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Lqts review

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https://www.readbyqxmd.com/read/29625280/long-qt-syndrome-type-5-lite-defining-the-clinical-phenotype-associated-with-the-potentially-pro-arrhythmic-p-asp85asn-kcne1-common-genetic-variant
#1
Conor Lane, John R Giudicessi, Dan Ye, David J Tester, Ram K Rohatgi, J Martijn Bos, Michael J Ackerman
BACKGROUND: Long QT syndrome (LQTS) genetic test reports commonly exclude potentially pro-arrhythmic common variants such as p.Asp85Asn-KCNE1. OBJECTIVE: To determine if a discernible phenotype is associated with p.Asp85Asn-KCNE1 and if relatively common KCNE1 variants underlie transient QT prolongation pedigrees with negative commercial LQTS genetic tests. METHODS: Retrospective review was used to compare demographics, symptomatology, and QT parameters of individuals with p...
April 3, 2018: Heart Rhythm: the Official Journal of the Heart Rhythm Society
https://www.readbyqxmd.com/read/29575607/the-maximum-qtc-of-holter-electrocardiography-in-a-pediatric-population
#2
Yoshiharu Ogawa, Toshikatsu Tanaka, Sachiko Kido
BACKGROUND: The corrected QT interval (QTc) of electrocardiograms (ECGs) at rest and after exercise in a short daytime recording period may be insufficient for the diagnosis and management of long QT syndrome (LQTS) patients, especially for those with LQTS type 2 and 3. Therefore, examining QTc using Holter ECG is important. We designed a method of analyzing QTc in Holter ECG that can be performed in daily clinical practice by combining automatic and manual measurements. METHODS: We reviewed the charts of healthy children (n=210) and LQTS patients (n=35) aged <16 years and analyzed QTc at rest, after exercise, and the maximum QTc of Holter ECG...
March 25, 2018: Pediatrics International: Official Journal of the Japan Pediatric Society
https://www.readbyqxmd.com/read/29550106/meta-analysis-of-t-peak-t-end-and-t-peak-t-end-qt-ratio-for-risk-stratification-in-congenital-long-qt-syndrome
#3
Gary Tse, Mengqi Gong, Lei Meng, Cheuk Wai Wong, Stamatis Georgopoulos, George Bazoukis, Martin C S Wong, Konstantinos P Letsas, Vassilios S Vassiliou, Yunlong Xia, Adrian M Baranchuk, Gan-Xin Yan, Tong Liu
BACKGROUND AND OBJECTIVES: Congenital long QT syndrome (LQTS) predisposes affected individuals to ventricular tachycardia/fibrillation (VF/VF), potentially resulting in sudden cardiac death. The Tpeak -Tend interval and the Tpeak -Tend /QT ratio, electrocardiographic markers of dispersion of ventricular repolarization, were proposed for risk stratification but their predictive values in LQTS have been controversial. A systematic review and meta-analysis was conducted to examine the value of Tpeak -Tend intervals and Tpeak -Tend /QT ratios in predicting arrhythmic and mortality outcomes in congenital LQTS...
March 6, 2018: Journal of Electrocardiology
https://www.readbyqxmd.com/read/29525288/cardiac-channelopathies-the-role-of-sodium-channel-mutations
#4
REVIEW
Diana João Fonseca, Manuel Joaquim Vaz da Silva
INTRODUCTION AND OBJECTIVES: The importance of sodium channels for the normal electrical activity of the heart is emphasized by the fact that mutations (inherited or de novo) in genes that encode for these channels or their associated proteins cause arrhythmogenic syndromes such as the Brugada syndrome and the long QT syndrome (LQTS). The aim of this study is to conduct a review of the literature on the mutations in the sodium channel complex responsible for heart disease and the implications of a close relationship between genetics and the clinical aspects of the main cardiac channelopathies, namely at the level of diagnosis, risk stratification, prognosis, screening of family members and treatment...
March 7, 2018: Portuguese Journal of Cardiology: An Official Journal of the Portuguese Society of Cardiology
https://www.readbyqxmd.com/read/29525134/thoracoscopic-sympathectomy-for-long-qt-syndrome-literature-review-and-case-study
#5
Timothy L Surman, Robert G Stuklis, Justin C Chan
BACKGROUND: Multiple case studies have suggested that video-assisted thoracoscopic sympathectomy (VATS) reduces the occurrence and frequency of symptoms in long QT syndrome (LQTS) [1,2,3]. To date there has not been a literature review to report on the short-term and long-term outcomes of this procedure. Our primary aims are to review the literature findings on the clinical outcomes of VATS sympathectomy for long QT and present a local centre case report on the outcomes of T2-T5 sympathectomy...
