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Prolonged QT syndrome

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https://www.readbyqxmd.com/read/28935680/cardiac-events-during-competitive-recreational-and-daily-activities-in-children-and-adolescents-with-long-qt-syndrome
#1
Kristina D Chambers, Virginie Beausejour Ladouceur, Mark E Alexander, Robyn J Hylind, Laura Bevilacqua, Douglas Y Mah, Vassilios Bezzerides, John K Triedman, Edward P Walsh, Dominic J Abrams
BACKGROUND: The 2005 Bethesda Conference Guidelines advise patients with long QT syndrome against competitive sports. We assessed cardiac event rates during competitive and recreational sports, and daily activities among treated long QT syndrome patients. METHODS AND RESULTS: Long QT syndrome patients aged ≥4 years treated with anti-adrenergic therapy were included. Demographics included mechanism of presentation, corrected QT interval pretreatment, symptom history, medication compliance, and administration of QT-prolonging medications...
September 21, 2017: Journal of the American Heart Association
https://www.readbyqxmd.com/read/28928055/electrophysiological-analyses-of-transgenic-mice-overexpressing-kcnj8-with-s422l-mutation-in-cardiomyocytes
#2
Yasuhiro Watanabe, Akio Matsumoto, Takashi Miki, Susumu Seino, Naohiko Anzai, Haruaki Nakaya
Genetic analysis of KCNJ8 has pointed a mutation (S422L) as a susceptible link to J wave syndrome (JWS). In vitro expression study indicated that the ATP-sensitive K(+) (KATP) channel with the S422L mutation has the gain-of-function with reduced sensitivity to ATP. However, the electrophysiological impact of KCNJ8 has not been elucidated in vivo. Transgenic mouse strains overexpressing KCNJ8 S422L variant (TGmt) or WT (TGWT) in cardiomyocytes have been created to investigate the influence of KCNJ8 in cardiomyocytes and the JWS-related feature of the S422L variant on the cardiac electrophysiology...
September 6, 2017: Journal of Pharmacological Sciences
https://www.readbyqxmd.com/read/28904293/characteristics-of-electromechanical-window-in-anesthetized-rabbit-models-of-short-qt-and-long-qt-syndromes
#3
Vudhiporn Limprasutr, Nakkawee Saengklub, Pradtana Meedech, Anusak Kijtawornrat, Robert L Hamlin
The current regulatory guidelines recommend the use of QT interval to assess the risk of arrhythmogenic potential of new chemical entities. Recently, the electromechanical window (EMW), the difference in duration between electrical and mechanical systole, has been proposed as markers for drug-induced torsades de pointes (TdP); however, data of EMW in short QT model are not available. This study aimed to characterize the EMW as a marker for drug-induced ventricular arrhythmias in anesthetized rabbit model of long QT syndrome type 2 (LQT2) and short QT syndrome (SQTS) infused with reference compounds known to lengthen or shorten QT intervals...
2017: Journal of Toxicological Sciences
https://www.readbyqxmd.com/read/28894151/the-effects-of-ageing-and-adrenergic-challenge-on-electrocardiographic-phenotypes-in-a-murine-model-of-long-qt-syndrome-type-3
#4
Karan R Chadda, Shiraz Ahmad, Haseeb Valli, Ingrid den Uijl, Ali Bak Al-Hadithi, Samantha C Salvage, Andrew A Grace, Christopher L-H Huang, Kamalan Jeevaratnam
Long QT Syndrome 3 (LQTS3) arises from gain-of-function Nav1.5 mutations, prolonging action potential repolarisation and electrocardiographic (ECG) QT interval, associated with increased age-dependent risk for major arrhythmic events, and paradoxical responses to β-adrenergic agents. We investigated for independent and interacting effects of age and Scn5a+/ΔKPQ genotype in anaesthetised mice modelling LQTS3 on ECG phenotypes before and following β-agonist challenge, and upon fibrotic change. Prolonged ventricular recovery was independently associated with Scn5a+/ΔKPQ and age...
September 11, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28856762/pregbalin-induced-recurrent-syncopal-attacks-with-prolong-qt-interval
#5
Adem Adar, Fahri Cakan, Orhan Önalan
Long QT syndrome may lead to fatal dysrhythmia. Prolongation of QT interval due to pregabalin has been shown in rats and no data is available in humans. We report a 80-year-old female patient using pregabalin. She was presented to emergency room with syncope attacks. Her admission electrocardiography demonstrated prolonged QT interval. After excluding the possible causes of the long QT syndrome, we attributed prolonged QT interval to pregabalin therapy. After discontinuation of pregabalin QT interval returned to normal range and patient experienced no further syncope attacks...
