Abstract:
INTRODUCTION:Beta-blockers are widely used to prevent the lethal cardiac events associated with the long QT syndrome (LQTS), especially in KCNQ1-related LQTS (LQT1) patients. Some LQT1 patients, however, are refractory to this therapy. METHODS AND RESULTS:Eighteen symptomatic LQTS patients (12 families) were genetically diagnosed as having heterozygous KCNQ1 variants and received beta-blocker therapy. Cardiac events recurred in 4 members (3 families) despite continued therapy during mean follow-up of 70 months. Three of these patients (2 families) had the same mutation [A341V (KCNQ1)]; and the other had R243H (KCNQ1). The latter patient took aprindine, which seemed to be responsible for the event. By functional assay using a heterologous mammalian expression system, we found that A341V (KCNQ1) is a loss-of-function type mutation (not dominant negative). Further genetic screening revealed that one A341V (KCNQ1) family cosegregated with S706C (KCNH2) and another with G144S (KCNJ2). Functional assay of the S706C (KCNH2) mutation was found to reduce the current density of expressed heterozygous KCNH2 channels with a positive shift (+8 mV) of the activation curve. Action potential simulation study was conducted based on the KYOTO model to estimate the influence of additional gene modifiers. In both models mimicking LQT1 plus 2 and LQT1 plus 7, the incidence of early afterdepolarization was increased compared with the LQT1 model under the setting of beta-adrenergic stimulation. CONCLUSION:Multiple mutations in different LQTS-related genes may modify clinical characteristics. Expanded gene survey may be required in LQT1 patients who are resistant to beta-blocker therapy.
journal_name
J Cardiovasc Electrophysioljournal_title
Journal of cardiovascular electrophysiologyauthors
Kobori A,Sarai N,Shimizu W,Nakamura Y,Murakami Y,Makiyama T,Ohno S,Takenaka K,Ninomiya T,Fujiwara Y,Matsuoka S,Takano M,Noma A,Kita T,Horie Mdoi
10.1046/j.1540-8167.2004.03212.xkeywords:
subject
Has Abstractpub_date
2004-02-01 00:00:00pages
190-9issue
2eissn
1045-3873issn
1540-8167pii
JCE03212journal_volume
15pub_type
杂志文章abstract:INTRODUCTION:Ventricular tachycardia (VT) after postinfarct ventricular septal defect (VSD) repair has not been well characterized. METHODS AND RESULTS:A 55-year-old man developed refractory VT after inferior wall infarction and VSD repair. Entrainment demonstrated a broad reentry circuit path (outer loop) between the...
journal_title:Journal of cardiovascular electrophysiology
pub_type: 杂志文章
doi:10.1111/j.1540-8167.1997.tb01003.x
更新日期:1997-10-01 00:00:00
abstract:INTRODUCTION:The current practice of percutaneous subxiphoid needle access to the "healthy" pericardial sac has significant limitations. We sought to examine the feasibility of a novel toolkit designed to improve this procedure. METHODS AND RESULTS:The toolkit included a pericardial access needle and a virtual imaging...
journal_title:Journal of cardiovascular electrophysiology
pub_type: 杂志文章
doi:10.1111/jce.12643
更新日期:2015-05-01 00:00:00
abstract:INTRODUCTION:It is the hypothesis of this article that the onset of fibrillation following a coronary artery occlusion is a direct consequence of the spatial inhomogeneity of chemical processes that occur following the occlusion. In particular, the localized increase of extracellular potassium and decrease of ATP avail...
journal_title:Journal of cardiovascular electrophysiology
pub_type: 杂志文章
doi:10.1046/j.1540-8167.2003.03045.x
更新日期:2003-11-01 00:00:00
abstract:INTRODUCTION:Following conversion of atrial fibrillation (AF), QT interval transiently and variably prolongs and can trigger torsades de pointes (TdP). However, quantitative analysis of risk in this setting is difficult because cycle length variability during AF makes rate-corrected QT impossible to calculate. In this ...
