Long QT Syndrome-Population Genetics and Cardiac Studies (LQTS)

Clinical Trial ID NCT00005176

PubWeight™ 89.78‹?›

🔗 Visit the ClinicalTrials.gov page for NCT00005176

Top papers

Rank Title Journal Year PubWeight™‹?›
1 SCN5A mutations associated with an inherited cardiac arrhythmia, long QT syndrome. Cell 1995 7.73
2 Spectrum of mutations in long-QT syndrome genes. KVLQT1, HERG, SCN5A, KCNE1, and KCNE2. Circulation 2000 7.50
3 Genotype-phenotype correlation in the long-QT syndrome: gene-specific triggers for life-threatening arrhythmias. Circulation 2001 7.47
4 Effectiveness and limitations of beta-blocker therapy in congenital long-QT syndrome. Circulation 2000 5.09
5 Long QT syndrome patients with mutations of the SCN5A and HERG genes have differential responses to Na+ channel blockade and to increases in heart rate. Implications for gene-specific therapy. Circulation 1995 4.48
6 Diagnostic criteria for the long QT syndrome. An update. Circulation 1993 4.48
7 Long QT syndrome. J Am Coll Cardiol 2008 4.08
8 The long QT syndrome. Prospective longitudinal study of 328 families. Circulation 1991 3.99
9 Influence of genotype on the clinical course of the long-QT syndrome. International Long-QT Syndrome Registry Research Group. N Engl J Med 1998 3.41
10 Implantable cardioverter defibrillator in high-risk long QT syndrome patients. J Cardiovasc Electrophysiol 2003 3.05
11 Spectrum of ST-T-wave patterns and repolarization parameters in congenital long-QT syndrome: ECG findings identify genotypes. Circulation 2000 2.95
12 Clinical aspects of type-1 long-QT syndrome by location, coding type, and biophysical function of mutations involving the KCNQ1 gene. Circulation 2007 2.88
13 Left cardiac sympathetic denervation in the therapy of congenital long QT syndrome. A worldwide report. Circulation 1991 2.87
14 Long QT Syndrome. JAMA 2003 2.70
15 Genotype-phenotype aspects of type 2 long QT syndrome. J Am Coll Cardiol 2009 2.65
16 Left cardiac sympathetic denervation in the management of high-risk patients affected by the long-QT syndrome. Circulation 2004 2.63
17 Age- and sex-related differences in clinical manifestations in patients with congenital long-QT syndrome: findings from the International LQTS Registry. Circulation 1998 2.53
18 Risk factors for aborted cardiac arrest and sudden cardiac death in children with the congenital long-QT syndrome. Circulation 2008 2.40
19 Modulating effects of age and gender on the clinical course of long QT syndrome by genotype. J Am Coll Cardiol 2003 2.22
20 Risk for life-threatening cardiac events in patients with genotype-confirmed long-QT syndrome and normal-range corrected QT intervals. J Am Coll Cardiol 2011 2.15
21 Risk of aborted cardiac arrest or sudden cardiac death during adolescence in the long-QT syndrome. JAMA 2006 2.12
22 Measurement of the QT interval and the risk associated with QTc interval prolongation: a review. Am J Cardiol 1993 2.01
23 Beta-blocker efficacy in high-risk patients with the congenital long-QT syndrome types 1 and 2: implications for patient management. J Cardiovasc Electrophysiol 2010 1.99
24 Location of mutation in the KCNQ1 and phenotypic presentation of long QT syndrome. J Cardiovasc Electrophysiol 2003 1.91
25 Clinical course and risk stratification of patients affected with the Jervell and Lange-Nielsen syndrome. J Cardiovasc Electrophysiol 2006 1.88
26 Relation between ventricular repolarization duration and cardiac cycle length during 24-hour Holter recordings. Findings in normal patients and patients with long QT syndrome. Circulation 1992 1.86
27 Clinical implications for patients with long QT syndrome who experience a cardiac event during infancy. J Am Coll Cardiol 2009 1.81
28 Long-QT syndrome after age 40. Circulation 2008 1.73
29 Long QT syndrome and pregnancy. J Am Coll Cardiol 2007 1.71
30 Long QT Syndrome. Curr Probl Cardiol 2008 1.70
31 Electrocardiographic quantitation of ventricular repolarization. Circulation 1989 1.65
32 Risk of death in the long QT syndrome when a sibling has died. Heart Rhythm 2008 1.64
33 Long QT syndrome. New electrocardiographic characteristics. Circulation 1990 1.60