August 30, 2017: Annals of Noninvasive Electrocardiology
https://www.readbyqxmd.com/read/28844584/consistency-of-psychotropic-drug-drug-interactions-listed-in-drug-monographs
#6
REVIEW
Xinyue Liu, Randy C Hatton, Yanmin Zhu, Juan M Hincapie-Castillo, Regina Bussing, Marie Barnicoat, Almut G Winterstein
BACKGROUND: With an increasing prevalence of psychotropic polypharmacy, clinicians depend on drug-drug interaction (DDI) references to ensure safe regimens, but the consistency of such information is frequently questioned. OBJECTIVES: To evaluate the consistency of psychotropic DDIs documented in Clinical Pharmacology (CP), Micromedex (MM), and Lexicomp (LC) and summarize consistent psychotropic DDIs. METHODS: In May 2016, we extracted severe or major psychotropic DDIs for 102 psychotropic drugs, including central nervous system (CNS) stimulants, antidepressants, an antimanic agent (lithium), antipsychotics, anticonvulsants, and anxiolytics-sedatives-hypnotics from CP, MM, and LC...
August 22, 2017: Journal of the American Pharmacists Association: JAPhA
https://www.readbyqxmd.com/read/28820736/long-qt-syndrome-diagnosed-in-two-sisters-with-propionic-acidemia-a-case-report
#7
Ensar Duras, Ahmet İrdem, Ozan Özkaya
Propionic acidemia (PA) is a rare autosomal recessive metabolic disorder caused by deficiency of the mitochondrial enzyme propionyl-CoA carboxylase (PCC). This disorder mostly progresses with episodes of metabolic acidosis. Cardiomyopathy is among the cardiac complications known to occur during metabolic decompensation episodes. However, several recent papers emphasized the association of PA and long QT syndrome (LQTS) which may lead to extremely serious and fatal consequences. In this report, we describe two sisters with PA who have prolonged QT duration that were incidentally detected in an outpatient setting...
August 18, 2017: Journal of Pediatric Endocrinology & Metabolism: JPEM
https://www.readbyqxmd.com/read/28812984/modelling-the-effects-of-quinidine-disopyramide-and-e-4031-on-short-qt-syndrome-variant-3-in-the-human-ventricles
#8
Cunjin Luo, Kuanquan Wang, Henggui Zhang
Short QT syndrome (SQTS) is an inherited cardiac channelopathy, but at present little information is available on its pharmacological treatment. SQT3 variant (linked to the inward rectifier potassium current IK1) of SQTS, results from a gain-of-function mutation (Kir2.1 D172N) in the KCNJ2-encoded channels, which is associated with ventricular fibrillation (VF). Using biophysically-detailed human ventricular computer models, this study investigated the potential effects of quinidine, disopyramide, and E-4031 on SQT3...
August 16, 2017: Physiological Measurement
https://www.readbyqxmd.com/read/28808933/cardiovascular-disease-in-clozapine-treated-patients-evidence-mechanisms-and-management
#9
REVIEW
Kathlyn J Ronaldson
Myocarditis occurs in about 3% of those initiated on clozapine but monitoring reduces the risk of serious outcome. Cardiomyopathy may develop after myocarditis, or from prolonged tachycardia. Monitoring using echocardiography is not deemed cost effective. Tachycardia, orthostatic hypotension and reduced heart rate variability are a group of clozapine-related adverse effects associated with autonomic dysfunction and may have serious consequences in the long term. Elevated heart rate and poor heart rate variability can be treated with a β-blocker or a non-dihydropyridine calcium channel blocker, while orthostatic hypotension can be alleviated by increased fluid intake and abdominal binding, but may require pharmacological intervention...
