PubRank

Isabelle Denjoy

Author PubWeight™ 61.32‹?›

Top papers

Rank Title Journal Year PubWeight™‹?›
1 High efficacy of beta-blockers in long-QT syndrome type 1: contribution of noncompliance and QT-prolonging drugs to the occurrence of beta-blocker treatment "failures". Circulation 2008 2.83
2 Absence of calsequestrin 2 causes severe forms of catecholaminergic polymorphic ventricular tachycardia. Circ Res 2002 2.66
3 The Jervell and Lange-Nielsen syndrome: natural history, molecular basis, and clinical outcome. Circulation 2006 2.58
4 Defining the cellular phenotype of "ankyrin-B syndrome" variants: human ANK2 variants associated with clinical phenotypes display a spectrum of activities in cardiomyocytes. Circulation 2007 2.38
5 Clinical aspects and prognosis of Brugada syndrome in children. Circulation 2007 2.29
6 Cardiac characteristics and long-term outcome in Andersen-Tawil syndrome patients related to KCNJ2 mutation. Europace 2013 2.14
7 Impact of the control of symptomatic paroxysmal atrial fibrillation on health-related quality of life. Europace 2010 2.12
8 Long-term follow-up of asymptomatic Brugada patients with inducible ventricular fibrillation under hydroquinidine. Europace 2013 2.02
9 MOG1: a new susceptibility gene for Brugada syndrome. Circ Cardiovasc Genet 2011 1.97
10 Incidence and risk factors of arrhythmic events in catecholaminergic polymorphic ventricular tachycardia. Circulation 2009 1.76
11 Dominant-negative effect of SCN5A N-terminal mutations through the interaction of Na(v)1.5 α-subunits. Cardiovasc Res 2012 1.74
12 R231C mutation in KCNQ1 causes long QT syndrome type 1 and familial atrial fibrillation. Heart Rhythm 2010 1.71
13 Absence of triadin, a protein of the calcium release complex, is responsible for cardiac arrhythmia with sudden death in human. Hum Mol Genet 2012 1.64
14 Safety of hydroxychloroquine in pregnant patients with connective tissue diseases: a study of one hundred thirty-three cases compared with a control group. Arthritis Rheum 2003 1.59
15 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
16 Female predominance and transmission distortion in the long-QT syndrome. N Engl J Med 2006 1.55
17 The role of stress test for predicting genetic mutations and future cardiac events in asymptomatic relatives of catecholaminergic polymorphic ventricular tachycardia probands. Europace 2012 1.54
18 A large candidate gene survey identifies the KCNE1 D85N polymorphism as a possible modulator of drug-induced torsades de pointes. Circ Cardiovasc Genet 2011 1.48
19 The effect of atropine on rhythm and conduction disturbances during 322 critical care intubations. Pediatr Crit Care Med 2013 1.44
20 Microvolt T-wave alternans in short QT syndrome. Pacing Clin Electrophysiol 2012 1.39
21 Who are the long-QT syndrome patients who receive an implantable cardioverter-defibrillator and what happens to them?: data from the European Long-QT Syndrome Implantable Cardioverter-Defibrillator (LQTS ICD) Registry. Circulation 2010 1.38
22 Long QT syndrome in neonates: conduction disorders associated with HERG mutations and sinus bradycardia with KCNQ1 mutations. J Am Coll Cardiol 2004 1.30
23 Novel calmodulin mutations associated with congenital arrhythmia susceptibility. Circ Cardiovasc Genet 2014 1.27
24 Hydroquinidine therapy in Brugada syndrome. J Am Coll Cardiol 2004 1.19
25 Catecholaminergic polymorphic ventricular tachycardia. Circ Arrhythm Electrophysiol 2012 1.12
26 Germline and somatic mosaicism for a mutation of the ryanodine receptor type 2 gene: implication for genetic counselling and patient caring. Europace 2010 1.11
27 Mutational spectrum in the Ca(2+)--activated cation channel gene TRPM4 in patients with cardiac conductance disturbances. Hum Mutat 2011 1.07
28 24-hour BP in children with congenital central hypoventilation syndrome. Chest 2003 1.06
29 Identification of a KCNQ1 polymorphism acting as a protective modifier against arrhythmic risk in long-QT syndrome. Circ Cardiovasc Genet 2013 1.03
30 Outcome of pregnancies in patients with anti-SSA/Ro antibodies: a study of 165 pregnancies, with special focus on electrocardiographic variations in the children and comparison with a control group. Arthritis Rheum 2004 1.01
31 Genotype- and Sex-Specific QT-RR Relationship in the Type-1 Long-QT Syndrome. J Am Heart Assoc 2012 0.92
32 New KCNQ1 mutations leading to haploinsufficiency in a general population; Defective trafficking of a KvLQT1 mutant. Cardiovasc Res 2004 0.92
33 Beat-to-beat T-wave amplitude variability in the long QT syndrome. Europace 2010 0.87
34 Gene-specific effect of beta-adrenergic blockade on corrected QT interval in the long QT syndrome. Ann Noninvasive Electrocardiol 2013 0.84
35 Short QT syndrome. Update on a recent entity. Arch Cardiovasc Dis 2008 0.83
36 Heart rate influences on repolarization duration and morphology in symptomatic versus asymptomatic KCNQ1 mutation carriers. Am J Cardiol 2005 0.82
37 Quantitative assessment of ST segment elevation in Brugada patients. Heart Rhythm 2006 0.81
38 Functional analysis reveals splicing mutations of the CASQ2 gene in patients with CPVT: implication for genetic counselling and clinical management. Hum Mutat 2011 0.81
39 Automatic analysis of cardiac repolarization morphology using Gaussian mesa function modeling. J Electrocardiol 2008 0.79
40 High-risk long QT syndrome mutations in the Kv7.1 (KCNQ1) pore disrupt the molecular basis for rapid K(+) permeation. Biochemistry 2012 0.79
41 Decreased nocturnal standard deviation of averaged NN intervals. An independent marker to identify patients at risk in the Brugada Syndrome. Eur Heart J 2003 0.78
42 Complex Brugada syndrome inheritance in a family harbouring compound SCN5A and CACNA1C mutations. Basic Res Cardiol 2014 0.78
43 A Common Mutation of Long QT Syndrome Type 1 in Japan. Circ J 2015 0.76
44 Advising a cardiac disease gene positive yet phenotype negative or borderline abnormal athlete: is sporting disqualification really necessary? Br J Sports Med 2012 0.76
45 Novel SCN5A mutations in two families with "Brugada-like" ST elevation in the inferior leads and conduction disturbances. J Interv Card Electrophysiol 2013 0.76
46 Effect of halofantrine on QT interval in children. Pathog Glob Health 2012 0.75
47 [Familial short QT syndrome]. Rev Prat 2007 0.75
48 The occurrence of Brugada syndrome and isolated cardiac conductive disease in the same family could be due to a single SCN5A mutation or to the accidental association of both diseases. Europace 2007 0.75
49 Spatiotemporal electrocardiographic characterization of ventricular depolarization and repolarization abnormalities in long QT syndrome. J Electrocardiol 2007 0.75
50 Electrocardiographic predictors of Brugada type response during Na channel blockade challenge. Europace 2005 0.75
51 Type 1 electrocardiographic burden is increased in symptomatic patients with Brugada syndrome. J Electrocardiol 2010 0.75