Long QT Syndrome

A condition that is characterized by episodes of fainting (SYNCOPE) and varying degree of ventricular arrhythmia as indicated by the prolonged QT interval. The inherited forms are caused by mutation of genes encoding cardiac ion channel proteins. The two major forms are ROMANO-WARD SYNDROME and JERVELL-LANGE NIELSEN SYNDROME.
Networked: 1251 relevant articles (25 outcomes, 64 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1. Torsades de Pointes (Torsade de Pointes)
2. Ventricular Tachycardia
3. Cardiac Arrhythmias (Arrythmia)
4. Ventricular Fibrillation
5. Atrioventricular Block


1. Ackerman, Michael J: 76 articles (08/2015 - 05/2002)
2. Tester, David J: 34 articles (08/2015 - 05/2002)
3. Wilde, Arthur A M: 27 articles (04/2014 - 05/2003)
4. Shimizu, Wataru: 25 articles (01/2015 - 02/2002)
5. Priori, Silvia G: 22 articles (04/2014 - 02/2002)
6. Moss, Arthur J: 19 articles (05/2013 - 01/2002)
7. Zareba, Wojciech: 18 articles (05/2013 - 01/2002)
8. Makielski, Jonathan C: 17 articles (08/2015 - 08/2002)
9. January, Craig T: 15 articles (12/2014 - 12/2002)
10. Schwartz, Peter J: 15 articles (08/2014 - 02/2002)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Long QT Syndrome:
1. Sodium Channels (Sodium Channel)IBA
2. Ion Channels (Ion Channel)IBA
3. Polymorphic catecholergic ventricular tachycardiaIBA
4. Atenolol (Tenormin)FDA LinkGeneric
5. Potassium Channels (Potassium Channel)IBA
6. Mexiletine (Mexitil)FDA LinkGeneric
7. DNA (Deoxyribonucleic Acid)IBA
8. MagnesiumIBA
9. Long QT syndrome type 1IBA
12/28/2006 - "Classic mendelian inheritance ratios were not observed in the offspring of either female carriers of the long-QT syndrome type 1 or male and female carriers of the long-QT syndrome type 2. Among the 1534 descendants, the proportion of genetically affected offspring was significantly greater than that expected according to mendelian inheritance: 870 were carriers of a mutation (57%), and 664 were noncarriers (43%, P<0.001). "
05/01/2013 - "The risk associated with QTcSD was pronounced among patients with long QT syndrome type 1 (hazard ratio 1.55 per 20 ms increment; P<.001), whereas among patients with long QT syndrome type 2, the risk associated with QTcSD was not statistically significant (hazard ratio 0.99; P = .95; P value for QTcSD-by-genotype interaction = .002). "
09/01/2010 - "We have generated transgenic rabbits lacking cardiac slow delayed-rectifier K(+) current [I(Ks); long QT syndrome type 1 (LQT1)] or rapidly activating delayed-rectifier K(+) current [I(Kr); long QT syndrome type 2 (LQT2)]. "
04/01/2013 - "CONCLUSIONS- In this study, we provide evidence that the recessive inheritance of a severe long-QT syndrome type 1 phenotype in the absence of an auditory phenotype may represent a more common pattern of long-QT syndrome inheritance than previously anticipated and that these cases should be treated as a higher-risk long-QT syndrome subset similar to their Jervell and Lange-Nielsen syndrome counterparts. "
06/01/2000 - "Long QT syndrome: ionic basis and arrhythmia mechanism in long QT syndrome type 1."
10. Adrenergic beta-Antagonists (beta-Adrenergic Blocking Agents)IBA

Therapies and Procedures

1. Sympathectomy (Sympathectomies)
12/01/1984 - "Efficacy of left cardiac sympathectomy in the treatment of patients with the long QT syndrome."
12/01/2008 - "The purpose of this study was to assess the feasibility and long-term effect of video-assisted thoracoscopic left cardiac sympathetic denervation for congenital long-QT syndrome. "
07/01/2005 - "The primary aim of the present study was to investigate the short-term effects of surgical left cardiac sympathetic denervation (LCSD) on the QT interval and heart rate in patients with congenital long QT syndrome (LQTS). "
08/01/1996 - "Next come reflections upon the current status of knowledge on the subject, combined with comments about remaining challenges and questions grouped as follows: (1) morphologic abnormalities of the cardiac conduction system and their pathophysiologic significance in the long QT syndromes; (2) cardioneuropathy in the pathogenesis of long QT syndromes; (3) variability of QT prolongation, including consideration of peripheral and central dysautonomic conditions with certain diagnostic and therapeutic implications; (4) some caveats about the popular use of left cervical sympathectomy to treat symptomatic individuals with long QT syndrome, emphasizing hazards for future electrical instability of the heart; (5) consideration of genetic influences in the long QT syndromes, a field holding great promise as well as fraught with many puzzling dilemmas; and (6) apoptosis and the pathogenesis of the long QT syndromes, based upon personal observations previously made with electron microscopic studies of the sinus node and now including new immunohistochemical evidence expanding the relevance of this novel view-point. "
10/15/2015 - "Renal sympathetic denervation: A potential therapeutic approach for long QT syndrome."
2. Heart Transplantation (Grafting, Heart)
3. Transplants (Transplant)
4. Anesthesia
5. Vagus Nerve Stimulation