August 14, 2017: CNS Drugs
https://www.readbyqxmd.com/read/28794082/loss-of-function-kcne2-variants-true-monogenic-culprits-of-long-qt-syndrome-or-proarrhythmic-variants-requiring-secondary-provocation
#10
Jason D Roberts, Andrew D Krahn, Michael J Ackerman, Ram K Rohatgi, Arthur J Moss, Babak Nazer, Rafik Tadros, Brenda Gerull, Shubhayan Sanatani, Yanushi D Wijeyeratne, Alban-Elouen Baruteau, Alison R Muir, Benjamin Pang, Julia Cadrin-Tourigny, Mario Talajic, Lena Rivard, David J Tester, Taylor Liu, Isaac R Whitman, Julianne Wojciak, Susan Conacher, Lorne J Gula, Peter Leong-Sit, Jaimie Manlucu, Martin S Green, Robert Hamilton, Jeff S Healey, Coeli M Lopes, Elijah R Behr, Arthur A Wilde, Michael H Gollob, Melvin M Scheinman
BACKGROUND: Insight into type 6 long-QT syndrome (LQT6), stemming from mutations in the KCNE2-encoded voltage-gated channel β-subunit, is limited. We sought to further characterize its clinical phenotype. METHODS AND RESULTS: Individuals with reported pathogenic KCNE2 mutations identified during arrhythmia evaluation were collected from inherited arrhythmia clinics and the Rochester long-QT syndrome (LQTS) registry. Previously reported LQT6 cases were identified through a search of the MEDLINE database...
August 2017: Circulation. Arrhythmia and Electrophysiology
https://www.readbyqxmd.com/read/28782696/heritability-in-a-scn5a-mutation-founder-population-with-increased-female-susceptibility-to-non-nocturnal-ventricular-tachyarrhythmia-and-sudden-cardiac-death
#11
Rachel M A Ter Bekke, Aaron Isaacs, Andrei Barysenka, Marije B Hoos, Jan D H Jongbloed, Jan C A Hoorntje, Alfons S M Patelski, Apollonia T J M Helderman-van den Enden, Arthur van den Wijngaard, Monika Stoll, Paul G A Volders
BACKGROUND: Heritable cardiac-sodium channel dysfunction is associated with various arrhythmia syndromes, some predisposing to ventricular fibrillation. Phenotypic diversity among carriers of identical-by-descent mutations is often remarkable, suggesting influences of genetic modifiers. OBJECTIVE: The purpose of this study was to identify a unique SCN5A-mutation founder population with mixed clinical phenotypes and sudden cardiac death, and to investigate the heritability of electromechanical traits besides the SCN5A-mutation effect...
August 3, 2017: Heart Rhythm: the Official Journal of the Heart Rhythm Society
https://www.readbyqxmd.com/read/28768059/-epicatechin-rescues-the-as2-o3-induced-herg-k-channel-deficiency-possibly-through-upregulating-transcription-factor-sp1-expression
#12
Zengxiang Dong, Yuanqi Shi, Lifang Feng, Zhaoqian Shen, Li Fang, Sijia Zheng, Xin Hai, Baoxin Li
(-)-Epicatechin (EPI) has beneficial effects on the cardiovascular disease. The human ether-a-go-go-related gene (HERG) potassium channel is crucial for repolarization of cardiac action potential. Dysfunction of the HERG channel can cause long QT syndrome type 2 (LQT2). Arsenic trioxide (As2 O3 ) has shown efficacy in the treatment of acute promyelocytic leukemia. However, As2 O3 can induce the deficiency of HERG channel and cause LQT2. In this study, we examined whether EPI could rescue the As2 O3 -induced HERG channel deficiency...
August 2, 2017: Journal of Biochemical and Molecular Toxicology
https://www.readbyqxmd.com/read/28765088/the-evolving-role-of-ankyrin-b-in-cardiovascular-disease
#13
Sara N Koenig, Peter J Mohler
Over the past decade, ankyrin-B has been identified as a prominent player in cardiac physiology. Ankyrin-B has a multitude of functions, with roles in expression, localization, and regulation of proteins critical for cardiac excitability, cytoskeletal integrity, and signaling. Furthermore, human ANK2 variants that result in ankyrin-B loss of function are associated with "ankyrin-B syndrome," a complex cardiac phenotype that may include bradycardia and heart rate variability, conduction block, atrial fibrillation, QT interval prolongation, and potentially fatal catecholaminergic polymorphic ventricular tachycardia...
July 29, 2017: Heart Rhythm: the Official Journal of the Heart Rhythm Society
https://www.readbyqxmd.com/read/28762916/-predictors-of-electrical-myocardial-instability-in-men-with-arterial-hypertension-and-obstructive-sleep-apnea-hypopnea-syndrome
#14
O V Lyshova, N V Borodin, I I Kostenko
OBJECTIVE: To access the detectability of corrected QT interval (QTc) prolongation ( more or equal 450 ms) and increased QT dispersion (QTd more or equal 60 ms) in men with obstructive sleep apnea-hypopnea syndrome (OSAS) and newly diagnosed arterial hypertension (AH). MATERIAL AND METHODS: We included in this study 138 men (mean age 40.5+/-6.9 years, body mass index 34.5+/-4.7 kg/m2, all with more or equal 3 risk factors of AH development, 34% with subclinical target organs damage)...
