuropaceHome page
N. Colman, A. Bakker, M. Linzer, J. B. Reitsma, W. Wieling, and A. A.M. Wilde
Value of history-taking in syncope patients: in whom to suspect long QT syndrome?
Europace, July 1, 2009; 11(7): 937 - 943.
[Abstract] [Full Text] [PDF]

Home page
 J. Physiol.Home page
R. M. Hardman and I. D. Forsythe
Ether-à-go-go-related gene K+ channels contribute to threshold excitability of mouse auditory brainstem neurons
J. Physiol., June 1, 2009; 587(11): 2487 - 2497.
[Abstract] [Full Text] [PDF]

Home page
 NeurologyHome page
J. N. Johnson, N. Hofman, C. M. Haglund, G. D. Cascino, A.A.M. Wilde, and M. J. Ackerman
Identification of a possible pathogenic link between congenital long QT syndrome and epilepsy
Neurology, January 20, 2009; 72(3): 224 - 231.
[Abstract] [Full Text] [PDF]

Home page
 Cleveland Clinic Journal of MedicineHome page
E. LEVINE, S. Z. ROSERO, A. S. BUDZIKOWSKI, A. J. MOSS, W. ZAREBA, and J. P. DAUBERT
Congenital long QT syndrome: Considerations for primary care physicians
Cleveland Clinic Journal of Medicine, August 1, 2008; 75(8): 591 - 600.
[Abstract] [Full Text] [PDF]

Home page
 NEJMHome page
D. M. Roden
Long-QT Syndrome
N. Engl. J. Med., January 10, 2008; 358(2): 169 - 176.
[Full Text] [PDF]

Home page
 Cardiovasc ResHome page
E. Gordon, G. Panaghie, L. Deng, K. J. Bee, T. K. Roepke, T. Krogh-Madsen, D. J. Christini, H. Ostrer, C. T. Basson, W. Chung, et al.
A KCNE2 mutation in a patient with cardiac arrhythmia induced by auditory stimuli and serum electrolyte imbalance
Cardiovasc Res, January 1, 2008; 77(1): 98 - 106.
[Abstract] [Full Text] [PDF]

Home page
 Am. J. Physiol. Regul. Integr. Comp. Physiol.Home page
E. Nalivaiko, P. G. Catcheside, A. Adams, A. S. Jordan, D. J. Eckert, and R. D. McEvoy
Cardiac changes during arousals from non-REM sleep in healthy volunteers
Am J Physiol Regulatory Integrative Comp Physiol, March 1, 2007; 292(3): R1320 - R1327.
[Abstract] [Full Text] [PDF]

Home page
 CirculationHome page
H. L. Tan, A. Bardai, W. Shimizu, A. J. Moss, E. Schulze-Bahr, T. Noda, and A. A. M. Wilde
Genotype-Specific Onset of Arrhythmias in Congenital Long-QT Syndrome: Possible Therapy Implications
Circulation, November 14, 2006; 114(20): 2096 - 2103.
[Abstract] [Full Text] [PDF]

Home page
 J Am Coll CardiolHome page
Developed in Collaboration With the European Heart, D. P. Zipes, A. J. Camm, M. Borggrefe, A. E. Buxton, B. Chaitman, M. Fromer, G. Gregoratos, G. Klein, A. J. Moss, et al.
ACC/AHA/ESC 2006 Guidelines for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death: A Report of the American College of Cardiology/American Heart Association Task Force and the European Society of Cardiology Committee for Practice Guidelines (Writing Committee to Develop Guidelines for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death)
J. Am. Coll. Cardiol., September 5, 2006; 48(5): e247 - e346.
[Full Text] [PDF]

Home page
 EuropaceHome page
Writing Committee Members, D. P. Zipes, A. J. Camm, M. Borggrefe, A. E. Buxton, B. Chaitman, M. Fromer, G. Gregoratos, G. Klein, A. J. Moss, et al.
ACC/AHA/ESC 2006 guidelines for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death: A report of the American College of Cardiology/American Heart Association Task Force and the European Society of Cardiology Committee for Practice Guidelines (Writing Committee to Develop Guidelines for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death) Developed in collaboration with the European Heart Rhythm Association and the Heart Rhythm Society
Europace, September 1, 2006; 8(9): 746 - 837.
[Full Text] [PDF]

Home page
 EuropaceHome page
A. Anastasakis, C.-M. Kotta, S. Kyriakogonas, B. Wollnik, A. Theopistou, and C. Stefanadis
Phenotype reveals genotype in a Greek long QT syndrome family.
Europace, April 1, 2006; 8(4): 241 - 244.
[Abstract] [Full Text] [PDF]

