Advertisement

Click here for more guidelines.
2012 ACCF/ AATS/ SCAI/ STS Expert Consensus Document on Transcatheter Aortic Valve Replacement
ACCF/ SCAI/ STS/ AATS/ AHA/ ASNC/ HFSA/ SCCT 2012 Appropriate Use Criteria for Coronary Revascularization Focused Update
2012 ACCF/ AHA/ ACR/ SCAI/ SIR/ STS/ SVM/ SVN Key Data Elements and Definitions for Peripheral Atherosclerotic Vascular Disease

 
About JACC CME
Policy on Privacy and Confidentiality
Disclosures
Important Information
 

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

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

Cardiosmart
Cardiosource
CVN
Imaging Library
JACC Imaging
JACC Interventions
 
 

About the Journal
Editorial Board & Staff
 
CME Topic Collections Past Issues Search Current Issue Home
     

J Am Coll Cardiol, 2008; 51:2291-2300, doi:10.1016/j.jacc.2008.02.068
© 2008 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 Web of Science
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 Web of Science (44)
Right arrow Citing Articles via Google Scholar
Google Scholar
Right arrow Articles by Goldenberg, I.
Right arrow Articles by Moss, A. J.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Goldenberg, I.
Right arrow Articles by Moss, A. J.

STATE-OF-THE-ART PAPER

Long QT Syndrome

Ilan Goldenberg, MD* and Arthur J. Moss, MD

Cardiology Division, Department of Medicine, University of Rochester Medical Center, Rochester, New York.

Manuscript received December 31, 2007; revised manuscript received February 19, 2008, accepted February 26, 2008.

* Reprint requests and correspondence: Dr. Ilan Goldenberg, Heart Research Follow-up Program, University of Rochester Medical Center, 601 Elmwood Avenue, Box 653, Rochester, New York 14642-8653. (Email: Ilan.Goldenberg{at}heart.rochester.edu).

The hereditary long QT syndrome (LQTS) is a genetic channelopathy with variable penetrance that is associated with increased propensity to syncope, polymorphous ventricular tachycardia (torsades de pointes), and sudden arrhythmic death. This inherited cardiac disorder constitutes an important cause of malignant ventricular arrhythmias and sudden cardiac death in young individuals with normal cardiac morphology. Risk assessment in affected LQTS patients relies upon a constellation of electrocardiographic, clinical, and genetic factors. Administration of beta-blockers is the mainstay therapy in affected patients, and primary prevention with an implantable cardioverter defibrillator or left cervicothoracic sympathetic denervation are therapeutic options in patients who remain symptomatic despite beta-blocker therapy. Accumulating data from the International LQTS Registry have recently facilitated a comprehensive analysis of risk factors for aborted cardiac arrest or sudden cardiac death in pre-specified age groups, including the childhood, adolescence, adulthood, and post-40 periods. These analyses have consistently indicated that the phenotypic expression of LQTS is time dependent and age specific, warranting continuous risk assessment in affected patients. Furthermore, the biophysical function, type, and location of the ion-channel mutation are currently emerging as important determinants of outcome in genotyped patients. These new data may be used to improve risk stratification and for the development of gene-specific therapies that may reduce the risk of life-threatening cardiac events in patients with this inherited cardiac disorder.

Abbreviations and Acronyms
  ACA = aborted cardiac arrest
  ECG = electrocardiogram
  ICD = implantable cardioverter-defibrillator
  LCSD = left cervicothoracic sympathetic denervation
  LQTS = long QT syndrome
  QTc = corrected QT
  SCD = sudden cardiac death




This article has been cited by other articles:


Home page
 Am. J. Physiol. Heart Circ. Physiol.Home page
Y.-C. Lin, J. Huang, H. Kan, V. Castranova, J. C. Frisbee, and H.-G. Yu
Defective calcium inactivation causes long QT in obese insulin-resistant rat
Am J Physiol Heart Circ Physiol, February 1, 2012; 302(4): H1013 - H1022.
[Abstract] [Full Text] [PDF]

Home page
 CirculationHome page
R. W. Sy, C. van der Werf, I. S. Chattha, P. Chockalingam, A. Adler, J. S. Healey, M. Perrin, M. H. Gollob, A. C. Skanes, R. Yee, et al.
Derivation and Validation of a Simple Exercise-Based Algorithm for Prediction of Genetic Testing in Relatives of LQTS Probands
Circulation, November 15, 2011; 124(20): 2187 - 2194.
[Abstract] [Full Text] [PDF]

Home page
 Circ Cardiovasc GenetHome page
A. Barsheshet, A. J. Moss, S. McNitt, S. Polonsky, C. M. Lopes, W. Zareba, J. L. Robinson, M. J. Ackerman, J. Benhorin, E. S. Kaufman, et al.
Risk of Syncope in Family Members Who Are Genotype-Negative for a Family-Associated Long-QT Syndrome Mutation
Circ Cardiovasc Genet, October 1, 2011; 4(5): 491 - 499.
[Abstract] [Full Text] [PDF]