February 13, 2018: Heart, Lung & Circulation
https://www.readbyqxmd.com/read/29405316/acquired-long-qt-syndrome-and-torsade-de-pointes
#6
REVIEW
Nabil El-Sherif, Gioia Turitto, Mohamed Boutjdir
Since its initial description by Jervell and Lange-Nielsen in 19571 , the congenital long QT syndrome (LQTS) has been the most investigated cardiac ion channelopathy. Although congenital LQTS continues to remain the domain of cardiologists, cardiac electrophysiologists, and specialized centers, the by far more frequent acquired drug-induced LQTS is the domain of all physicians and other members of the health care team who are required to make therapeutic decisions. This report will review the electrophysiological mechanisms of LQTS and TdP, electrocardiographic (ECG) characteristics of acquired LQTS, its clinical presentation, management, and future directions in the field...
February 6, 2018: Pacing and Clinical Electrophysiology: PACE
https://www.readbyqxmd.com/read/29258620/refractory-ventricular-fibrillations-after-surgical-repair-of-atrial-septal-defects-in-a-patient-with-cacna1c-gene-mutation-case-report
#7
Ai Kojima, Fumiaki Shikata, Toru Okamura, Takashi Higaki, Seiko Ohno, Minoru Horie, Shunji Uchita, Yujiro Kawanishi, Kenji Namiguchi, Takumi Yasugi, Hironori Izutani
BACKGROUND: Congenital long QT syndrome (LQTS) can cause ventricular arrhythmic events with syncope and sudden death resulting from malignant torsades de pointes (TdP) followed by ventricular fibrillations (VFs). However, the syndrome is often overlooked prior to the development of arrhythmic events in patients with congenital heart diseases demonstrating right bundle branch block on electrocardiogram (ECG). We present a case of an adult patient with congenital heart disease who developed VFs postoperatively, potentially due to his mutation in a LQTS related gene, which was not identified on preoperative assessment due to incomplete evaluation of his family history...
December 19, 2017: Journal of Cardiothoracic Surgery
https://www.readbyqxmd.com/read/29234865/electromechanical-heterogeneity-in-the-heart-a-key-to-long-qt-syndrome
#8
REVIEW
F F Dressler, J Brado, K E Odening
In the healthy heart, physiological heterogeneities in structure and in electrical and mechanical activity are crucial for normal, efficient excitation and pumping. Alterations of heterogeneity have been linked to arrhythmogenesis in various cardiac disorders such as long QT syndrome (LQTS). This inherited arrhythmia disorder is caused by mutations in different ion channel genes and is characterized by (heterogeneously) prolonged cardiac repolarization and increased risk for ventricular tachycardia, syncope and sudden cardiac death...
March 2018: Herzschrittmachertherapie & Elektrophysiologie
https://www.readbyqxmd.com/read/29221324/the-role-of-sympathectomy-in-long-qt-syndrome
#9
REVIEW
Achilleas Antonopoulos, David Lawrence, Davide Patrini, Marco Scarci, Robert George, Martin Hayward, Sofoklis Mitsos, Nikolaos Panagiotopoulos
Long QT syndrome (LQTS) is an uncommon and potentially fatal cardiac channelopathy. Treatment options can be medical with β-blockers or surgical with implantable cardioverter defibrillator (ICD) implantations and left cardiac sympathetic denervation (LCSD). Purpose of this paper is through a literature review to identify the management algorithm and the role of sympathectomy in LQTS.
September 2017: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/29219203/automated-qt-analysis-on-holter-monitors-in-pediatric-patients-can-differentiate-long-qt-syndrome-from-controls
#10
Christopher W Follansbee, Lee Beerman, Gaurav Arora
BACKGROUND: Borderline QTc is a common referral to the pediatric cardiology clinic. Evaluation is challenging due to significant overlap of normal and abnormal QTc ranges. We hypothesized that automated QT analysis on Holter could differentiate between patients with long QT syndrome (LQTS) and healthy controls. METHODS: We conducted a retrospective review of 39 patients with known genotype-positive, phenotype-positive LQTS who underwent Holter monitoring between January 2010 and January 2016...