Home page
 J Am Coll CardiolHome page
M. Viitasalo, L. Oikarinen, H. Swan, K. A. Glatter, H. Vaananen, H. Fodstad, N. Chiamvimonvat, K. Kontula, L. Toivonen, and M. M. Scheinman
Ratio of Late to Early T-Wave Peak Amplitude in 24-h Electrocardiographic Recordings as Indicator of Symptom History in Patients With Long-QT Syndrome Types 1 and 2
J. Am. Coll. Cardiol., January 3, 2006; 47(1): 112 - 120.
[Abstract] [Full Text] [PDF]

Home page
 J Am Coll CardiolHome page
M. Viitasalo, L. Oikarinen, H. Swan, K. A. Glatter, H. Vaananen, H. Fodstad, N. Chiamvimonvat, K. Kontula, L. Toivonen, and M. M. Scheinman
Ratio of Late to Early T-Wave Peak Amplitude in 24-h Electrocardiographic Recordings as Indicator of Symptom History in Patients With Long-QT Syndrome Types 1 and 2
J. Am. Coll. Cardiol., December 13, 2005; (2005) j.jacc.2005.07.068v1.
[Abstract] [Full Text] [PDF]

Home page
 Cardiovasc ResHome page
W. Shimizu
The long QT syndrome: Therapeutic implications of a genetic diagnosis
Cardiovasc Res, August 15, 2005; 67(3): 347 - 356.
[Abstract] [Full Text] [PDF]

Home page
 Physiol. Rev.Home page
C. E. Clancy and R. S. Kass
Inherited and Acquired Vulnerability to Ventricular Arrhythmias: Cardiac Na+ and K+ Channels
Physiol Rev, January 1, 2005; 85(1): 33 - 47.
[Abstract] [Full Text] [PDF]

Home page
 J Am Coll CardiolHome page
E. F. D. Wever and E. O. Robles de Medina
Sudden death in patients without structural heart disease
J. Am. Coll. Cardiol., April 7, 2004; 43(7): 1137 - 1144.
[Abstract] [Full Text] [PDF]

Home page
 Circ. Res.Home page
K. A. Glatter and N. Chiamvimonvat
Tachy- or Bradyarrhythmias: Implications for Therapeutic Intervention in LQT3 Families
Circ. Res., May 16, 2003; 92(9): 941 - 943.
[Full Text] [PDF]

Home page
 JAMAHome page
S. M. Al-Khatib, N. M. A. LaPointe, J. M. Kramer, and R. M. Califf
What Clinicians Should Know About the QT Interval
JAMA, April 23, 2003; 289(16): 2120 - 2127.
[Abstract] [Full Text] [PDF]

Home page
 Cardiovasc ResHome page
H. L Tan, C. R Bezzina, J. P.P Smits, A. O Verkerk, and A. A.M Wilde
Genetic control of sodium channel function
Cardiovasc Res, March 15, 2003; 57(4): 961 - 973.
[Abstract] [Full Text] [PDF]

Home page
 J. Med. Genet.Home page
I M Van Langen, E Birnie, M Alders, R J Jongbloed, H Le Marec, and A A M Wilde
The use of genotype-phenotype correlations in mutation analysis for the long QT syndrome
J. Med. Genet., February 1, 2003; 40(2): 141 - 145.
[Full Text] [PDF]

Home page
 ANN INTERN MEDHome page
X. H.T. Wehrens, M. A. Vos, P. A. Doevendans, and H. J.J. Wellens
Novel Insights in the Congenital Long QT Syndrome
Ann Intern Med, December 17, 2002; 137(12): 981 - 992.
[Abstract] [Full Text] [PDF]

Home page
 Eur Heart JHome page
C. Antzelevitch
Sympathetic modulation of the long QT syndrome
Eur. Heart J., August 2, 2002; 23(16): 1246 - 1252.
[PDF]

Home page
 J Am Coll CardiolHome page
W. Shimizu, Y. Tanabe, T. Aiba, M. Inagaki, T. Kurita, K. Suyama, N. Nagaya, A. Taguchi, N. Aihara, K. Sunagawa, et al.
Differential effects of beta-blockade on dispersion of repolarization in the absence and presence of sympathetic stimulation between the lqt1 and lqt2 forms of congenital long qt syndrome
J. Am. Coll. Cardiol., June 19, 2002; 39(12): 1984 - 1991.
[Abstract] [Full Text] [PDF]

Home page
 Eur Heart JHome page
T. Noda, H. Takaki, T. Kurita, K. Suyama, N. Nagaya, A. Taguchi, N. Aihara, S. Kamakura, K. Sunagawa, K. Nakamura, et al.
Gene-specific response of dynamic ventricular repolarization to sympathetic stimulation in LQT1, LQT2 and LQT3 forms of congenital long QT syndrome
Eur. Heart J., June 2, 2002; 23(12): 975 - 983.
[Abstract] [Full Text] [PDF]