journal_title:Journal of cardiovascular electrophysiology
pub_type: 临床试验,杂志文章
doi:10.1111/j.1540-8167.2007.00817.x
更新日期:2007-06-01 00:00:00
abstract:INTRODUCTION:The aim of this study was to determine the characteristics of heart rate variability (HRV), blood pressure variability (BPV), and baroreflex gain (BRG) at rest and during orthostatic stress in patients with clinical criteria of inappropriate sinus tachycardia (IST). METHODS AND RESULTS:Beat-to-beat HRV an...
journal_title:Journal of cardiovascular electrophysiology
pub_type: 临床试验,杂志文章
doi:10.1046/j.1540-8167.2005.04441.x
更新日期:2005-01-01 00:00:00
abstract::Atrial fibrillation (AF) and congestive heart failure (CHF) are two increasingly common cardiac disorders with a growing prevalence in the overall population. Improved treatment of acute medical conditions has increased the incidence of these cardiac disorders. AF and CHF have similar epidemiologic characteristics and...
journal_title:Journal of cardiovascular electrophysiology
pub_type: 杂志文章,评审
doi:10.1046/j.1540-8167.2002.00399.x
更新日期:2002-04-01 00:00:00
abstract::Dual chamber pacemakers have inbuilt advanced safety systems such as ventricular safety standby (crosstalk detection) to prevent ventricular oversensing resulting in inappropriate pacing inhibition. We describe a case where this safety mechanism does not reliably work and the management required to rectify the situati...
journal_title:Journal of cardiovascular electrophysiology
pub_type: 杂志文章
doi:10.1111/jce.13162
更新日期:2017-05-01 00:00:00
abstract:INTRODUCTION:Little data exists on the electrophysiological differences between sustained atrial fibrillation (sAF; >5 minutes) vs self-terminating nonsustained AF (nsAF; <5 minutes). We sought to investigate the electrophysiological characteristics of coronary sinus (CS) activity during postpulmonary vein isolation (P...
journal_title:Journal of cardiovascular electrophysiology
pub_type: 杂志文章
doi:10.1111/jce.14296
更新日期:2020-01-01 00:00:00
abstract:INTRODUCTION:Blockade of the rapid delayed rectifier potassium current (IKr) as an important mechanism for current Class III antiarrhythmics is less effective in action potential prolongation during beta-adrenergic activation. We hypothesized that blockade of the increased slow IK (IKs) current during beta-adrenergic s...
journal_title:Journal of cardiovascular electrophysiology
pub_type: 杂志文章
doi:10.1111/j.1540-8167.1997.tb01039.x
更新日期:1997-12-01 00:00:00
abstract:BACKGROUND:Close association between atrial fibrillation (AF) and brain natriuretic peptide (BNP) has been demonstrated by several studies. Important gender differences exist in AF patients including a higher plasma BNP level in women. Therefore, it is imperative to evaluate the relationship between AF and BNP separate...
journal_title:Journal of cardiovascular electrophysiology
pub_type: 杂志文章
doi:10.1111/j.1540-8167.2011.02036.x
更新日期:2011-08-01 00:00:00
abstract::Cost-efficacy assessment of implantable cardioverter defibrillator (ICD) therapy has proved contentious and may have limited uptake of ICD therapy, particularly in Europe. Published modeling assessments are too inaccurate to determine clinical practice, and assessments based on clinical studies are incomplete (from th...
journal_title:Journal of cardiovascular electrophysiology
pub_type: 杂志文章,评审
doi:10.1111/j.1540-8167.2002.tb01964.x
更新日期:2002-01-01 00:00:00
abstract:INTRODUCTION:Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is an inherited cardiomyopathy characterized by ventricular arrhythmias and heart failure. The variable phenotype suggesting that determined environmental factors may have an influence. The aim of our study was to discover the impact of the...
journal_title:Journal of cardiovascular electrophysiology
pub_type: 杂志文章
doi:10.1111/jce.13704
更新日期:2018-11-01 00:00:00
abstract:INTRODUCTION:The purpose of this study is to describe a simple and reliable diagnostic maneuver that allows for the rapid differentiation of atypical AV nodal reentrant tachycardia (AVNRT) from other causes of long RP tachycardia. Long RP tachycardias may be caused by atypical AVNRT, orthodromic reciprocating tachycard...
