Abstract:
INTRODUCTION:A new strategy to elucidate the molecular mechanisms underlying the long QT syndrome (LQTS) is now available with genetic mutational analyses and characterization of ion channel mutations. METHODS AND RESULTS:In a 26-year-old woman with LQTS, we identified a novel missense mutation in the pore of HERG by using polymerase chain reaction/single-strand conformation polymorphism (PCR/SSCP) and sequencing of her genomic DNA. The mutation resulted in an amino acid substitution of a positively charged lysine for a highly conserved uncharged asparagine at codon 629 (N629K). Whole cell, patch clamp studies were conducted in COS7 cells by transfecting with wild-type (WT) and/or the mutant N629K HERG. The WT HERG produced an I(Kr)-like, E-4031-sensitive conductance with an inward rectification. In contrast, the cells transfected with the N629K HERG did not display any time-dependent current. Cotransfection of WT and N629K HERG (at a ratio of 1:1) produced a significantly smaller conductance when compared with WT HERG (WT 59.9 +/- 7.3 pA/pF [n = 22] vs WT+N629K 5.5 +/- 2.3 pA/pF [n = 11]; P < 0.01), but did not alter K+ ion selectivity and tail current-voltage dependence. Because aprindine hydrochloride was effective in preventing ventricular tachycardias, we also tested the effect of the drug on WT HERG (I(Kr)) and KvLQT1/KCNE1 (I(Ks)) currents expressed in COS7. CONCLUSION:Functional analyses of a novel missense mutation in the pore of HERG suggest that the mutation causes marked reduction of I(Kr) via a dominant negative effect.
journal_name
J Cardiovasc Electrophysioljournal_title
Journal of cardiovascular electrophysiologyauthors
Yoshida H,Horie M,Otani H,Takano M,Tsuji K,Kubota T,Fukunami M,Sasayama Sdoi
10.1111/j.1540-8167.1999.tb00304.xkeywords:
subject
Has Abstractpub_date
1999-09-01 00:00:00pages
1262-70issue
9eissn
1045-3873issn
1540-8167journal_volume
10pub_type
杂志文章abstract::Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an uncommon cardiomyopathy most classically associated with mutations in genes encoding desmosomal proteins. Recent literature has identified mutations in several non-desmosomal proteins including lamins that may result in the ARVC phenotype. We describe a pati...
journal_title:Journal of cardiovascular electrophysiology
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pub_type: 杂志文章,随机对照试验
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更新日期:2015-10-01 00:00:00
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journal_title:Journal of cardiovascular electrophysiology
pub_type: 杂志文章,meta分析,评审
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pub_type: 杂志文章
doi:10.1111/j.1540-8167.2007.00914.x
更新日期:2007-09-01 00:00:00
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journal_title:Journal of cardiovascular electrophysiology
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更新日期:2012-09-01 00:00:00
abstract:INTRODUCTION:Phrenic nerve (PN) injury is a well-known complication of cryoballoon ablation (CBA) for pulmonary vein (PV) isolation in patients with atrial fibrillation. However, it is still insufficient to practically predict phrenic nerve injury (PNI) before freezing. We hypothesized that phrenic nerve capture (PNC) ...
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pub_type: 杂志文章
doi:10.1111/jce.13962
更新日期:2019-08-01 00:00:00
abstract:INTRODUCTION:Atrial arrhythmias have emerged as a topic of great interest for clinical electrophysiologists. Noninvasive imaging of electrical function in humans may be useful for computer-aided diagnosis and treatment of cardiac arrhythmias, which can be accomplished by the fusion of data from ECG mapping and magnetic...
journal_title:Journal of cardiovascular electrophysiology
pub_type: 临床试验,杂志文章
doi:10.1046/j.1540-8167.2003.02558.x
更新日期:2003-07-01 00:00:00
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journal_title:Journal of cardiovascular electrophysiology
pub_type: 临床试验,杂志文章
doi:10.1111/jce.12659
更新日期:2015-06-01 00:00:00
abstract::Enduring isolation of the pulmonary veins (PV) is becoming increasingly common in patients undergoing repeat catheter ablation for atrial fibrillation. We describe our approach to ablation strategy in a patient with enduring isolation of PV and briefly discuss the role of non-PV triggers. ...
