Genotype-specific onset of arrhythmias in congenital long-QT syndrome: possible therapy implications.

Genetic testing for long-QT syndrome: distinguishing pathogenic mutations from benign variants. Circulation (2009) 3.11

The response of the QT interval to the brief tachycardia provoked by standing: a bedside test for diagnosing long QT syndrome. J Am Coll Cardiol (2010) 2.56

Identification of a possible pathogenic link between congenital long QT syndrome and epilepsy. Neurology (2008) 2.21

Risk of recurrent cardiac events after onset of menopause in women with congenital long-QT syndrome types 1 and 2. Circulation (2011) 1.41

Isogenic human pluripotent stem cell pairs reveal the role of a KCNH2 mutation in long-QT syndrome. EMBO J (2013) 1.24

Differential conditions for early after-depolarizations and triggered activity in cardiomyocytes derived from transgenic LQT1 and LQT2 rabbits. J Physiol (2011) 1.21

Pharmacological approach to the treatment of long and short QT syndromes. Pharmacol Ther (2008) 1.09

Autonomic modulation and antiarrhythmic therapy in a model of long QT syndrome type 3. Cardiovasc Res (2010) 1.03

Long QT syndrome: beyond the causal mutation. J Physiol (2013) 1.00

Cardiac potassium channel dysfunction in sudden infant death syndrome. J Mol Cell Cardiol (2007) 0.96

Short-coupled variant of torsade de pointes. Neth Heart J (2008) 0.93

Genotype- and Sex-Specific QT-RR Relationship in the Type-1 Long-QT Syndrome. J Am Heart Assoc (2012) 0.92

Congenital long-QT syndromes: a clinical and genetic update from infancy through adulthood. Trends Cardiovasc Med (2008) 0.92

Impact of gene patents and licensing practices on access to genetic testing for long QT syndrome. Genet Med (2010) 0.92

Diastolic transient inward current in long QT syndrome type 3 is caused by Ca2+ overload and inhibited by ranolazine. J Mol Cell Cardiol (2009) 0.90

The Electro-Mechanical window: a risk marker for Torsade de Pointes in a canine model of drug induced arrhythmias. Br J Pharmacol (2010) 0.88

Evolutionary analyses of KCNQ1 and HERG voltage-gated potassium channel sequences reveal location-specific susceptibility and augmented chemical severities of arrhythmogenic mutations. BMC Evol Biol (2008) 0.85

Congenital Long QT Syndrome: An Update and Present Perspective in Saudi Arabia. Front Pediatr (2013) 0.81

Sodium overload due to a persistent current that attenuates the arrhythmogenic potential of a novel LQT3 mutation. Front Pharmacol (2013) 0.80

Recurrent and Founder Mutations in the Netherlands: the Long-QT Syndrome. Neth Heart J (2010) 0.80

Short and long QT syndromes: does QT length really matter? J Electrocardiol (2010) 0.80

Complex excitation dynamics underlie polymorphic ventricular tachycardia in a transgenic rabbit model of long QT syndrome type 1. Heart Rhythm (2014) 0.79

Vasovagal syncope or ventricular fibrillation. Your diagnosis better be accurate. Clin Auton Res (2007) 0.77

Measure of the QT-RR dynamic coupling in patients with the long QT syndrome. Ann Noninvasive Electrocardiol (2012) 0.75

Contemporary definitions and classification of the cardiomyopathies: an American Heart Association Scientific Statement from the Council on Clinical Cardiology, Heart Failure and Transplantation Committee; Quality of Care and Outcomes Research and Functional Genomics and Translational Biology Interdisciplinary Working Groups; and Council on Epidemiology and Prevention. Circulation (2006) 9.44

Brugada syndrome: report of the second consensus conference: endorsed by the Heart Rhythm Society and the European Heart Rhythm Association. Circulation (2005) 8.76

ACC/AHA/ESC 2006 Guidelines for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death: a report of the American College of Cardiology/American Heart Association Task Force and the European Society of Cardiology Committee for Practice Guidelines (writing committee to develop Guidelines for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death): developed in collaboration with the European Heart Rhythm Association and the Heart Rhythm Society. Circulation (2006) 5.95

ACC/AHA/ESC 2006 guidelines for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death: a report of the American College of Cardiology/American Heart Association Task Force and the European Society of Cardiology Committee for Practice Guidelines (Writing Committee to Develop Guidelines for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death). J Am Coll Cardiol (2006) 5.81