journal_title:Journal of cardiovascular electrophysiology
pub_type: 杂志文章
doi:10.1111/j.1540-8167.1995.tb00446.x
更新日期:1995-09-01 00:00:00
abstract::We describe the case of 2-year-old baby with compound heterozygosity for paternal and maternal alleles mutation of α-subunit of the cardiac sodium channel (SCN5A), sinus node dysfunction, atrial flutter recurrences, and drug induced long-QT syndrome. In this setting, we chose at first to perform linear ablation of cav...
journal_title:Journal of cardiovascular electrophysiology
pub_type: 杂志文章
doi:10.1111/jce.12570
更新日期:2015-03-01 00:00:00
abstract:BACKGROUND:Catheter ablation is an effective treatment for symptomatic individuals with atrial fibrillation (AF) but is associated with a risk of periprocedual stroke. Recent data suggest that this risk may be abolished if catheter ablation is performed with uninterrupted warfarin (UW). We sought to compare the inciden...
journal_title:Journal of cardiovascular electrophysiology
pub_type: 杂志文章,多中心研究
doi:10.1111/jce.12411
更新日期:2014-06-01 00:00:00
abstract:INTRODUCTION:The incidence of left atrial appendage (LAA) thrombus in patients with paroxysmal atrial fibrillation (PAF) who present for pulmonary vein antrum isolation procedure (PVAI) is unknown. METHODS AND RESULTS:All consecutive patients from January 2000 to June 2004 who underwent a PVAI received a computed tomo...
journal_title:Journal of cardiovascular electrophysiology
pub_type: 杂志文章
doi:10.1111/j.1540-8167.2007.01070.x
更新日期:2008-04-01 00:00:00
abstract:UNLABELLED:Epicardial Cryoablation in Swine. INTRODUCTION:Cryoablation is an alternative to radiofrequency (RF) energy used in some ablation procedures. Its role and effectiveness compared to irrigated RF in epicardial tissue and epicardial substrates is not yet fully established. METHODS AND RESULTS:Using a swine ch...
journal_title:Journal of cardiovascular electrophysiology
pub_type: 杂志文章
doi:10.1111/j.1540-8167.2012.02334.x
更新日期:2012-09-01 00:00:00
abstract:INTRODUCTION:Substrate-based ablation for scar-related ventricular tachycardia (VT) has gained prominence: however, there is limited data comparing it to ablation guided predominantly by activation and entrainment mapping of inducible and hemodynamically tolerated VTs. We compared the acute procedural efficacy and outc...
journal_title:Journal of cardiovascular electrophysiology
pub_type: 杂志文章,meta分析,评审
doi:10.1111/jce.13088
更新日期:2016-12-01 00:00:00
abstract::Atrial fibrillation is the most common sustained tachyarrhythmia and, as such, has become the recent focus of intense clinical and experimental interest. Because of its associated morbidity and mortality, there is a multidisciplinary effort to understand the pathophysiology that may ultimately lead to improved therape...
journal_title:Journal of cardiovascular electrophysiology
pub_type: 杂志文章,评审
doi:10.1111/j.1540-8167.1997.tb00855.x
更新日期:1997-08-01 00:00:00
abstract::This case report demonstrates the use of an automated voltage mapping algorithm to facilitate the rapid mapping of the low-voltage zone and isolate the critical diastolic pathway of an intra-atrial reentrant tachycardia circuit. Catheter ablation targeted to this pathway successfully terminated the arrhythmia. ...
journal_title:Journal of cardiovascular electrophysiology
pub_type: 杂志文章
doi:10.1111/j.1540-8167.2006.00422.x
更新日期:2006-07-01 00:00:00
abstract:INTRODUCTION:We assessed findings in cardiac magnetic resonance (CMR) as predictors of ventricular tachycardia (VT) after myocardial infarction (MI), which could allow for more precise identification of patients at risk of sudden cardiac death. METHODS:Forty-eight patients after prior MI were enrolled and divided into...
journal_title:Journal of cardiovascular electrophysiology
pub_type: 杂志文章
doi:10.1111/jce.14461
更新日期:2020-06-01 00:00:00
abstract:INTRODUCTION:Sudden cardiac death is a substantial cause of mortality in patients with cardiomyopathy, but evidence supporting implantable cardioverter-defibrillator (ICD) implantation is less robust in nonischemic cardiomyopathy (NICM) than in ischemic cardiomyopathy. Improved risk stratification is needed. We assesse...
