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https://www.readbyqxmd.com/read/29339628/downregulation-of-long-non-coding-rna-kcnq1ot1-an-important-mechanism-of-arsenic-trioxide-induced-long-qt-syndrome
#1
Yanan Jiang, Weijie Du, Qun Chu, Ying Qin, Gulnara Tuguzbaeva, Hui Wang, Anqi Li, Guiyang Li, Yanyao Li, Lu Chai, Er Yue, Xi Sun, Zhiguo Wang, Valentin Pavlov, Baofeng Yang, Yunlong Bai
BACKGROUND/AIMS: Arsenic trioxide (ATO) is a known anti-acute promyelocytic leukemia (APL) reagent, whose clinical applications are limited by its serious cardiac toxicity and fatal adverse effects, such as sudden cardiac death resulting from long QT syndrome (LQTS). The mechanisms of cardiac arrhythmia due to ATO exposure still need to be elucidated. Long non-coding RNAs (lncRNAs) are emerging as major regulators of various pathophysiological processes. This study aimed to explore the involvement of lncRNAs in ATO-induced LQTS in vivo and in vitro...
January 15, 2018: Cellular Physiology and Biochemistry
https://www.readbyqxmd.com/read/29331839/functional-characterization-of-a-novel-herg-variant-in-a-family-with-recurrent-sudden-infant-death-syndrome-retracting-a-genetic-diagnosis
#2
Valentine Sergeev, Frances Perry, Thomas M Roston, Shubhayan Sanatani, Glen F Tibbits, Thomas W Claydon
Long QT syndrome (LQTS) is the most common cardiac ion channelopathy and has been found to be responsible for approximately 10% of sudden infant death syndrome (SIDS) cases. Despite increasing use of broad panels and now whole exome sequencing (WES) in the investigation of SIDS, the probability of identifying a pathogenic mutation in a SIDS victim is low. We report a family-based study who are afflicted by recurrent SIDS in which several members harbor a variant, p.Pro963Thr, in the C-terminal region of the human-ether-a-go-go (hERG) gene, published to be responsible for cases of LQTS type 2...
December 20, 2017: Forensic Science International
https://www.readbyqxmd.com/read/29314443/ryanodine-receptor-inhibition-by-dantrolene-effectively-suppresses-ventricular-arrhythmias-in-an-ex-vivo-model-of-long-qt-syndrome
#3
Gerrit Frommeyer, Julius Krawczyk, Christian Ellermann, Nils Bögeholz, Simon Kochhäuser, Dirk G Dechering, Michael Fehr, Lars Eckardt
AIMS: A significant antiarrhythmic potential of ryanodine receptor inhibition was reported in experimental studies. The aim of the present study was to assess potential antiarrhythmic effects of dantrolene in an experimental whole-heart model of drug-induced long-QT syndrome (LQTS). METHODS: In 12 isolated rabbit hearts long-QT-2-syndrome was simulated by infusion of erythromycin (300μM). 12 rabbit hearts were treated with veratridine (0.5μM) to mimic long-QT-3-syndrome...
January 4, 2018: Journal of Cardiovascular Electrophysiology
https://www.readbyqxmd.com/read/29275885/dynamic-qt-interval-changes-from-supine-to-standing-in-healthy-children
#4
Audrey Dionne, Anne Fournier, Nagib Dahdah, Dominic Abrams, Paul Khairy, Sylvia Abadir
BACKGROUND: QT-interval variations in response to exercise-induced increases in heart rate have been reported in children and adults in the diagnosis of long QT syndrome (LQTS). A quick standing challenge has been proposed as an alternative provocative test in adults, with no pediatric data yet available. METHODS: A standing test was performed in 100 healthy children (mean age, 9.7 ± 3.1 years) after 10 minutes in a supine position with continuous electrocardiographic recording...
January 2018: Canadian Journal of Cardiology
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
#5
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/29236230/gene-patents-in-canada-is-there-a-new-legal-landscape
#6
Katherine L Bonter, Carmela De Luca, Christi J Guerrini
In 2016, the Children's Hospital of Eastern Ontario (CHEO) announced the settlement of its patent lawsuit against US-based Transgenomic, Inc. At issue in the case was CHEO's ability to test for gene mutations associated with long QT syndrome (LQTS) that are described in Transgenomic's patents. CHEO challenged the patents as invalid, and Transgenomic ultimately agreed to license them on a royalty-free basis to CHEO and other healthcare institutions for LQTS testing and research. While widely celebrated in the media, the ethical rhetoric surrounding the settlement has at times obscured the practical and legal context in which it was made and will operate...