January 2018: Pacing and Clinical Electrophysiology: PACE
https://www.readbyqxmd.com/read/29127493/late-sodium-current-associated-cardiac-electrophysiological-and-mechanical-dysfunction
#11
REVIEW
Shandong Yu, Gang Li, Christopher L-H Huang, Ming Lei, Lin Wu
Late sodium current (INaL ) is a small sustained inward current observed during the cardiac action potential plateau phase following decay of the early peak INa . The endogenous INaL is relatively small in normal hearts but exerts functionally significant effects on cardiomyocyte repolarization with potentially pro-arrhythmic effects in hearts with reduced repolarization reserve. Enhanced INa,L occurs in long QT syndrome 3 (LQTS 3) patients, and under a number of pathological and pharmacological cardiovascular conditions, including bradycardia, myocardial ischemia, reperfusion injury, and heart failure...
March 2018: Pflügers Archiv: European Journal of Physiology
https://www.readbyqxmd.com/read/29083088/clinical-applications-of-qt-rr-hysteresis-assessment-a-systematic-review
#12
REVIEW
Hugo Gravel, Vincent Jacquemet, Nagib Dahdah, Daniel Curnier
BACKGROUND: QT/RR hysteresis (QT-hys) is an index of the time accommodation of ventricular repolarization to heart rate changes. This report comprehensively reviews studies addressing QT-hys as a biomarker of medical conditions. METHODS: This is a secondary analysis of data from a recent systematic review pertaining to methods of assessment of QT-hys. Articles included in the former review were filtered in order to select original articles investigating the association of QT-hys with medical conditions in humans...
January 2018: Annals of Noninvasive Electrocardiology
https://www.readbyqxmd.com/read/28917558/cardiac-sympathectomy-for-the-management-of-ventricular-arrhythmias-refractory-to-catheter-ablation
#13
Travis Richardson, Ricardo Lugo, Pablo Saavedra, George Crossley, Walter Clair, Sharon Shen, Juan Carlos Estrada, Jay Montgomery, M Benjamin Shoemaker, Christopher Ellis, Gregory F Michaud, Eric Lambright, Arvindh N Kanagasundram
BACKGROUND: Catheter ablation is now a mainstay of therapy for ventricular arrhythmias (VAs). However, there are scenarios where either physiological or anatomical factors make ablation less likely to be successful. OBJECTIVE: The purpose of this study was to demonstrate that cardiac sympathetic denervation (CSD) may be an alternate therapy for patients with difficult-to-ablate VAs. METHODS: We identified all patients referred for CSD at a single center for indications other than long QT syndrome and catecholaminergic polymorphic ventricular tachycardia who had failed catheter ablation...
January 2018: Heart Rhythm: the Official Journal of the Heart Rhythm Society
https://www.readbyqxmd.com/read/28725320/splice-site-variants-in-the-kcnq1-and-scn5a-genes-transcript-analysis-as-a-tool-in-supporting-pathogenicity
#14
Ivone U S Leong, Philippa A Dryland, Debra O Prosser, Stella W-S Lai, Mandy Graham, Martin Stiles, Jackie Crawford, Jonathan R Skinner, Donald R Love
BACKGROUND: Approximately 75% of clinically definite long QT syndrome (LQTS) cases are caused by mutations in the KCNQ1, KCNH2 and SCN5A genes. Of these mutations, a small proportion (3.2-9.2%) are predicted to affect splicing. These mutations present a particular challenge in ascribing pathogenicity. METHODS: Here we report an analysis of the transcriptional consequences of two mutations, one in the KCNQ1 gene (c.781_782delinsTC) and one in the SCN5A gene (c.2437-5C>A), which are predicted to affect splicing...
August 2017: Journal of Clinical Medicine Research
https://www.readbyqxmd.com/read/28685698/medical-therapy-for-long-qt-syndrome
#15
George Adamos, Nicoletta Iacovidou, Theodoros Xanthos
Long QT syndrome (LQTS) is an arrhythmogenic disorder characterized by repolarization abnormalities with a propensity to cause life threatening cardiac events. The first manifestation of the syndrome may be sudden death, therefore, early diagnosis and therapy is of great importance. LQTS can be both congenital and acquired. The latter is most commonly seen in hospitalized patients and such individuals have an easily recognizable and reversible precipitating factor (electrolyte disturbances, certain drugs etc...