34 Risk factors for recurrent syncope and subsequent fatal or near-fatal events in children and adolescents with long QT syndrome. J Am Coll Cardiol 2011 1.59
35 The common long-QT syndrome mutation KCNQ1/A341V causes unusually severe clinical manifestations in patients with different ethnic backgrounds: toward a mutation-specific risk stratification. Circulation 2007 1.58
36 Two long QT syndrome loci map to chromosomes 3 and 7 with evidence for further heterogeneity. Nat Genet 1994 1.55
37 Repolarization morphology in adult LQT2 carriers with borderline prolonged QTc interval. Heart Rhythm 2006 1.54
38 Mutation and gender-specific risk in type 2 long QT syndrome: implications for risk stratification for life-threatening cardiac events in patients with long QT syndrome. Heart Rhythm 2011 1.54
39 New mutations in the KVLQT1 potassium channel that cause long-QT syndrome. Circulation 1998 1.53
40 Corrected QT variability in serial electrocardiograms in long QT syndrome: the importance of the maximum corrected QT for risk stratification. J Am Coll Cardiol 2006 1.48
41 Effects of flecainide in patients with new SCN5A mutation: mutation-specific therapy for long-QT syndrome? Circulation 2000 1.46
42 Genotype-specific onset of arrhythmias in congenital long-QT syndrome: possible therapy implications. Circulation 2006 1.42
43 Risk of syncope in family members who are genotype-negative for a family-associated long-QT syndrome mutation. Circ Cardiovasc Genet 2011 1.40
44 Influence of pregnancy on the risk for cardiac events in patients with hereditary long QT syndrome. LQTS Investigators. Circulation 1998 1.39
45 Dental treatment of patients with long QT syndrome. J Am Dent Assoc 2006 1.38
46 Normalization of ventricular repolarization with flecainide in long QT syndrome patients with SCN5A:DeltaKPQ mutation. Ann Noninvasive Electrocardiol 2001 1.33
47 Prolonged QT-interval syndromes. JAMA 1986 1.31
48 Comparison of clinical and genetic variables of cardiac events associated with loud noise versus swimming among subjects with the long QT syndrome. Am J Cardiol 1999 1.25
49 alpha-1-syntrophin mutation and the long-QT syndrome: a disease of sodium channel disruption. Circ Arrhythm Electrophysiol 2008 1.19
50 Efficacy of permanent pacing in the management of high-risk patients with long QT syndrome. Circulation 1991 1.15
51 Impaired T-amplitude adaptation to heart rate characterizes I(Kr) inhibition in the congenital and acquired forms of the long QT syndrome. J Cardiovasc Electrophysiol 2007 1.12
52 Defective human Ether-à-go-go-related gene trafficking linked to an endoplasmic reticulum retention signal in the C terminus. J Biol Chem 2002 1.12
53 Unsuspected echocardiographic abnormality in the long QT syndrome. Diagnostic, prognostic, and pathogenetic implications. Circulation 1991 1.09
54 Genotype-specific ECG patterns in long QT syndrome. J Electrocardiol 2006 1.08
55 T wave alternans in idiopathic long QT syndrome. J Am Coll Cardiol 1994 1.08
56 Risk of fatal arrhythmic events in long QT syndrome patients after syncope. J Am Coll Cardiol 2010 1.06
57 Effect of calcium channel block on the wall motion abnormality of the idiopathic long QT syndrome. Circulation 1994 1.04
58 Mapping of body surface potentials in patients with the idiopathic long QT syndrome. Circulation 1986 1.03
59 The long-QT syndrome Genetic considerations. Trends Cardiovasc Med 2011 1.00
60 Risk of cardiac events in family members of patients with long QT syndrome. J Am Coll Cardiol 1995 1.00
61 A quantitative assessment of T-wave morphology in LQT1, LQT2, and healthy individuals based on Holter recording technology. Heart Rhythm 2007 0.98
62 Long QT syndrome in patients over 40 years of age: increased risk for LQTS-related cardiac events in patients with coronary disease. Ann Noninvasive Electrocardiol 2008 0.98
63 25th anniversary of the International Long-QT Syndrome Registry: an ongoing quest to uncover the secrets of long-QT syndrome. Circulation 2005 0.97
64 Stress and sudden death. The case of the long QT syndrome. Circulation 1991 0.96
65 Novel compound heterozygous mutations in the KCNQ1 gene associated with autosomal recessive long QT syndrome (Jervell and Lange-Nielsen syndrome). Ann Noninvasive Electrocardiol 2003 0.95