journal_title:Journal of cardiovascular electrophysiology
pub_type: 杂志文章
doi:10.1111/jce.14395
更新日期:2020-05-01 00:00:00
abstract:INTRODUCTION:Many patients with atrial fibrillation (AF) receive amiodarone. Amiodarone-induced hyperthyroidism (AIH) may develop as a complication. We hypothesized that pulmonary vein (PV) isolation in patients with paroxysmal AF and history of AIH may yield a lower success rate. METHODS AND RESULTS:Among 704 patient...
journal_title:Journal of cardiovascular electrophysiology
pub_type: 杂志文章
doi:10.1111/jce.12140
更新日期:2013-08-01 00:00:00
abstract:INTRODUCTION:The exit site and central common pathway of slow conduction are preferred sites to guide radiofrequency ablation of postinfarction ventricular tachycardia (VT). Both require inducibility of VT. In addition, their low amplitude hampers direct recording of potentials generated by activation in pathways of sl...
journal_title:Journal of cardiovascular electrophysiology
pub_type: 杂志文章
doi:10.1046/j.1540-8167.2001.00662.x
更新日期:2001-06-01 00:00:00
abstract:INTRODUCTION:We conducted this study to examine the effect of the ICD on the outcomes of patients with prior coronary revascularization enrolled in the Sudden Cardiac Death in Heart Failure Trial (SCD-HeFT) and to assess the association of time from coronary revascularization to enrollment with death and sudden cardiac...
journal_title:Journal of cardiovascular electrophysiology
pub_type: 杂志文章
doi:10.1111/j.1540-8167.2008.01191.x
更新日期:2008-10-01 00:00:00
abstract:INTRODUCTION:Prevention of sudden arrhythmic cardiac death depends on accurate identification of individuals at high risk. Previous studies of signals recorded directly from arrhythmogenic tissue suggested that the predictive value of the signal-averaged ECG could be enhanced by expanded temporal, spectral, and spatial...
journal_title:Journal of cardiovascular electrophysiology
pub_type: 临床试验,杂志文章
doi:10.1111/j.1540-8167.2000.tb00315.x
更新日期:2000-02-01 00:00:00
abstract:INTRODUCTION:The incidence and characteristics of dissociated arrhythmia confined to the pulmonary vein (PV) following disconnection have not been described in a large number of patients with paroxysmal atrial fibrillation. METHODS AND RESULTS:This was a prospective study of 152 patients (29 female, mean age 51 +/- 11...
journal_title:Journal of cardiovascular electrophysiology
pub_type: 临床试验,杂志文章
doi:10.1046/j.1540-8167.2003.02583.x
更新日期:2003-11-01 00:00:00
abstract:INTRODUCTION:Cerebral thromboembolic event (CTE) is a possible complication of pulmonary vein antrum isolation (PVAI). The objective of this study was to report long-term functional and neurocognitive recovery in patients who had a CTE during or within 48 hours of catheter ablation. METHODS AND RESULTS:We screened 3,0...
journal_title:Journal of cardiovascular electrophysiology
pub_type: 杂志文章
doi:10.1111/j.1540-8167.2009.01650.x
更新日期:2010-04-01 00:00:00
abstract:BACKGROUND:The incidence of atrial fibrillation (AF) in heart transplant patients has not been well documented. METHODS:To determine the incidence of AF in a cohort of patients undergoing cardiac transplantation, clinical data were obtained from a prospectively collected database for all consecutive orthotopic heart t...
journal_title:Journal of cardiovascular electrophysiology
pub_type: 杂志文章
doi:10.1111/j.1540-8167.2006.00497.x
更新日期:2006-08-01 00:00:00
abstract:BACKGROUND:The presence of a single left superior vena cava in the absence of complex congenital heart disease is uncommon, and, in the absence of hemodynamic consequences, it would not be expected to result in cardiovascular signs or symptoms. Single case reports and our anecdotal experience suggested to us that this ...
journal_title:Journal of cardiovascular electrophysiology
pub_type: 杂志文章
doi:10.1111/j.1540-8167.2008.01307.x
更新日期:2009-02-01 00:00:00