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pub_type: 杂志文章,评审
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更新日期:2020-07-01 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:INTRODUCTION:We report the case of a 36-year-old patient with a longstanding history of paroxysmal tachycardia. METHODS AND RESULTS:During the electrophysiological study, the H-V interval was prolonged in sinus rhythm, and a second potential (H'') with distal to proximal activation pattern was recorded in the region o...
journal_title:Journal of cardiovascular electrophysiology
pub_type: 杂志文章
doi:10.1111/j.1540-8167.2006.00468.x
更新日期:2006-08-01 00:00:00
abstract::When an implanted device is infected, complete explantation of the device system, including lead extraction, is usually required. Superficial problems with wound healing may be managed more conservatively, but distinguishing between a surface process and deeper infection can pose a clinical challenge. We present a cas...
journal_title:Journal of cardiovascular electrophysiology
pub_type: 杂志文章
doi:10.1111/j.1540-8167.2011.02224.x
更新日期:2012-03-01 00:00:00
abstract:INTRODUCTION:Pacing is believed to prevent atrial fibrillation by reducing atrial activation time. Exact correlation between P wave duration (PWD) on surface ECG and endocardial atrial activation time is still unexplored. METHODS AND RESULTS:In 15 patients without structural heart disease (9 women, age 45 +/- 14 years...
journal_title:Journal of cardiovascular electrophysiology
pub_type: 杂志文章
doi:10.1046/j.1540-8167.2002.01240.x
更新日期:2002-12-01 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::Left-sided anteroseptal accessory pathways that course through the aortomitral fibrous continuity are some of the rarest types of accessory pathways. At this region the atrium and ventricle are separated by their greatest distance because of the intervening aortic valve. These pathways often have a long circuitous cou...
journal_title:Journal of cardiovascular electrophysiology
pub_type: 杂志文章
doi:10.1111/j.1540-8167.2012.02440.x
更新日期:2013-04-01 00:00:00
abstract:INTRODUCTION:High recurrence rate is still a major problem associated with ablation of paroxysmal atrial fibrillation (AF). Most of the recurrences occur within 6 months after ablation. The characteristics of very late recurrent AF (>12 months after ablation) have not been reported. METHODS AND RESULTS:Two hundred sev...
journal_title:Journal of cardiovascular electrophysiology
pub_type: 杂志文章
doi:10.1046/j.1540-8167.2003.03047.x
更新日期:2003-06-01 00:00:00
abstract:INTRODUCTION:Quinidine has been evaluated in patients with a short QT-1 syndrome caused by an IKr gain-of-function mutation of HERG. Recently, in vitro data with disopyramide showed an even stronger effect on the N588K mutant current. The aim of the present study was to test the in vivo effects of disopyramide in patie...
journal_title:Journal of cardiovascular electrophysiology
pub_type: 杂志文章
doi:10.1111/j.1540-8167.2007.00925.x
更新日期:2007-11-01 00:00:00
abstract::Syncope is frequently neurally mediated and can seriously affect quality of life. Different ablation strategies have been successfully performed. These approaches have not gained wide acceptance and are quite extensive and complex, exposing patients to significant risks. This article reports the case of a 16-year-old ...
journal_title:Journal of cardiovascular electrophysiology
pub_type:
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更新日期:2016-09-01 00:00:00
abstract:INTRODUCTION:The pulmonary veins (PVs) are a dominant source of triggers initiating atrial fibrillation (AF). While recent evidence implicates these structures in the maintenance of paroxysmal AF, their role in permanent AF is not known. The current study aims to compare the contribution of PV activity to the maintenan...
journal_title:Journal of cardiovascular electrophysiology
pub_type: 杂志文章
doi:10.1111/j.1540-8167.2006.00546.x
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abstract:INTRODUCTION:Early recurrence of atrial fibrillation (AF) after cardioversion may be related to shortening of the atrial effective refractory period (ERP). This study compared the effects of verapamil and ibutilide on AF cycle length (AFCL), atrial ERP, and susceptibility to recurrent AF. METHODS AND RESULTS:In 33 adu...