December 13, 2017: Molecular Diagnosis & Therapy
https://www.readbyqxmd.com/read/29234865/electromechanical-heterogeneity-in-the-heart-a%C3%A2-key-to-long-qt-syndrome
#7
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
#8
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
#9
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/29214556/influence-of-genetic-modifiers-on-sudden-cardiac-death-cases
#10
Tina Jenewein, Thomas Neumann, Damir Erkapic, Malte Kuniss, Marcel A Verhoff, Gerhard Thiel, Silke Kauferstein
Sequence variants in the ion channel genes KCNH2 and SCN5A may cause the cardiac disorder long QT syndrome (LQTS). This disorder is associated with incomplete penetrance and variable expression in KCNH2- or SCN5A-mutation carriers. Common genetic variants, if associated with a mutation, may affect the severity of this cardiac disorder. This study identified rare mutations in the cardiac ion channel genes KCNH2 and SCN5A in a SCD case, as well as in a LQTS-affected family with a history of SCD. Moreover, common variants were found to occur together within the same genes...
December 6, 2017: International Journal of Legal Medicine
https://www.readbyqxmd.com/read/29197658/utilizing-the-genome-aggregation-database-computational-pathogenicity-prediction-tools-and-patch-clamp-heterologous-expression-studies-to-demote-previously-published-type-1-long-qt-syndrome-mutations-from-pathogenic-to-benign
#11
Daniel J Clemens, Anne R Lentino, Jamie D Kapplinger, Dan Ye, Wei Zhou, David J Tester, Michael J Ackerman
BACKGROUND: Mutations in the KCNQ1-encoded Kv7.1 potassium channel cause type 1 long QT syndrome (LQT1). It has been suggested that ∼10-20% of rare LQTS case-derived variants in the literature may have been published erroneously as LQT1-causative mutations and may be "false positives." OBJECTIVE: To determine which previously published KCNQ1 case variants are likely false positives. METHODS: A list of all published, case-derived KCNQ1 missense variants (MVs) was compiled...
November 29, 2017: Heart Rhythm: the Official Journal of the Heart Rhythm Society
https://www.readbyqxmd.com/read/29183619/non-sustained-microvolt-level-t-wave-alternans-in-congenital-long-qt-syndrome-types-1-and-2
#12
Jovil A Kannampuzha, Prasanna Sengodan, Sravani Avula, Bartholomew White, Stephen J Ganocy, Peter J Leo, Elizabeth S Kaufman
BACKGROUND: Patients with long QT syndrome (LQTS) are predisposed to polymorphic ventricular tachycardia (VT) during adrenergic stimulation. Microvolt T-wave alternans (MTWA) is linked to vulnerability to VT in structural heart disease. The prevalence of non-sustained MTWA (NS-MTWA) in LQTS is unknown. METHODS: 31 LQT1, 42 LQT2, and 80 controls underwent MTWA testing during exercise. MTWA tests were classified per standardized criteria, and re-analyzed according to the modified criteria to account for NS-MTWA...
November 21, 2017: Journal of Electrocardiology
https://www.readbyqxmd.com/read/29174490/risk-of-cardiac-events-associated-with-antidepressant-therapy-in-patients-with-long-qt-syndrome
#13
Meng Wang, Barbara Szepietowska, Bronislava Polonsky, Scott McNitt, Arthur J Moss, Wojciech Zareba, David S Auerbach
Patients with long QT syndrome (LQTS) are at a high risk of cardiac events. Many patients with LQTS are treated with antidepressant drugs (ADs). We investigated the LQTS genotype-specific risk of recurrent cardiac arrhythmic events (CAEs) associated with AD therapy. The study included 59 LQT1 and 72 LQT2 patients from the Rochester-based LQTS Registry with corrected QT (QTc) prolongation and a history of AD therapy. Using multivariate Anderson-Gill models, we estimated the LQTS genotype-specific risk of recurrent CAEs (ventricular tachyarrhythmias, aborted cardiac arrest, or sudden cardiac death) associated with time-dependent ADs...
November 13, 2017: American Journal of Cardiology
https://www.readbyqxmd.com/read/29174246/incidence-of-drug-induced-torsades-de-pointes-with-intravenous-amiodarone
#14
Jayaprakash Shenthar, Jayasheelan Mambally Rachaiah, Vivek Pillai, Siva Sankara Chakali, Vidhyakar Balasubramanian, Manjunath Chollenhalli Nanjappa
AIM: To define the incidence, presentation, and outcomes of drug-induced Torsades de Pointes (TdP) with intravenous (IV) amiodarone. METHODS: From January 2014 to August 2016 a total of 268 patients received IV amiodarone, 142 for ventricular tachycardia, 104 for atrial flutter/fibrillation, and 22 for incessant atrial tachycardia. A uniform dosing of amiodarone to yield 1gm/day was used in all patients. RESULTS: Four of the 268 patients (M:F 1:3) with mean age of 51...
