|1.||Torsades de Pointes (Torsade de Pointes)
|3.||Cardiac Arrhythmias (Arrythmia)
|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)|
|1.||Sodium Channels (Sodium Channel)IBA
09/16/1997 - "This study examines the contribution of transmural heterogeneity of transmembrane activity to phenotypic T-wave patterns and the effects of pacing and of sodium channel block under conditions mimicking HERG and SCN5A defects linked to the congenital long-QT syndrome (LQTS). "
07/01/2014 - "A missense mutation in the sodium channel β1b subunit reveals SCN1B as a susceptibility gene underlying long QT syndrome."
05/10/2013 - "Novel deletion mutation in the cardiac sodium channel inactivation gate causes long QT syndrome."
03/01/2013 - "To construct the sodium channel gene SCN5A-delQKP1507-1509 mutation associated with congenital long QT syndrome, and its eukaryotic expression vector, and to examine the expression of mutation protein in human embryonic kidney (HEK) 293 cells. "
01/01/2013 - "A heterozygous deletion mutation in the cardiac sodium channel gene SCN5A with loss- and gain-of-function characteristics manifests as isolated conduction disease, without signs of Brugada or long QT syndrome."
|2.||Ion Channels (Ion Channel)IBA
01/01/1999 - "Genetic studies have identified four forms of congenital long QT syndrome (LQTS) caused by mutations in ion channel genes located on chromosomes 3 (LQT3), 7 (LQT2), 11 (LQT1), and 21 (LQT5). "
12/15/2015 - "We report that human KCNQ1 Long QT syndrome disease alleles regulate renal ciliogenesis; KCNQ1-p.R518X, -p.A178T and -p.K362R could not rescue ciliogenesis after Kcnq1-siRNA-mediated depletion in contrast to wild-type KCNQ1 and benign KCNQ1-p.R518Q, suggesting that the ion channel function of KCNQ1 regulates ciliogenesis. "
08/01/2015 - "Long QT syndrome (LQTS) is caused by a change in cardiac repolarization due to functional ion channel dysfunction which is associated with an elongation of the QT interval (hence the name) in the electrocardiogram and a predisposition to cardiac rhythm disorders (e.g. "
08/01/2015 - "The long QT syndrome is caused by a change in cardiac repolarization due to functional ion channel defects. "
01/01/2015 - "Congenital ion channel disorders, including congenital long QT syndrome (LQTS), cause significant morbidity in pediatric patients. "
|3.||Polymorphic catecholergic ventricular tachycardiaIBA
03/01/2011 - "β-Blockers are often very effective in preventing arrhythmic episodes associated with catecholaminergic polymorphic ventricular tachycardia and some subtypes of long QT syndrome. "
01/01/2013 - "Family screening studies are able to identify a cause in up to 50% of cases-most commonly long QT syndrome (LQTS), Brugada and early repolarization syndrome, and catecholaminergic polymorphic ventricular tachycardia (CPVT) using standard clinical screening investigations including pharmacological challenge testing. "
03/01/2015 - "Patients with catecholaminergic polymorphic ventricular tachycardia (CPVT) and long-QT syndrome (LQTS) are susceptible to cardiac events during sympathetic nervous system activation. "
03/01/2015 - "Long QT syndrome was genetically proven in eight and catecholaminergic polymorphic ventricular tachycardia in five patients. "
04/01/2014 - "Long QT syndrome (LQTS) and catecholaminergic polymorphic ventricular tachycardia (CPVT) are electric diseases characterized by catecholamine-induced ventricular arrhythmias. "
|4.||Atenolol (Tenormin)FDA LinkGeneric
06/01/1990 - "Atenolol was effective in treating 4 of 10 patients with long QT syndrome with a dosage of approximately 1.5 mg/kg/day. "
01/01/2011 - "The data on the efficacy of atenolol for long-QT syndrome (LQTS) are controversial. "
06/01/1990 - "Twenty children and adolescents treated orally with atenolol for chronic paroxysmal ventricular tachycardia (n = 10) or Long QT Syndrome (n = 10) over a 5 year period were retrospectively evaluated to ascertain the efficacy of arrhythmia suppression, the effective dosage, the cardiovascular effects, and the incidence of adverse effects. "
09/01/2002 - "A 60-year-old woman who had been successfully treated with atenolol and cardiac pacing for hypertension and long QT syndrome developed recurrent episodes of palpitations and syncope. "
03/01/2006 - "Atenolol and metoprolol may be preferable for patients with type 1 and 2 long QT syndrome. "
|5.||Potassium Channels (Potassium Channel)IBA
12/31/2011 - "The main goal of this study was to examine the patient age and sex dependent expression of KCNQ1 and HERG genes that encode potassium channels responsible for the occurrence of long QT syndrome (LQTS). "
04/15/2008 - "The hERG potassium channel has recently been a matter of extensive studies both at experimental and computational levels, because of its possible involvement in the potentially lethal drug-induced long QT syndrome. "
02/01/2007 - "Here, we employed combined computation methods to study the specific binding of BeKm-1 peptide to the hERG potassium channel, which is an essential determinant of the long-QT syndrome. "
11/01/2015 - "The Mutation P.T613a in the Pore Helix of the Kv 11.1 Potassium Channel is Associated with Long QT Syndrome."