An international compendium of mutations in the SCN5A-encoded cardiac sodium channel in patients referred for Brugada syndrome genetic testing. Heart Rhythm (2009) 4.99

Interleukin-6 receptor pathways in coronary heart disease: a collaborative meta-analysis of 82 studies. Lancet (2012) 4.63

Inappropriate implantable cardioverter-defibrillator shocks in MADIT II: frequency, mechanisms, predictors, and survival impact. J Am Coll Cardiol (2008) 4.63

HRS/EHRA/APHRS expert consensus statement on the diagnosis and management of patients with inherited primary arrhythmia syndromes: document endorsed by HRS, EHRA, and APHRS in May 2013 and by ACCF, AHA, PACES, and AEPC in June 2013. Heart Rhythm (2013) 4.62

HRS/EHRA expert consensus statement on the state of genetic testing for the channelopathies and cardiomyopathies this document was developed as a partnership between the Heart Rhythm Society (HRS) and the European Heart Rhythm Association (EHRA). Heart Rhythm (2011) 4.31

Long QT syndrome. J Am Coll Cardiol (2008) 4.08

Mutations in the desmosomal protein plakophilin-2 are common in arrhythmogenic right ventricular cardiomyopathy. Nat Genet (2004) 3.88

Survival with cardiac-resynchronization therapy in mild heart failure. N Engl J Med (2014) 3.80

Time dependence of mortality risk and defibrillator benefit after myocardial infarction. Circulation (2004) 3.78

Risk stratification for primary implantation of a cardioverter-defibrillator in patients with ischemic left ventricular dysfunction. J Am Coll Cardiol (2008) 3.73

Plakophilin-2 mutations are the major determinant of familial arrhythmogenic right ventricular dysplasia/cardiomyopathy. Circulation (2006) 3.44

Flecainide prevents catecholaminergic polymorphic ventricular tachycardia in mice and humans. Nat Med (2009) 3.35

Right ventricular fibrosis and conduction delay in a patient with clinical signs of Brugada syndrome: a combined electrophysiological, genetic, histopathologic, and computational study. Circulation (2005) 3.30

Genetic testing for long-QT syndrome: distinguishing pathogenic mutations from benign variants. Circulation (2009) 3.11

ACC/AHA/ESC 2006 guidelines for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death: a report of the American College of Cardiology/American Heart Association Task Force and the European Society of Cardiology Committee for Practice Guidelines (Writing Committee to Develop guidelines for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death) developed in collaboration with the European Heart Rhythm Association and the Heart Rhythm Society. Europace (2006) 3.11

Implantable cardioverter defibrillator in high-risk long QT syndrome patients. J Cardiovasc Electrophysiol (2003) 3.05

Allelic variants in long-QT disease genes in patients with drug-associated torsades de pointes. Circulation (2002) 2.98

Long QT syndrome in adults. J Am Coll Cardiol (2007) 2.93

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

Thoracoscopic video-assisted pulmonary vein antrum isolation, ganglionated plexus ablation, and periprocedural confirmation of ablation lesions: first results of a hybrid surgical-electrophysiological approach for atrial fibrillation. Circ Arrhythm Electrophysiol (2011) 2.84

Cardiac resynchronization therapy is more effective in women than in men: the MADIT-CRT (Multicenter Automatic Defibrillator Implantation Trial with Cardiac Resynchronization Therapy) trial. J Am Coll Cardiol (2011) 2.84

Mutation in the KCNQ1 gene leading to the short QT-interval syndrome. Circulation (2004) 2.82

Spectrum and prevalence of mutations from the first 2,500 consecutive unrelated patients referred for the FAMILION long QT syndrome genetic test. Heart Rhythm (2009) 2.81

Common sodium channel promoter haplotype in asian subjects underlies variability in cardiac conduction. Circulation (2006) 2.72

Genetic variations of KCNQ1, KCNH2, SCN5A, KCNE1, and KCNE2 in drug-induced long QT syndrome patients. J Mol Med (Berl) (2004) 2.68

Impact of carvedilol and metoprolol on inappropriate implantable cardioverter-defibrillator therapy: the MADIT-CRT trial (Multicenter Automatic Defibrillator Implantation With Cardiac Resynchronization Therapy). J Am Coll Cardiol (2013) 2.68