66 Compound heterozygous mutations in KvLQT1 cause Jervell and Lange-Nielsen syndrome. Mol Genet Metab 2002 0.95
67 Trigger-specific risk factors and response to therapy in long QT syndrome type 2. Heart Rhythm 2010 0.94
68 T-wave morphology abnormalities in benign, potent, and arrhythmogenic I(kr) inhibition. Heart Rhythm 2011 0.93
69 Risk of cardiac events in patients with asthma and long-QT syndrome treated with beta(2) agonists. Am J Cardiol 2008 0.93
70 Quantitative analysis of T wave abnormalities and their prognostic implications in the idiopathic long QT syndrome. J Am Coll Cardiol 1994 0.93
71 Clinical implications for affected parents and siblings of probands with long-QT syndrome. Circulation 2001 0.92
72 Variable expression of long QT syndrome among gene carriers from families with five different HERG mutations. Ann Noninvasive Electrocardiol 2002 0.92
73 Clinical and genetic variables associated with acute arousal and nonarousal-related cardiac events among subjects with long QT syndrome. Am J Cardiol 2000 0.92
74 Trigger-specific ion-channel mechanisms, risk factors, and response to therapy in type 1 long QT syndrome. Heart Rhythm 2011 0.92
75 Restoration of conduction and growth of axons through injured spinal cord of neonatal opossum in culture. Proc Natl Acad Sci U S A 1992 0.91
76 Long QT syndrome and short QT syndrome. Prog Cardiovasc Dis 2008 0.91
77 Management of patients with the hereditary long QT syndrome. J Cardiovasc Electrophysiol 1998 0.89
78 Identification of a new SCN5A mutation, D1840G, associated with the long QT syndrome. Mutations in brief no. 153. Online. Hum Mutat 1998 0.88
79 Clinical features of the idiopathic long QT syndrome. Circulation 1992 0.88
80 Beat-to-Beat repolarization variability in LQTS patients with the SCN5A sodium channel gene mutation. Pacing Clin Electrophysiol 1999 0.87
81 Dispersion of ventricular repolarization in the long QT syndrome. Am J Cardiol 1991 0.87
82 Asthma and the risk of cardiac events in the Long QT syndrome. Long QT Syndrome Investigative Group. Am J Cardiol 1999 0.87
83 Analysis of HLA and disease susceptibility: chromosome 6 genes and sex influence long-QT phenotype. Am J Hum Genet 1994 0.84
84 LQTS gene LOVD database. Hum Mutat 2010 0.84
85 T-wave patterns associated with the hereditary long QT syndrome. Card Electrophysiol Rev 2002 0.84
86 Long QT syndrome. Heart Dis Stroke 1994 0.84
87 Long QT syndrome in African-Americans. Ann Noninvasive Electrocardiol 2010 0.84
88 Locus heterogeneity of autosomal dominant long QT syndrome. J Clin Invest 1993 0.84
89 Sudden cardiac death without structural heart disease: update on the long QT and Brugada syndromes. Curr Cardiol Rep 2005 0.82
90 ECG features of microvolt T-wave alternans in coronary artery disease and long QT syndrome patients. J Electrocardiol 1998 0.81
91 Happiness and stress alter susceptibility to cardiac events in Long QT Syndrome. Ann Noninvasive Electrocardiol 2009 0.81
92 Homozygous deletion in KVLQT1 associated with Jervell and Lange-Nielsen syndrome. Circulation 1999 0.80
93 Clinical aspects of the idiopathic long QT syndrome. Ann N Y Acad Sci 1992 0.80
94 Cost-effectiveness of implanted defibrillators in young people with inherited cardiac arrhythmias. Ann Noninvasive Electrocardiol 2005 0.79
95 Long QT Syndromes. Curr Treat Options Cardiovasc Med 2000 0.79
96 Counting mRNA in blood of LQTS - new direction? Kardiol Pol 2011 0.78
97 Heart rate variability in patients with congenital long QT syndrome. Ann Noninvasive Electrocardiol 2001 0.78
98 Clinical management of patients with the long QT syndrome: drugs, devices, and gene-specific therapy. Pacing Clin Electrophysiol 1997 0.78
99 Ion channel mechanisms related to sudden cardiac death in phenotype-negative long-QT syndrome genotype-phenotype correlations of the KCNQ1(S349W) mutation. J Cardiovasc Electrophysiol 2011 0.78
100 Genotype-specific QT correction for heart rate and the risk of life-threatening cardiac events in adolescents with congenital long-QT syndrome. Heart Rhythm 2011 0.78
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