04/01/2015 - "Congenital long QT syndrome type 2 (abnormal hERG potassium channel) patients can develop flat, asymmetric, and notched T waves. "
|6.||Mexiletine (Mexitil)FDA LinkGeneric
08/01/2000 - "The purpose of this study was to test the potential utility of mexiletine for the treatment of drug-induced long QT syndrome in vivo. "
11/01/2008 - "Mexiletine may protect patients with long QT syndrome (LQTS) type 3 from arrhythmias. "
04/01/2006 - "The first attempt for a gene-specific therapy was made in 1995 with the use of mexiletine in long-QT syndrome (LQTS) patients with mutations in the SCN5A gene. "
08/03/2005 - "Antiarrhythmic potential of mexiletine in patients with congenital and acquired long-QT syndrome (LQTS) has been attributed to a reduction of transmural dispersion of repolarization (TDR). "
08/22/2003 - "Thus, mexiletine may become a promising pharmacological strategy against the drug-induced long QT syndrome."
|7.||DNA (Deoxyribonucleic Acid)IBA
04/01/2010 - "In 2002, DNA Sciences, Inc., "cleared the market" by sending cease-and-desist patent enforcement letters to university and reference laboratories offering long QT syndrome genetic testing. "
01/04/2011 - "DNA structural variants as genetic risk factors for the long QT syndrome."
06/15/2010 - "Letter by Kaufman regarding article, "Closer look at genetic testing in long-QT syndrome: will DNA diagnostics ever be enough?"."
11/03/2009 - "Closer look at genetic testing in long-QT syndrome: will DNA diagnostics ever be enough?"
08/01/2009 - "Using polymerase chain reaction, denaturing high-performance liquid chromatography, and direct DNA sequencing, comprehensive open-reading frame/splice mutational analysis of the 12 known long QT syndrome susceptibility genes was performed. "
01/01/2002 - "Studies of magnesium in congenital long QT syndrome."
01/01/2002 - "We studied the role of magnesium (Mg) in congenital long QT syndrome (LQTS). "
06/01/1994 - "Antiarrhythmic effects of intravenous magnesium in the patients who are not hypomagnesaemic were mainly demonstrated in the patients with acquired long QT syndrome. "
10/01/2004 - "Intravenous administration of magnesium sulphate (MgSO(4)) is a very effective and safe treatment for torsades de pointes (TdP) associated with acquired long QT syndrome (LQTS) in adults. "
10/01/2009 - "Fetal ventricular tachycardia secondary to long QT syndrome treated with maternal intravenous magnesium: case report and review of the literature."
|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
10/01/1992 - "The objective of this study was to review our current experience using a combination of beta-adrenergic blocking agents and long-term cardiac pacing to treat patients with the idiopathic long QT syndrome. "
02/10/1998 - "Prophylactic treatment with beta-adrenergic blockers should be continued during the pregnancy and postpartum intervals in probands with the long QT syndrome."
12/01/1995 - "A multivariate logistic regression model was used to evaluate the risk of cardiac events in 637 family members who were first- and second-degree relatives of 151 probands with the long QT syndrome and in a subset of 513 family members who were not receiving beta-adrenergic blocking agents. "
10/01/1992 - "Combined use of beta-adrenergic blocking agents and long-term cardiac pacing for patients with the long QT syndrome."
11/24/1998 - "Our data provide a mechanistic understanding of the cellular basis for the therapeutic actions of beta-adrenergic blockers in LQT1 and suggest that sodium channel block with class IB antiarrhythmic agents may be effective in suppressing TdP in LQT1, as they are in LQT2 and LQT3, as well as in acquired (drug-induced) forms of the long-QT syndrome."
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)
11/01/1996 - "Congenital long-QT syndrome: deleterious effect of long-term high-rate ventricular pacing and definitive treatment by cardiac transplantation."
03/11/1999 - "Pacing for long QT syndrome, pacing after cardiac transplantation, and pacing for hemodynamic improvement in the occasional patient with first-degree atrioventricular block and hemodynamic compromise also warrant attention. "
11/01/1990 - "[Treatment of tachyarrhythmia in a patient with long QT syndrome by autologous heart transplantation and atrial stimulation controlled by the sinoatrial node]."
09/15/1995 - "Five major areas were identified and searched: cardiomyopathies, atrial fibrillation, the long QT syndrome, cardiac transplantation, and vasovagal syncope. "
08/01/2013 - "Areas of variance included physical activity recommendations for hypertrophic cardiomyopathy, long QT syndrome, catecholaminergic polymorphic ventricular tachycardia, and heart transplantation, among others. "
12/01/2000 - "Pacemaker for long QT syndrome, after cardiac transplant and for haemodynamic improvement in occasional cases of first degree atrio-ventricular block is getting attention. "
12/01/2005 - "We present a 15-year-old boy with Long QT Syndrome who presented for an elective renal transplant. "
10/01/2014 - "The safety of modern anesthesia for children with long QT syndrome."
12/01/2011 - "Manifestation of Long QT syndrome with normal QTc interval under anesthesia: a case report."
05/01/2011 - "Implications of anesthesia in children with long QT syndrome."
10/01/2007 - "The long QT syndrome, possible cause of intra- and postoperative morbidity and mortality, can be related to drugs used during anesthesia. "
10/01/2007 - "[Anesthesia and the long QT syndrome.]."
|5.||Vagus Nerve Stimulation
08/01/2015 - "The aim of this study was to investigate the effect of vagus nerve stimulation (VNS) on the heterogeneity of ventricular repolarisation, as assessed using the T-wave peak-to-end interval (TpTe) and monophasic action potentials (MAPs), in normal hearts and in hearts with acquired long QT syndrome. "