Absence of calsequestrin 2 causes severe forms of catecholaminergic polymorphic ventricular tachycardia. Circ Res (2002) 2.66

Genotype-phenotype aspects of type 2 long QT syndrome. J Am Coll Cardiol (2009) 2.65

Use of a wearable defibrillator in terminating tachyarrhythmias in patients at high risk for sudden death: results of the WEARIT/BIROAD. Pacing Clin Electrophysiol (2004) 2.63

Familial sudden death is an important risk factor for primary ventricular fibrillation: a case-control study in acute myocardial infarction patients. Circulation (2006) 2.63

Left cardiac sympathetic denervation in the management of high-risk patients affected by the long-QT syndrome. Circulation (2004) 2.63

The Jervell and Lange-Nielsen syndrome: natural history, molecular basis, and clinical outcome. Circulation (2006) 2.58

Genotype-phenotype relationship in Brugada syndrome: electrocardiographic features differentiate SCN5A-related patients from non-SCN5A-related patients. J Am Coll Cardiol (2002) 2.58

The response of the QT interval to the brief tachycardia provoked by standing: a bedside test for diagnosing long QT syndrome. J Am Coll Cardiol (2010) 2.56

Effectiveness of Cardiac Resynchronization Therapy by QRS Morphology in the Multicenter Automatic Defibrillator Implantation Trial-Cardiac Resynchronization Therapy (MADIT-CRT). Circulation (2011) 2.56

Diagnostic criteria for congenital long QT syndrome in the era of molecular genetics: do we need a scoring system? Eur Heart J (2006) 2.54

Sodium channel β1 subunit mutations associated with Brugada syndrome and cardiac conduction disease in humans. J Clin Invest (2008) 2.54

Impaired endocytosis of the ion channel TRPM4 is associated with human progressive familial heart block type I. J Clin Invest (2009) 2.54

HRS/EHRA expert consensus statement on the state of genetic testing for the channelopathies and cardiomyopathies: this document was developed as a partnership between the Heart Rhythm Society (HRS) and the European Heart Rhythm Association (EHRA). Europace (2011) 2.54

ACC/AHA/ESC 2006 guidelines for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death--executive summary: A report of the American College of Cardiology/American Heart Association Task Force and the European Society of Cardiology Committee for Practice Guidelines (Writing Committee to Develop Guidelines for Management of Patients with Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death) Developed in collaboration with the European Heart Rhythm Association and the Heart Rhythm Society. Eur Heart J (2006) 2.54

Brugada syndrome: report of the second consensus conference. Heart Rhythm (2005) 2.48

Sudden unexplained death: heritability and diagnostic yield of cardiological and genetic examination in surviving relatives. Circulation (2005) 2.42

Risk factors for aborted cardiac arrest and sudden cardiac death in children with the congenital long-QT syndrome. Circulation (2008) 2.40

A gain-of-function TBX5 mutation is associated with atypical Holt-Oram syndrome and paroxysmal atrial fibrillation. Circ Res (2008) 2.39

Time dependence of defibrillator benefit after coronary revascularization in the Multicenter Automatic Defibrillator Implantation Trial (MADIT)-II. J Am Coll Cardiol (2006) 2.32

Distinguishing arrhythmogenic right ventricular cardiomyopathy/dysplasia-associated mutations from background genetic noise. J Am Coll Cardiol (2011) 2.31

Predictors of super-response to cardiac resynchronization therapy and associated improvement in clinical outcome: the MADIT-CRT (multicenter automatic defibrillator implantation trial with cardiac resynchronization therapy) study. J Am Coll Cardiol (2012) 2.31

Genetic variation in SCN10A influences cardiac conduction. Nat Genet (2010) 2.30

Unmasking of brugada syndrome by lithium. Circulation (2005) 2.30

Impact of genetics on the clinical management of channelopathies. J Am Coll Cardiol (2013) 2.30

Clinical aspects and prognosis of Brugada syndrome in children. Circulation (2007) 2.29

Risk factors for malignant ventricular arrhythmias in lamin a/c mutation carriers a European cohort study. J Am Coll Cardiol (2012) 2.28

Left ventricular lead position and clinical outcome in the multicenter automatic defibrillator implantation trial-cardiac resynchronization therapy (MADIT-CRT) trial. Circulation (2011) 2.26