Home page
 Mol. Pharmacol.Home page
V. Garg, A. Stary-Weinzinger, F. Sachse, and M. C. Sanguinetti
Molecular Determinants for Activation of Human Ether-a-go-go-related Gene 1 Potassium Channels by 3-Nitro-N-(4-phenoxyphenyl) Benzamide
Mol. Pharmacol., October 1, 2011; 80(4): 630 - 637.
[Abstract] [Full Text] [PDF]

Home page
 Eur Heart JHome page
M. Cerrone and S. G. Priori
Genetics of sudden death: focus on inherited channelopathies
Eur. Heart J., September 1, 2011; 32(17): 2109 - 2118.
[Abstract] [Full Text] [PDF]

Home page
 Circ Cardiovasc GenetHome page
S. K. Westaway, K. Reinier, A. Huertas-Vazquez, A. Evanado, C. Teodorescu, J. Navarro, M. F. Sinner, K. Gunson, J. Jui, P. Spooner, et al.
Common Variants in CASQ2, GPD1L, and NOS1AP Are Significantly Associated With Risk of Sudden Death in Patients With Coronary Artery Disease
Circ Cardiovasc Genet, August 1, 2011; 4(4): 397 - 402.
[Abstract] [Full Text] [PDF]

Home page
 Anesth. Analg.Home page
A. T. Nathan, D. H. Berkowitz, L. M. Montenegro, S. C. Nicolson, V. L. Vetter, and D. R. Jobes
Implications of Anesthesia in Children with Long QT Syndrome
Anesth. Analg., May 1, 2011; 112(5): 1163 - 1168.
[Abstract] [Full Text] [PDF]

Home page
 Psychosom. Med.Home page
R. D. Lane, C. Carmichael, and H. T. Reis
Differentiation in the Momentary Rating of Somatic Symptoms Covaries With Trait Emotional Awareness in Patients at Risk for Sudden Cardiac Death
Psychosom Med, February 1, 2011; 73(2): 185 - 192.
[Abstract] [Full Text] [PDF]

Home page
 J Am Coll CardiolHome page
I. Goldenberg, S. Horr, A. J. Moss, C. M. Lopes, A. Barsheshet, S. McNitt, W. Zareba, M. L. Andrews, J. L. Robinson, E. H. Locati, et al.
Risk for Life-Threatening Cardiac Events in Patients With Genotype-Confirmed Long-QT Syndrome and Normal-Range Corrected QT Intervals
J. Am. Coll. Cardiol., January 4, 2011; 57(1): 51 - 59.
[Abstract] [Full Text] [PDF]

Home page
 CirculationHome page
A. A. Alsheikh-Ali, C. Madias, S. Supran, and M. S. Link
Marked Variability in Susceptibility to Ventricular Fibrillation in an Experimental Commotio Cordis Model
Circulation, December 14, 2010; 122(24): 2499 - 2504.
[Abstract] [Full Text] [PDF]

Home page
 The ESC Textbook of Acute and Intensive Cardiac CareHome page
H.-R. Arntz
Chapter 2 Sudden cardiac death: epidemiology and prevention
The ESC Textbook of Acute and Intensive Cardiac Care, December 1, 2010; 1(1): med-9780199584314-chapter - med-9780199584314-chapter.
[Abstract] [Full Text] [PDF]

Home page
 EuropaceHome page
C. J. Garratt, P. Elliott, E. Behr, A. J. Camm, C. Cowan, S. Cruickshank, A. Grace, M. J. Griffith, A. Jolly, P. Lambiase, et al.
Heart Rhythm UK position statement on clinical indications for implantable cardioverter defibrillators in adult patients with familial sudden cardiac death syndromes
Europace, August 1, 2010; 12(8): 1156 - 1175.
[Abstract] [Full Text] [PDF]

Home page
 Circ Arrhythm ElectrophysiolHome page
M. G. Hoogendijk, T. Opthof, P. G. Postema, A. A. M. Wilde, J. M. T. de Bakker, and R. Coronel
The Brugada ECG Pattern: A Marker of Channelopathy, Structural Heart Disease, or Neither? Toward a Unifying Mechanism of the Brugada Syndrome
Circ Arrhythm Electrophysiol, June 1, 2010; 3(3): 283 - 290.
[Full Text] [PDF]

Home page
 J Am Coll CardiolHome page
C. Jons, A. J. Moss, I. Goldenberg, J. Liu, S. McNitt, W. Zareba, M. Qi, and J. L. Robinson
Risk of Fatal Arrhythmic Events in Long QT Syndrome Patients After Syncope
J. Am. Coll. Cardiol., February 23, 2010; 55(8): 783 - 788.
[Abstract] [Full Text] [PDF]

Home page
 Sports Health: A Multidisciplinary ApproachHome page
T. S. Baman, S. Gupta, and S. M. Day
Cardiovascular Health, Part 2: Sports Participation in Athletes With Cardiovascular Conditions
Sports Health: A Multidisciplinary Approach, January 1, 2010; 2(1): 19 - 28.
[Abstract] [Full Text] [PDF]