Home page
 Cardiovasc ResHome page
C. E Conrath, A. A.M Wilde, R. J.E Jongbloed, M. Alders, I. M van Langen, J Peter van Tintelen, P. A Doevendans, and T. Opthof
Gender differences in the long QT syndrome: effects of {beta}-adrenoceptor blockade
Cardiovasc Res, February 15, 2002; 53(3): 770 - 776.
[Abstract] [Full Text] [PDF]

Home page
 Eur Heart J SupplHome page
C. Antzelevitch
Heterogeneity of cellular repolarization in LQTS: the role of M cells
Eur. Heart J. Suppl., September 1, 2001; 3(suppl_K): K2 - K16.
[Abstract] [PDF]

Home page
 Cardiovasc ResHome page
A. A.M. Wilde and D. Escande
LQT genotype-phenotype relationships: patients and patches
Cardiovasc Res, September 1, 2001; 51(4): 627 - 629.
[Full Text] [PDF]

Home page
 Cardiovasc ResHome page
C. E. Clancy and Y. Rudy
Cellular consequences of HERG mutations in the long QT syndrome: precursors to sudden cardiac death
Cardiovasc Res, May 1, 2001; 50(2): 301 - 313.
[Abstract] [Full Text] [PDF]

Home page
 J Am Coll CardiolHome page
Y. Tanabe, M. Inagaki, T. Kurita, N. Nagaya, A. Taguchi, K. Suyama, N. Aihara, S. Kamakura, K. Sunagawa, K. Nakamura, et al.
Sympathetic stimulation produces a greater increase in both transmural and spatial dispersion of repolarization in LQT1 than LQT2 forms of congenital long QT syndrome
J. Am. Coll. Cardiol., March 1, 2001; 37(3): 911 - 919.
[Abstract] [Full Text] [PDF]

Home page
 Am. J. Physiol. Heart Circ. Physiol.Home page
W. Han, Z. Wang, and S. Nattel
Slow delayed rectifier current and repolarization in canine cardiac Purkinje cells
Am J Physiol Heart Circ Physiol, March 1, 2001; 280(3): H1075 - H1080.
[Abstract] [Full Text] [PDF]

Home page
 CirculationHome page
P. J. Schwartz, S. G. Priori, C. Spazzolini, A. J. Moss, G. M. Vincent, C. Napolitano, I. Denjoy, P. Guicheney, G. Breithardt, M. T. Keating, et al.
Genotype-Phenotype Correlation in the Long-QT Syndrome : Gene-Specific Triggers for Life-Threatening Arrhythmias
Circulation, January 2, 2001; 103(1): 89 - 95.
[Abstract] [Full Text] [PDF]

Home page
 CirculationHome page
A. A. M. Wilde and D. M. Roden
Predicting the Long-QT Genotype From Clinical Data : From Sense to Science
Circulation, December 5, 2000; 102(23): 2796 - 2798.
[Full Text] [PDF]

Home page
 HeartHome page
D. M RODEN and M. E ANDERSON
The pause that refreshes, or does it? Mechanisms in torsades de pointes
Heart, September 1, 2000; 84(3): 235 - 237.
[Full Text] [PDF]

Home page
 J Am Coll CardiolHome page
C.-E. Chiang and D. M. Roden
The long QT syndromes: genetic basis and clinical implications
J. Am. Coll. Cardiol., July 1, 2000; 36(1): 1 - 12.
[Abstract] [Full Text] [PDF]

Home page
 J Am Coll CardiolHome page
F. E. S. Cruz Filho, I. G. Maia, M.a. L. A. Fagundes, R. C. P. Barbosa, P. A. G. Alves, R. M. S. Sa, S. H. Boghossian, and J. C. Ribeiro
Electrical behavior of T-Wave polarity alternans in patients with congenital long QT syndrome
J. Am. Coll. Cardiol., July 1, 2000; 36(1): 167 - 173.
[Abstract] [Full Text] [PDF]

Home page
 HeartHome page
S Viskin, R Fish, D Zeltser, B Belhassen, K Heller, D Brosh, S Laniado, and H V Barron
Arrhythmias in the congenital long QT syndrome: how often is torsade de pointes pause dependent?
Heart, June 1, 2000; 83(6): 661 - 666.
[Abstract] [Full Text]

Home page
 J Am Coll CardiolHome page
W. Shimizu and C. Antzelevitch
Differential effects of beta-adrenergic agonists and antagonists in LQT1, LQT2 and LQT3 models of the long QT syndrome
J. Am. Coll. Cardiol., March 1, 2000; 35(3): 778 - 786.
[Abstract] [Full Text] [PDF]

Home page
 J. Biol. Chem.Home page
S. Kathofer, W. Zhang, C. Karle, D. Thomas, W. Schoels, and J. Kiehn
Functional Coupling of Human beta 3-Adrenoreceptors to the KvLQT1/MinK Potassium Channel
J. Biol. Chem., August 25, 2000; 275(35): 26743 - 26747.
[Abstract] [Full Text] [PDF]


 
  CME Topic Collections Past Issues Search Current Issue Home

Advertisement