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https://www.readbyqxmd.com/read/29234865/electromechanical-heterogeneity-in-the-heart-a%C3%A2-key-to-long-qt-syndrome
#1
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...
December 12, 2017: Herzschrittmachertherapie & Elektrophysiologie
https://www.readbyqxmd.com/read/29221324/the-role-of-sympathectomy-in-long-qt-syndrome
#2
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
#3
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 to January 2016...
December 8, 2017: Pacing and Clinical Electrophysiology: PACE
https://www.readbyqxmd.com/read/29127493/late-sodium-current-associated-cardiac-electrophysiological-and-mechanical-dysfunction
#4
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...
November 10, 2017: Pflügers Archiv: European Journal of Physiology
https://www.readbyqxmd.com/read/29083088/clinical-applications-of-qt-rr-hysteresis-assessment-a%C3%A2-systematic-review
#5
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...
October 30, 2017: Annals of Noninvasive Electrocardiology
https://www.readbyqxmd.com/read/28917558/cardiac-sympathectomy-for-the-management-of-ventricular-arrhythmias-refractory-to-catheter-ablation
#6
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...
September 14, 2017: 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
#7
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
#8
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
#9
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
#10
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
#11
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
#12
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
#13
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
https://www.readbyqxmd.com/read/28382321/electrophysiological-mechanisms-of-long-and-short-qt-syndromes
#14
REVIEW
Gary Tse, Yin Wah Fiona Chan, Wendy Keung, Bryan P Yan
The QT interval on the human electrocardiogram is normally in the order of 450 ms, and reflects the summated durations of action potential (AP) depolarization and repolarization of ventricular myocytes. Both prolongation and shortening in the QT interval have been associated with ventricular tachy-arrhythmias, which predispose affected individuals to sudden cardiac death. In this article, the molecular determinants of the AP duration and the causes of long and short QT syndromes (LQTS and SQTS) are explored...
March 2017: IJC Heart & Vasculature
https://www.readbyqxmd.com/read/28343764/temporary-removal-channelopathies-genetic-testing-and-risk-stratification
#15
Arthur A M Wilde, Ahmad Amin
The publisher regrets that this article has been temporarily removed. A replacement will appear as soon as possible in which the reason for the removal of the article will be specified, or the article will be reinstated. The full Elsevier Policy on Article Withdrawal can be found at http://www.elsevier.com/locate/withdrawalpolicy.
March 18, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/28334543/-functional-impact-of-herg-from-physiological-role-to-target-of-anticancer-therapy
#16
REVIEW
Júlia Šatková, Markéta Bébarová
The human ether-à-go-go related gene (hERG; officially designated as KCNH2) encodes the structure of protein forming α-subunit of voltage-gated ion channel which conducts the rapid component of delayed rectifier K+ current (IKr). This current plays an important role namely in the cardiac repolarization. Mutations in hERG result in inherited arrhythmogenic syndromes characterized by a lenghtening or shortening of QT interval on the electrocardiogram and by an increased occurrence of life-threatening arrhythmias...
December 0: Vnitr̆ní Lékar̆ství
https://www.readbyqxmd.com/read/28316956/sudden-infant-death-syndrome-due-to-long-qt-syndrome-a-brief-review-of-the-genetic-substrate-and-prevalence
#17
REVIEW
Nikolaos S Ioakeimidis, Theodora Papamitsou, Soultana Meditskou, Zafiroula Iakovidou-Kritsi
The pathophysiological mechanisms which lead to sudden infant death syndrome (SIDS) are not completely understood. Cardiac channelopathies are a well-established causative factor with long QT syndrome (LQTS) being the most frequent one, accounting for approximately 12% of SIDS cases. The genetic substrate of the above arrhythmogenic syndrome has been thoroughly described but only specific gene mutations or polymorphisms have been identified as SIDS causative. The review will focus on the prevalence of LQTS-induced SIDS or near-SIDS cases and the mutations held responsible...
December 2017: Journal of Biological Research
https://www.readbyqxmd.com/read/28290961/kcnq1-gene-variants-in-large-asymptomatic-populations-considerations-for-genomic-screening-of-military-cohorts
#18
Paul Kruszka, Karin Weiss, Donald W Hadley
INTRODUCTION: The advances in genomic technology of large populations make the potential for genomic screening of military cohorts and recruits feasible, affording the potential to identify at-risk individuals before occurrence of potentially life-threatening events. Exploring sudden cardiac death, known to cause significant morbidity and mortality in young military service members, we focused on the most common gene associated with long QT syndrome (LQTS), KCNQ1. MATERIALS AND METHODS: Using the publicly available database Exome Aggregation Consortium as a surrogate for a military population, variants in KCNQ1 were filtered on the basis of population prevalence, classification as a disease mutation in the Human Gene Mutation database, and classification as pathogenic or likely pathogenic in the ClinVar database...
March 2017: Military Medicine
https://www.readbyqxmd.com/read/28279743/vectrocardiography-identifies-patients-with-electrocardiographically-concealed-long-qt-syndrome
#19
Daniel Cortez, J Martijn Bos, Michael J Ackerman
BACKGROUND: Long QT syndrome (LQTS) and genotypic subtypes are associated with distinctive T-wave patterns, arrhythmogenic triggers, and corrected QT (QTc) interval risk associations. Twenty percent of patients with LQTS have normal QTc values, defined as electrographically concealed LQTS (ecLQTS). Vectorcardiography (VCG) has value for sudden cardiac death risk assessment. OBJECTIVE: The purpose of this study was to determine the use of VCG to identify patients with ecLQTS...
March 6, 2017: Heart Rhythm: the Official Journal of the Heart Rhythm Society
https://www.readbyqxmd.com/read/28155223/evaluation-of-prolonged-qt-interval-structural-heart-disease-mimicking-long-qt-syndrome
#20
Adaya Weissler-Snir, Michael H Gollob, Vijay Chauhan, Melanie Care, Danna A Spears
BACKGROUND: In about 20-25% of patients with congenital long QT syndrome (LQTS) a causative pathogenic mutation is not found. The aim of this study was to explore the prevalence of alternative cardiac diagnoses among patients exhibiting prolongation of QT interval with negative genetic testing for LQTS genes. METHODS: We conducted a retrospective analysis of 239 consecutive patients who were evaluated in the inherited arrhythmia clinic at the Toronto General Hospital between July 2013 and December 2015 for possible LQTS...
February 3, 2017: Pacing and Clinical Electrophysiology: PACE
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