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Prolonged QT syndrome

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https://www.readbyqxmd.com/read/28728494/the-clinical-significance-of-qt-prolongation-associated-with-tamoxifen-a-review-of-the-literature
#1
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
#2
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
#3
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
#4
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
#5
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
https://www.readbyqxmd.com/read/28654209/sensitivity-and-reliability-of-halothane-anaesthetized-microminipigs-to-assess-risk-of-drug-induced-long-qt-syndrome
#6
Xin Cao, Takeshi Wada, Yuji Nakamura, Suchitra Matsukura, Hiroko Izumi-Nakaseko, Kentaro Ando, Atsuhiko T Naito, Atsushi Sugiyama
Using moxifloxacin and terfenadine, which are known to induce benign and malignant QT interval prolongation, respectively, we analysed whether halothane-anaesthetized microminipigs are an appropriate model for assessing the risk of drug-induced long QT syndrome. Moxifloxacin (0.03, 0.3 and 3 mg/kg) and terfenadine (0.03, 0.3 and 3 mg/kg) were intravenously infused over 10 min. with a pause of 20 min. to the halothane-anaesthetized microminipigs (n=4 for each drug). Moxifloxacin decreased the heart rate, whereas it increased the blood pressure in a dose-related manner...
June 27, 2017: Basic & Clinical Pharmacology & Toxicology
https://www.readbyqxmd.com/read/28648896/inhibition-of-cdk5-alleviates-the-cardiac-phenotypes-in-timothy-syndrome
#7
LouJin Song, Seon-Hye E Park, Yehuda Isseroff, Kumi Morikawa, Masayuki Yazawa
L-type calcium channel CaV1.2 plays an essential role in cardiac function. The gain-of-function mutations in CaV1.2 have been reported to be associated with Timothy syndrome, a disease characterized by QT prolongation and syndactyly. Previously we demonstrated that roscovitine, a cyclin-dependent kinase (CDK) inhibitor, could rescue the phenotypes in induced pluripotent stem cell-derived cardiomyocytes from Timothy syndrome patients. However, exactly how roscovitine rescued the phenotypes remained unclear. Here we report a mechanism potentially underlying the therapeutic effects of roscovitine on Timothy syndrome cardiomyocytes...
July 11, 2017: Stem Cell Reports
https://www.readbyqxmd.com/read/28645448/takotsubo-syndrome-presenting-as-syncope-in-a-patient-with-permanent-pacemaker
#8
Bharatraj Banavalikar, Sivasankaran Sivasubramonian, Narayanan Namboodiri, Krishna Kumar Mohanan Nair, Dibya Ranjan Behera, Ajitkumar Valaparambil
Takotsubo syndrome is an acute reversible heart failure syndrome triggered by physical or emotional stress, especially in postmenopausal women. Herein, we describe a case of a 31-year-old pacemaker dependent lady who presented with syncope after she was bereaved of her father. Electrocardiogram at admission revealed ventricular paced rhythm at 60/min, prolonged QT interval and frequent runs of torsades de pointes. Transthoracic echocardiogram and left ventriculogram revealed findings typical of Takotsubo syndrome...
May 18, 2017: Journal of Electrocardiology
https://www.readbyqxmd.com/read/28632743/in-silico-assessment-of-the-effects-of-quinidine-disopyramide-and-e-4031-on-short-qt-syndrome-variant-1-in-the-human-ventricles
#9
Cunjin Luo, Kuanquan Wang, Henggui Zhang
AIMS: Short QT syndrome (SQTS) is an inherited disorder associated with abnormally abbreviated QT intervals and an increased incidence of atrial and ventricular arrhythmias. SQT1 variant (linked to the rapid delayed rectifier potassium channel current, IKr) of SQTS, results from an inactivation-attenuated, gain-of-function mutation (N588K) in the KCNH2-encoded potassium channels. Pro-arrhythmogenic effects of SQT1 have been well characterized, but less is known about the possible pharmacological antiarrhythmic treatment of SQT1...
2017: PloS One
https://www.readbyqxmd.com/read/28619993/low-extracellular-potassium-prolongs-repolarization-and-evokes-early-afterdepolarization-in-human-induced-pluripotent-stem-cell-derived-cardiomyocytes
#10
Jukka Kuusela, Kim Larsson, Disheet Shah, Chandra Prajapati, Katriina Aalto-Setälä
Long QT syndrome (LQTS) is characterized by a prolonged QT-interval on electrocardiogram and by increased risk of sudden death. One of the most common and potentially life-threatening electrolyte disturbances is hypokalemia, characterized by low concentrations of K(+) Using a multielectrode array platform and current clamp technique, we investigated the effect of low extracellular K(+) concentration ([K(+)]Ex) on the electrophysiological properties of hiPSC-derived cardiomyocytes (CMs) generated from a healthy control subject (WT) and from two symptomatic patients with type 1 of LQTS carrying G589D (LQT1A) or IVS7-2A>G mutation (LQT1B) in KCNQ1 The baseline prolongations of field potential durations (FPDs) and action potential durations (APDs) were longer in LQT1-CMs than in WT-CMs...