Modulating effects of age and gender on the clinical course of long QT syndrome by genotype. J Am Coll Cardiol (2003) 2.22

Survival benefit of primary prevention implantable cardioverter-defibrillator therapy after myocardial infarction: does time to implant matter? A meta-analysis using patient-level data from 4 clinical trials. Heart Rhythm (2013) 2.22

A mutation in the human cardiac sodium channel (E161K) contributes to sick sinus syndrome, conduction disease and Brugada syndrome in two families. J Mol Cell Cardiol (2005) 2.21

Ventricular fibrillation with prominent early repolarization associated with a rare variant of KCNJ8/KATP channel. J Cardiovasc Electrophysiol (2009) 2.20

Cellular mechanisms underlying the long QT syndrome. Curr Opin Cardiol (2002) 2.19

ECGScan: a method for conversion of paper electrocardiographic printouts to digital electrocardiographic files. J Electrocardiol (2005) 2.17

Genetic and biophysical basis of sudden unexplained nocturnal death syndrome (SUNDS), a disease allelic to Brugada syndrome. Hum Mol Genet (2002) 2.16

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

A cardiac sodium channel mutation cosegregates with a rare connexin40 genotype in familial atrial standstill. Circ Res (2003) 2.15

Contribution of sodium channel mutations to bradycardia and sinus node dysfunction in LQT3 families. Circ Res (2003) 2.14

Mechanisms of ventricular fibrillation initiation in MADIT II patients with implantable cardioverter defibrillators. Pacing Clin Electrophysiol (2008) 2.13

Inaccessibility to quinidine therapy is about to get worse. J Am Coll Cardiol (2013) 2.12

Risk of aborted cardiac arrest or sudden cardiac death during adolescence in the long-QT syndrome. JAMA (2006) 2.12

Activation delay and VT parameters in arrhythmogenic right ventricular dysplasia/cardiomyopathy: toward improvement of diagnostic ECG criteria. J Cardiovasc Electrophysiol (2008) 2.09

Predictors of response to cardiac resynchronization therapy in the Multicenter Automatic Defibrillator Implantation Trial with Cardiac Resynchronization Therapy (MADIT-CRT). Circulation (2011) 2.08

Pathophysiological mechanisms of Brugada syndrome: depolarization disorder, repolarization disorder, or more? Cardiovasc Res (2005) 2.06

Active cascade screening in primary inherited arrhythmia syndromes: does it lead to prophylactic treatment? J Am Coll Cardiol (2010) 2.06

Effect of metoprolol versus carvedilol on outcomes in MADIT-CRT (multicenter automatic defibrillator implantation trial with cardiac resynchronization therapy). J Am Coll Cardiol (2013) 2.05

Arrhythmogenic right ventricular dysplasia/cardiomyopathy: pathogenic desmosome mutations in index-patients predict outcome of family screening: Dutch arrhythmogenic right ventricular dysplasia/cardiomyopathy genotype-phenotype follow-up study. Circulation (2011) 2.04

Predictive value of ventricular arrhythmia inducibility for subsequent ventricular tachycardia or ventricular fibrillation in Multicenter Automatic Defibrillator Implantation Trial (MADIT) II patients. J Am Coll Cardiol (2005) 2.03

Participation of a concealed atriohisian tract in the reentrant circuit of the slow-fast type of atrioventricular nodal reentrant tachycardia. Heart Rhythm (2007) 2.03

Brugada syndrome. Circ Arrhythm Electrophysiol (2012) 2.03

Con: Newborn screening to prevent sudden cardiac death? Heart Rhythm (2006) 2.02

Impact of onsite or dispatched automated external defibrillator use on survival after out-of-hospital cardiac arrest. Circulation (2011) 2.01

Long QT syndrome with compound mutations is associated with a more severe phenotype: a Japanese multicenter study. Heart Rhythm (2010) 2.00

Role of programmed ventricular stimulation in patients with Brugada syndrome: a meta-analysis of worldwide published data. Eur Heart J (2007) 1.98

Prolonged atrial action potential durations and polymorphic atrial tachyarrhythmias in patients with long QT syndrome. J Cardiovasc Electrophysiol (2003) 1.98

Executive summary: HRS/EHRA/APHRS expert consensus statement on the diagnosis and management of patients with inherited primary arrhythmia syndromes. Europace (2013) 1.97