Home page
 J. Biol. Chem.Home page
A. Sato, T. Arimura, N. Makita, T. Ishikawa, Y. Aizawa, H. Ushinohama, Y. Aizawa, and A. Kimura
Novel Mechanisms of Trafficking Defect Caused by KCNQ1 Mutations Found in Long QT Syndrome
J. Biol. Chem., December 11, 2009; 284(50): 35122 - 35133.
[Abstract] [Full Text] [PDF]

Home page
 CirculationHome page
P. J. Schwartz, M. Stramba-Badiale, L. Crotti, M. Pedrazzini, A. Besana, G. Bosi, F. Gabbarini, K. Goulene, R. Insolia, S. Mannarino, et al.
Prevalence of the Congenital Long-QT Syndrome
Circulation, November 3, 2009; 120(18): 1761 - 1767.
[Abstract] [Full Text] [PDF]

Home page
 Eur Heart JHome page
Developed in collaboration with, European Heart Rhythm Association (EHRA), Heart Failure Association (HFA), and Heart Rhythm Society (HRS), Endorsed by the following societies, European Society of Emergency Medicine (EuSEM), European Federation of Internal Medicine (EFIM), European Union Geriatric Medicine Society (EUGMS), American Geriatrics Society (AGS), European Neurological Society (ENS), et al.
Guidelines for the diagnosis and management of syncope (version 2009): The Task Force for the Diagnosis and Management of Syncope of the European Society of Cardiology (ESC)
Eur. Heart J., November 1, 2009; 30(21): 2631 - 2671.
[Full Text] [PDF]

Home page
 Sci Transl MedHome page
A. M. Goldman, E. Glasscock, J. Yoo, T. T. Chen, T. L. Klassen, and J. L. Noebels
Arrhythmia in Heart and Brain: KCNQ1 Mutations Link Epilepsy and Sudden Unexplained Death
Science Translational Medicine, October 14, 2009; 1(2): 2ra6 - 2ra6.
[Abstract] [Full Text] [PDF]

Home page
 Br. J. Sports. Med.Home page
M S Link
Prevention of sudden cardiac death: return to sport considerations in athletes with identified cardiovascular abnormalities
Br. J. Sports Med., September 1, 2009; 43(9): 685 - 689.
[Abstract] [Full Text] [PDF]

Home page
 J Am Coll CardiolHome page
G. M. Marcus, E. Keung, and M. M. Scheinman
The Year in Review of Clinical Cardiac Electrophysiology
J. Am. Coll. Cardiol., August 25, 2009; 54(9): 777 - 787.
[Full Text] [PDF]

Home page
 J Am Coll CardiolHome page
C. Spazzolini, J. Mullally, A. J. Moss, P. J. Schwartz, S. McNitt, G. Ouellet, T. Fugate, I. Goldenberg, C. Jons, W. Zareba, et al.
Clinical Implications for Patients With Long QT Syndrome Who Experience a Cardiac Event During Infancy
J. Am. Coll. Cardiol., August 25, 2009; 54(9): 832 - 837.
[Abstract] [Full Text] [PDF]

Home page
 Ther Adv Cardiovasc DisHome page
R. Shephard and C. Semsarian
Advances in the prevention of sudden cardiac death in the young
Therapeutic Advances in Cardiovascular Disease, April 1, 2009; 3(2): 145 - 155.
[Abstract] [PDF]

Home page
 Cleveland Clinic Journal of MedicineHome page
E. LEVINE and J. P. DAUBERT
IN REPLY:
Cleveland Clinic Journal of Medicine, February 1, 2009; 76(2): 88 - 88.
[Full Text] [PDF]

Home page
 J Am Coll CardiolHome page
A. N. DeMaria, O. Ben-Yehuda, J. J. Bax, G. K. Feld, B. H. Greenberg, W. Y.W. Lew, J. A.C. Lima, A. S. Maisel, S. M. Narayan, D. J. Sahn, et al.
Highlights of the Year in JACC 2008
J. Am. Coll. Cardiol., January 27, 2009; 53(4): 373 - 398.
[Full Text] [PDF]

Home page
 CirculationHome page
S. Viskin and A. Halkin
Treating the Long-QT Syndrome in the Era of Implantable Defibrillators
Circulation, January 20, 2009; 119(2): 204 - 206.
[Full Text] [PDF]

Home page
 ESC Textbook of Cardiovascular MedicineHome page
F. G. Cosío, J. &#x. ; Palacios, A. &#x. ;n. Pastor, and A. Núñez
CHAPTER 2 The Electrocardiogram
ESC Textbook of Cardiovascular Medicine, January 1, 2009; 2(1): med-9780199566990-chapter - med-9780199566990-chapter.
[Abstract] [Full Text] [PDF]

Home page
 J Am Coll CardiolHome page
J. R. Kapoor
Inherited Long QT Syndromes: Be Mindful of the Potassium Level
J. Am. Coll. Cardiol., November 4, 2008; 52(19): 1605 - 1605.
[Full Text] [PDF]

Home page
 J Am Coll CardiolHome page
I. Goldenberg and A. J. Moss
Reply
J. Am. Coll. Cardiol., November 4, 2008; 52(19): 1605 - 1606.
[Full Text] [PDF]


 
  CME Topic Collections Past Issues Search Current Issue Home

Advertisement