June 15, 2017: Biology Open
https://www.readbyqxmd.com/read/28616803/amitriptyline-may-have-possibility-to-induce-brugada-syndrome-rather-than-long-qt-syndrome
#11
Nur Jaharat Lubna, Takeshi Wada, Yuji Nakamura, Koki Chiba, Xin Cao, Hiroko Izumi-Nakaseko, Kentaro Ando, Atsuhiko T Naito, Yoshioki Satoh, Atsushi Sugiyama
Amitriptyline has been reported to induce long QT syndrome in addition to Brugada syndrome. We qualitatively and quantitatively analyzed the potential of amitriptyline to induce these lethal syndromes by using the halothane-anesthetized dogs (n = 6). Amitriptyline was intravenously administered in doses of 0.1, 1 and 10 mg/kg over 10 min every 20 min, which would provide approximately 1, 10 and 100 times higher plasma concentrations than a therapeutic one, respectively. The low dose hardly altered any of the cardiovascular variables...
June 14, 2017: Cardiovascular Toxicology
https://www.readbyqxmd.com/read/28613240/an-isoform-of-nedd4-2-plays-a-pivotal-role-in-electrophysiological-cardiac-abnormalities
#12
Shintaro Minegishi, Tomoaki Ishigami, Hisho Kawamura, Tabito Kino, Lin Chen, Rie Nakashima-Sasaki, Hiroshi Doi, Kengo Azushima, Hiromichi Wakui, Yumi Chiba, Kouichi Tamura
We have previously shown that neural precursor cell-expressed developmentally downregulated gene 4-2 (Nedd4-2) isoforms with a C2 domain are closely related to ubiquitination of epithelial sodium channel (ENaC), resulting in salt-sensitive hypertension by Nedd4-2 C2 targeting in mice. The sodium voltage-gated channel alpha subunit 5 (SCN5A) gene encodes the α subunit of the human cardiac voltage-gated sodium channel (I Na), and the potassium voltage-gated channel subfamily H member 2 (KCNH2) gene encodes rapidly activating delayed rectifier K channels (I Kr)...
June 14, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/28606656/divergent-antiarrhythmic-effects-of-resveratrol-and-piceatannol-in-a-whole-heart-model-of-long-qt-syndrome
#13
Christian Ellermann, Julian Wolfes, Simon Kochhäuser, Dirk G Dechering, Florian Reinke, Kristina Wasmer, Lars Eckardt, Gerrit Frommeyer
BACKGROUND: The polyphenol resveratrol and its metabolite piceatannol have beneficial health effects including antiarrhythmic properties in ischemia/reperfusion. The objective of this study was to determine potential antiarrhythmic effects in acquired long-QT-syndrome (LQTS). METHODS AND RESULTS: 26 rabbit hearts were isolated and Langendorff-perfused. The IKr-blocker sotalol (100μM) was infused to mimic LQTS-2. Hearts were assigned to two groups. Sotalol significantly prolonged action potential duration (APD90) and QT-interval in both groups (group 1:APD90: +18ms, p<0...
June 3, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/28600177/compound-heterozygous-kcnq1-mutations-a300t-p535t-in-a-child-with-sudden-unexplained-death-insights-into-possible-molecular-mechanisms-based-on-protein-modeling
#14
Erika Antúnez-Argüelles, Arturo Rojo-Domínguez, Ana Leticia Arregui-Mena, Leonor Jacobo-Albavera, Manlio Fabio Márquez, Pedro Iturralde-Torres, María Teresa Villarreal-Molina
Sudden death in a child is a devastating event with important medical implications for surviving relatives. Because it may be the first manifestation of unknown inherited cardiac disease, molecular autopsy can be helpful to determine the cause of death and identify at risk family members. The aim of the study was to perform a molecular autopsy in a seven year-old girl with sudden unexplained death, to find evidence supporting the possible pathogenicity of mutations identified in inherited cardiac disease genes, and to clinically and genetically assess first-degree relatives...