May 2017: Kardiologiia
https://www.readbyqxmd.com/read/28734073/differential-calcium-sensitivity-in-nav-1-5-mixed-syndrome-mutants
#15
Mena Abdelsayed, Alban-Elouen Baruteau, Karen Gibbs, Shubhayan Sanatani, Andrew D Krahn, Vincent Probst, Peter C Ruben
KEY POINTS: SCN5a mutations may express gain-of-function (Long QT Syndrome-3), loss-of-function (Brugada Syndrome 1) or both (mixed syndromes), depending on the mutation and environmental triggers. One such trigger may be an increase in cytosolic calcium, accompanying exercise. Many mixed syndromes mutants, including ∆KPQ, E1784K, 1795insD and Q1909R, are found in calcium-sensitive regions. Elevated cytosolic calcium attenuates gain-of-function properties in ∆KPQ, 1795insD and Q1909R, but not in E1784K...
September 15, 2017: Journal of Physiology
https://www.readbyqxmd.com/read/28728494/the-clinical-significance-of-qt-prolongation-associated-with-tamoxifen-a-review-of-the-literature
#16
Katherine Fung, Julia Imeson, Frances Cusano
Objective To review the literature discussing QT prolongation associated with the use of tamoxifen in order to evaluate the clinical significance. Data sources A search of PubMed (1946 to 2017), MEDLINE (1946 to 2017) and EMBASE (1947 to 2017) was performed using a combination of the following search terms: tamoxifen, estrogen antagonist, selective estrogen receptor modulator, QT prolongation, QT interval, long QT syndrome and torsades de pointes. All searches were limited to human subjects. Reference lists of the literature found were also reviewed but did not reveal any further articles...
January 1, 2017: Journal of Oncology Pharmacy Practice
https://www.readbyqxmd.com/read/28720088/sex-is-a-moderator-of-the-association-between-nos1ap-sequence-variants-and-qtc-in-two-long-qt-syndrome-founder-populations-a-pedigree-based-measured-genotype-association-analysis
#17
Annika Winbo, Eva-Lena Stattin, Ida Maria Westin, Anna Norberg, Johan Persson, Steen M Jensen, Annika Rydberg
BACKGROUND: Sequence variants in the NOS1AP gene have repeatedly been reported to influence QTc, albeit with moderate effect sizes. In the long QT syndrome (LQTS), this may contribute to the substantial QTc variance seen among carriers of identical pathogenic sequence variants. Here we assess three non-coding NOS1AP sequence variants, chosen for their previously reported strong association with QTc in normal and LQTS populations, for association with QTc in two Swedish LQT1 founder populations...
July 18, 2017: BMC Medical Genetics
https://www.readbyqxmd.com/read/28710299/takotsubo-cardiomyopathy-related-complete-heart-block-and-torsades-de-pointes
#18
Faisal Inayat, Hafeez Ul Hassan Virk, Waqas Ullah, Iqra Riaz
Takotsubo cardiomyopathy (TCM) is a relatively recently recognised clinical entity. It frequently mimics acute coronary syndrome and is accompanied by reversible left ventricular apical ballooning in the absence of angiographically significant coronary artery stenosis. TCM is a reversible condition, and the prognosis is usually well. However, in rare instances, it can be associated with life-threatening arrhythmic complications. Herein, we report the case of a patient with TCM who developed complete atrioventricular block followed by QT prolongation and torsades de pointes...
July 14, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28679017/a-comparison-of-qtc-intervals-in-alcohol-withdrawal-patients-versus-acute-coronary-syndrome-patients
#19
COMPARATIVE STUDY
Timothy Chu, Keith Azevedo, Amy A Ernst, Dusadee Sarangarm, Steven J Weiss
OBJECTIVES: Patients with an alcohol use disorder experiencing acute intoxication or withdrawal may be at risk for electrocardiograph (ECG) abnormalities, including prolongation of repolarization (long QTc [corrected QT]) that may contribute to arrhythmias and may be associated with a threefold increase in the likelihood of sudden cardiac events. Patients with acute coronary syndrome may have prolonged QTc as well. To our knowledge, no previous studies have compared the QTc of ACS with acute ethanol (EtOH) withdrawal syndromes in the emergency department (ED)...
July 2017: Southern Medical Journal
https://www.readbyqxmd.com/read/28670758/congenital-long-qt-syndrome-and-torsade-de-pointes
#20
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...
July 2, 2017: Annals of Noninvasive Electrocardiology
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