journal_title:Journal of cardiovascular electrophysiology
pub_type: 杂志文章
doi:10.1046/j.1540-8167.2002.00151.x
更新日期:2002-02-01 00:00:00
abstract:INTRODUCTION:The autonomic nervous system richly innervates the peri-AV nodal region and may be activated during radiofrequency (RF) ablation for AV nodal reentrant tachycardia, resulting in the generation of junctional tachycardia. The purpose of this prospective study was to determine the role of the autonomic nervou...
journal_title:Journal of cardiovascular electrophysiology
pub_type: 杂志文章
doi:10.1111/j.1540-8167.1999.tb00642.x
更新日期:1999-01-01 00:00:00
abstract:INTRODUCTION:Bipolar voltage criteria to delineate left atrial (LA) scar have been derived using point-by-point (PBP) contact electroanatomical mapping. It remains unclear how PBP-derived LA scar correlates with multielectrode fast automated mapping (ME-FAM) derived scar. We aimed to correlate scar and bipolar voltages...
journal_title:Journal of cardiovascular electrophysiology
pub_type: 杂志文章
doi:10.1111/jce.13151
更新日期:2017-03-01 00:00:00
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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
abstract:INTRODUCTION:The relationship between the determining factors and beating rates of pulmonary vein (PV) pacemaker cardiomyocytes has not been fully elucidated. The purposes of this study were to compare the electrophysiological characteristics between PV fast and slow pacemaker cardiomyocytes. METHODS:Whole-cell patch ...
journal_title:Journal of cardiovascular electrophysiology
pub_type: 杂志文章
doi:10.1111/j.1540-8167.2009.01480.x
更新日期:2009-09-01 00:00:00
abstract:BACKGROUND:Low conduction velocity (CV) in the area showing low electrogram amplitude (EA) is characteristic of reentry circuit of atypical atrial flutter (AFL). The quantitative relationship between CV and EA remains unclear. We characterized AFL reentry circuit in the right atrium (RA), focusing on the relationship b...
journal_title:Journal of cardiovascular electrophysiology
pub_type: 杂志文章
doi:10.1111/jce.12329
更新日期:2014-04-01 00:00:00
abstract:BACKGROUND:Individual risk factors of intraprocedural cardiac injury (cardiac perforation and tamponade) during implantable cardioverter defibrillator (ICD) placement have been documented. However, the prognostic impact of their coexistence has not been explored. OBJECTIVE:To develop a risk score model to identify pat...
journal_title:Journal of cardiovascular electrophysiology
pub_type: 杂志文章
doi:10.1111/jce.13823
更新日期:2019-02-01 00:00:00
abstract:INTRODUCTION:Significant species-, tissue-, and age-dependent differences have been described for the L-type calcium current (ICa). Therefore, extrapolation of data obtained from the many animal models to human cardiac physiology is difficult. In this study, we have characterized the voltage-dependent properties of ICa...
journal_title:Journal of cardiovascular electrophysiology
pub_type: 杂志文章
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更新日期:1993-08-01 00:00:00
abstract:BACKGROUND:Patients with left bundle branch block (LBBB) undergoing right heart catheterization can develop complete heart block (CHB) or right bundle branch block (RBBB) in response to right bundle branch (RBB) trauma. We hypothesized that LBBB patients with an initial r wave (>or=1 mm) in lead V1 have intact left to ...
journal_title:Journal of cardiovascular electrophysiology
pub_type: 杂志文章
doi:10.1111/j.1540-8167.2009.01714.x
更新日期:2010-07-01 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:The latest STS guidelines recommend concomitant atrial fibrillation (AF) ablation not only during mitral surgery (Class IA) but also during other-than-mitral cardiac surgery procedures (Class IB) in patients with preoperative AF. Conventional Cox-Maze III/IV procedures are performed on both atria (BA), but...
journal_title:Journal of cardiovascular electrophysiology
pub_type: 杂志文章,meta分析,评审
doi:10.1111/jce.14117
更新日期:2019-10-01 00:00:00