July 7, 2017: Mini Reviews in Medicinal Chemistry
https://www.readbyqxmd.com/read/28670758/congenital-long-qt-syndrome-and-torsade-de-pointes
#16
REVIEW
Nabil El-Sherif, Gioia Turitto, Mohamed Boutjdir
Since its initial description by Jervell and Lange-Nielsen in 1957, the congenital long QT syndrome (LQTS) has been the most investigated cardiac ion channelopathy. A prolonged QT interval in the surface electrocardiogram is the sine qua non of the LQTS and is a surrogate measure of the ventricular action potential duration (APD). Congenital as well as acquired alterations in certain cardiac ion channels can affect their currents in such a way as to increase the APD and hence the QT interval. The inhomogeneous lengthening of the APD across the ventricular wall results in dispersion of APD...
November 2017: Annals of Noninvasive Electrocardiology
https://www.readbyqxmd.com/read/28532774/genotype-positive-long-qt-syndrome-in-patients-with-coexisting-congenital-heart-disease
#17
Mohammed A Ebrahim, Matthew R Williams, Suzanne Shepard, James C Perry
Congenital long QT syndrome (LQTS) is characterized by QT prolongation with predisposition to life-threatening arrhythmia. There have been sporadic reports of LQTS coexisting with more common forms of congenital heart disease (CHD). However, the diagnosis of LQTS when CHD is present may be confounded by several common variables including postoperative electromechanical factors predisposing to ventricular arrhythmia, intrinsic, and postoperative QRS abnormalities. This report documents a single-center experience with patients who have both genetically confirmed LQTS and CHD to examine their modes of presentation and factors associated with making the diagnosis of LQTS in this patient population, as well as potential confounding variables that may mask or delay both LQTS diagnosis and initiation of therapy...
July 15, 2017: American Journal of Cardiology
https://www.readbyqxmd.com/read/28416468/cardiac-transplantation-in-children-and-adolescents-with-long-qt-syndrome
#18
Angela M Kelle, J Martijn Bos, Susan P Etheridge, Bryan C Cannon, Randall M Bryant, Jonathan N Johnson, Michael J Ackerman
BACKGROUND: Long QT syndrome (LQTS) is a potentially lethal, yet highly treatable, cardiac channelopathy. Cardiac transplantation has been reported anecdotally for patients with severe LQTS refractory to standard therapies. OBJECTIVE: The purpose of this study was to evaluate the incidence of and risk factors for cardiac transplantation in children evaluated and treated in an LQTS specialty center. METHODS: This was a retrospective review of 349 children with LQTS (mean age at diagnosis, 8...
August 2017: Heart Rhythm: the Official Journal of the Heart Rhythm Society
https://www.readbyqxmd.com/read/28413954/computational-models-for-understanding-of-structure-function-and-pharmacology-of-the-cardiac-potassium-channel-kv11-1-herg
#19
REVIEW
Soren Wacker, Sergei Yu Noskov, Laura L Perissinotti
The rapid delayed rectifier current IKr is one of the major K+ currents involved in repolarization of the human cardiac action potential. Various inherited or drug-induced forms of the long QT syndrome (LQTS) in humans are linked to functional and structural modifications in the IKr conducting channels. IKr is carried by the potassium channel Kv11.1 encoded by the gene KCNH2 (commonly referred to as human ether-a-go-go-related gene or hERG) [1, 2]. The first necessary step for predicting emergent drug effects on the heart is determining and modeling the binding thermodynamics and kinetics of primary and major off-target drug interactions with subcellular targets...
2017: Current Topics in Medicinal Chemistry
https://www.readbyqxmd.com/read/28412158/lidocaine-attenuation-testing-an-in%C3%A2-vivo-investigation-of-putative-lqt3-associated-variants-in-the-scn5a-encoded-sodium-channel
#20
Heather N Anderson, J Martijn Bos, Jamie D Kapplinger, Jana M Meskill, Dan Ye, Michael J Ackerman
BACKGROUND: Long QT syndrome type 3 (LQT3) accounts for 5%-10% of long QT syndrome and results from gain-of-function mutations in the SCN5A-encoded sodium channel. Approximately 2% of healthy individuals host rare SCN5A variants of uncertain significance (VUS). Distinction of true LQT3-causative mutations from background genetic noise is essential. OBJECTIVE: The purpose of this study was to assess the use of the lidocaine attenuation test (LAT) in evaluating patients with possible LQT3...
August 2017: Heart Rhythm: the Official Journal of the Heart Rhythm Society
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