November 2017: Indian Heart Journal
https://www.readbyqxmd.com/read/29162896/%C3%AE-adrenergic-stimulation-augments-transmural-dispersion-of-repolarization-via-modulation-of-delayed-rectifier-currents-iks-and-ikr-in-the-human-ventricle
#15
C Kang, A Badiceanu, J A Brennan, C Gloschat, Y Qiao, N A Trayanova, I R Efimov
Long QT syndrome (LQTS) is an inherited or drug induced condition associated with delayed repolarization and sudden cardiac death. The cardiac potassium channel, IKr, and the adrenergic-sensitive cardiac potassium current, IKs, are two primary contributors to cardiac repolarization. This study aimed to elucidate the role of β-adrenergic (β-AR) stimulation in mediating the contributions of IKr and IKs to repolarizing the human left ventricle (n = 18). Optical mapping was used to measure action potential durations (APDs) in the presence of the IKs blocker JNJ-303 and the IKr blocker E-4031...
November 21, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29146210/novel-intracellular-transport-refractory-mutations-in-kcnh2-identified-in-patients-with-symptomatic-long-qt-syndrome
#16
Daisuke Fukumoto, Wei-Guang Ding, Yuko Wada, Yusuke Fujii, Mari Ichikawa, Koichiro Takayama, Megumi Fukuyama, Koichi Kato, Hideki Itoh, Takeru Makiyama, Mariko Omatsu-Kanbe, Hiroshi Matsuura, Minoru Horie, Seiko Ohno
BACKGROUND: Missense mutations in KCNH2, a gene encoding the Kv11.1 channel, cause long QT syndrome (LQTS) type 2 primarily by disrupting the intracellular transport of Kv11.1 to the plasma membrane. The present study aimed to clarify the functional changes by two novel KCNH2 missense mutations. METHODS: We performed genetic screening of three unrelated symptomatic LQTS probands with family histories of cardiac symptoms. Chinese hamster ovary cells were transfected with wild-type (WT) and/or mutant KCNH2 plasmid and examined by patch-clamp technique...
November 13, 2017: Journal of Cardiology
https://www.readbyqxmd.com/read/29141844/architectural-t-wave-analysis-and-identification-of-on-therapy-breakthrough-arrhythmic-risk-in-type-1-and-type-2-long-qt-syndrome
#17
Alan Sugrue, Ram K Rohatgi, Peter A Noseworthy, Vaclav Kremen, J Martijn Bos, Bo Qiang, Yehu Sapir, Zachi I Attia, Christopher G Scott, Peter Brady, Samuel J Asirvatham, Paul A Friedman, Michael J Ackerman
BACKGROUND: Although the hallmark of long-QT syndrome (LQTS) is abnormal cardiac repolarization, there are varying degrees of phenotypic expression and arrhythmic risk. Our aim was to evaluate the performance of a morphological T-wave analysis program in defining breakthrough LQTS arrhythmic risk beyond the QTc value. METHODS AND RESULTS: We analyzed 407 genetically confirmed patients with LQT1 (n=246; 43% men) and LQT2 (n=161; 41% men) over the mean follow-up period of 6...
November 2017: Circulation. Arrhythmia and Electrophysiology
https://www.readbyqxmd.com/read/29127493/late-sodium-current-associated-cardiac-electrophysiological-and-mechanical-dysfunction
#18
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/29121487/risk-of-cardiac-events-in-long-qt-syndrome-patients-when-taking-antiseizure-medications
#19
David S Auerbach, Yitschak Biton, Bronislava Polonsky, Scott McNitt, Robert A Gross, Robert T Dirksen, Arthur J Moss
Many antiseizure medications (ASMs) affect ion channel function. We investigated whether ASMs alter the risk of cardiac events in patients with corrected QT (QTc) prolongation. The study included people from the Rochester-based Long QT syndrome (LQTS) Registry with baseline QTc prolongation and history of ASM therapy (n = 296). Using multivariate Anderson-Gill models, we assessed the risk of recurrent cardiac events associated with ASM therapy. We stratified by LQTS genotype and predominant mechanism of ASM action (Na(+) channel blocker and gamma-aminobutyric acid modifier...
October 20, 2017: Translational Research: the Journal of Laboratory and Clinical Medicine
https://www.readbyqxmd.com/read/29104543/simultaneous-quantification-of-spatially-discordant-alternans-in-voltage-and-intracellular-calcium-in-langendorff-perfused-rabbit-hearts-and-inconsistencies-with-models-of-cardiac-action-potentials-and-ca-transients
#20
Ilija Uzelac, Yanyan C Ji, Daniel Hornung, Johannes Schröder-Scheteling, Stefan Luther, Richard A Gray, Elizabeth M Cherry, Flavio H Fenton
Rationale: Discordant alternans, a phenomenon in which the action potential duration (APDs) and/or intracellular calcium transient durations (CaDs) in different spatial regions of cardiac tissue are out of phase, present a dynamical instability for complex spatial dispersion that can be associated with long-QT syndrome (LQTS) and the initiation of reentrant arrhythmias. Because the use of numerical simulations to investigate arrhythmic effects, such as acquired LQTS by drugs is beginning to be studied by the FDA, it is crucial to validate mathematical models that may be used during this process...
2017: Frontiers in Physiology
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