June 6, 2017: Gene
https://www.readbyqxmd.com/read/28595573/a-novel-kcnq1-nonsense-variant-in-the-isoform-specific-first-exon-causes-both-jervell-and-lange-nielsen-syndrome-1-and-long-qt-syndrome-1-a-case-report
#15
Motoi Nishimura, Marehiko Ueda, Ryota Ebata, Emi Utsuno, Takuma Ishii, Kazuyuki Matsushita, Osamu Ohara, Naoki Shimojo, Yoshio Kobayashi, Fumio Nomura
BACKGROUND: According to previous KCNQ1 (potassium channel, voltage gated, KQT-like subfamily, member 1) gene screening studies, missense variants, but not nonsense or frame-shift variants, cause the majority of long QT syndrome (LQTS; Romano-Ward syndrome [RWS]) 1 cases. Several missense variants are reported to cause RWS by a dominant-negative mechanism, and some KCNQ1 variants can cause both Jervell and Lange-Nielsen Syndrome (JLNS; in an autosomal recessive manner) and LQTS1 (in an autosomal dominant manner), while other KCNQ1 variants cause only JLNS...
June 8, 2017: BMC Medical Genetics
https://www.readbyqxmd.com/read/28592292/effects-of-amiodarone-on-short-qt-syndrome-variant-3-in-human-ventricles-a-simulation-study
#16
Cunjin Luo, Kuanquan Wang, Henggui Zhang
BACKGROUND: Short QT syndrome (SQTS) is a newly identified clinical disorder associated with atrial and/or ventricular arrhythmias and increased risk of sudden cardiac death (SCD). The SQTS variant 3 is linked to D172N mutation to the KCNJ2 gene that causes a gain-of-function to the inward rectifier potassium channel current (I K1), which shortens the ventricular action potential duration (APD) and effective refractory period (ERP). Pro-arrhythmogenic effects of SQTS have been characterized, but less is known about the possible pharmacological treatment of SQTS...
June 7, 2017: Biomedical Engineering Online
https://www.readbyqxmd.com/read/28590366/effect-of-gwas-identified-genetic-variants-on-maximum-qt-interval-in-patients-with-schizophrenia-receiving-antipsychotic-agents-a-24-hour-holter-ecg-study
#17
Junzo Watanabe, Naoki Fukui, Yutaro Suzuki, Takuro Sugai, Shin Ono, Nobuto Tsuneyama, Mami Saito, Misuzu Tajiri, Toshiyuki Someya
BACKGROUND: Users of antipsychotics (APs) have a risk of sudden cardiac death (SCD). Sudden cardiac death in such patients is thought to be largely due to drug-induced QT prolongation. It has been reported that many subjects with drug-induced torsades de pointes (TdP) have risk alleles associated with subclinical congenital long QT syndrome. METHODS: We investigated the effects of the risk alleles associated with long QT on the QT interval in patients receiving APs using 24-hour Holter electrocardiograms to take into account the circadian fluctuation of QT intervals...
August 2017: Journal of Clinical Psychopharmacology
https://www.readbyqxmd.com/read/28588847/a-case-of-long-qt-syndrome-challenges-on-a-bumpy-road
#18
Peter Magnusson, Per-Erik Gustafsson
Beta-agonist treatment during pregnancy may unmask the diagnosis of long QT syndrome. The QT prolongation can result in functional AV block. A history of seizure and/or sudden death in a family member should raise suspicion of ventricular tachycardia. More than one mutation may coexist. Refusal of beta-blocker therapy complicates risk stratification.
June 2017: Clinical Case Reports
https://www.readbyqxmd.com/read/28577045/prevalence-and-risk-factors-of-drug-associated-corrected-qt-prolongation-in-elderly-hospitalized-patients-results-of-a-retrospective-analysis-of-data-obtained-over-6%C3%A2-months
#19
Ophélie Maison, Blandine de la Gastine, Laurent Dayot, Sylvain Goutelle
OBJECTIVES: Little information exists on the frequency and determinants of drug-associated long QT syndrome in older adults. The objectives of this study were to assess the prevalence and identify risk factors of drug-associated long QT syndrome in a population of elderly hospitalized patients. METHODS: This was a retrospective study performed over 6 months in hospital geriatric medicine. Various QT-correction equations were fitted to the individual QT-RR data to evaluate the most appropriate equation...
June 2, 2017: Drugs & Aging
https://www.readbyqxmd.com/read/28576628/incidence-of-torsade-de-pointes-in-a-tertiary-hospital-population
#20
Eline Vandael, Bert Vandenberk, Joris Vandenberghe, Hilde Pincé, Rik Willems, Veerle Foulon
BACKGROUND: Multiple risk factors play a role in the development of QTc-prolongation and Torsade de Pointes (TdP). Cases of TdP are underreported and data on the incidence of TdP is scarce. The aim of this study was to investigate the incidence of TdP in a Belgian university hospital and describe the characteristics of TdP-cases using a risk score. METHODS: All cases from 2011 till 2013 coded with the ICD-9 code 427.1 in the University Hospitals of Leuven were selected...
May 19, 2017: International Journal of Cardiology
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