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https://www.readbyqxmd.com/read/29040441/advancing-precision-medicine-for-the-treatment-of-long-qt-syndrome-type-2-shedding-light-on-lumacaftor
#1
Brian P Delisle, Craig T January
No abstract text is available yet for this article.
October 11, 2017: European Heart Journal
https://www.readbyqxmd.com/read/29036623/predictors-of-mortality-in-high-risk-patients-with-qt-prolongation-in-a-community-hospital
#2
Charlotte Gibbs, Jacob Thalamus, Kristian Heldal, Øystein Lunde Holla, Kristina H Haugaa, Jan Hysing
Aims: To determine predictors of mortality in patients with corrected QT interval (QTc) ≥ 500 ms in a community hospital. Methods and results: In this retrospective observational study, we searched the electrocardiogram (ECG) database at Telemark Hospital Trust, Norway, from January 2004 to December 2014. Medication, electrolyte abnormalities, and medical conditions known to prolong the QT interval were recorded. From the medical records, we assessed whether the prolonged QTc was noted by the health care providers...
October 3, 2017: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
https://www.readbyqxmd.com/read/29036559/the-brisk-standing-test-for-long-qt-syndrome-in-prepubertal-school-children-defining-normal
#3
L H P M Filippini, P G Postema, K Zoubin, B J M Hermans, N A Blom, T Delhaas, A A M Wilde
Aims: Long QT syndrome (LQTS) is associated with malignant arrhythmias and sudden death from birth to advanced age. Prolongation of the QT-interval, may however be concealed on standard electrocardiograms (ECG). The brisk-standing-test (BST) was developed to guide LQTS-diagnosis and treatment in adults. We hypothesized that the BST may be used in prepubertal children to identify LQTS subjects. Accordingly, reference values for the BST should be available to prevent incorrect diagnosis and treatment of LQTS...
October 3, 2017: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
https://www.readbyqxmd.com/read/29036457/feasibility-evaluation-of-long-term-use-of-beta-blockers-and-calcium-antagonists-in-patients-with-brugada-syndrome
#4
Tsukasa Kamakura, Mitsuru Wada, Kohei Ishibashi, Yuko Y Inoue, Koji Miyamoto, Hideo Okamura, Satoshi Nagase, Takashi Noda, Takeshi Aiba, Satoshi Yasuda, Wataru Shimizu, Shiro Kamakura, Kengo Kusano
Aims: Beta-blockers (BBs) and calcium antagonists (CAs) are reported to aggravate ST-segment elevation in some patients with Brugada syndrome (BrS). The feasibility of their long-term use in BrS still remains unknown. We investigated the safety of long-term use of BB and CA in BrS patients. Methods and results: Of the 360 consecutive BrS patients, 29 [5: a history of ventricular fibrillation (VF), 17: syncope, 7: asymptomatic] took BB and/or CA (BB: 22, CA: 8) for more than 1 year for the treatment of co-morbidities such as atrial tachyarrhythmia, vasospastic angina, and neurally mediated syncope...
September 23, 2017: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
https://www.readbyqxmd.com/read/29035985/qt-corrections-for-long-qt-risk-assessment-implications-for-the-preparticipation-examination
#5
David Hadley, David Hsu, David Pickham, Jonathan A Drezner, Victor F Froelicher
BACKGROUND: Because sudden cardiac death (SCD) in the young mainly occur in individuals with structurally normal hearts, improved screening techniques for detecting inherited arrhythmic diseases are needed. The QT interval is an important screening measurement; however, the criteria for detecting an abnormal QT interval are based on Bazett formula and older populations. OBJECTIVE: To define the normal upper limits for QT interval from the electrocardiograms (ECGs) of healthy young individuals, compare the major correction formula and propose new QT interval thresholds for detecting those at risk of SCD...
October 10, 2017: Clinical Journal of Sport Medicine: Official Journal of the Canadian Academy of Sport Medicine
https://www.readbyqxmd.com/read/29033053/identification-and-characterization-of-a-novel-recessive-kcnq1-mutation-associated-with-romano-ward-long-qt-syndrome-in-two-iranian-families
#6
Zahra Zafari, Mohammad Dalili, Sirus Zeinali, Siamak Saber, Amir Farjam Fazeli Far, Mohammad Taghi Akbari
BACKGROUND: One of the foremost causes of sudden cardiac death in the young is an inherent cardiac arrhythmia known as Long-QT syndrome (LQTS). Whereas heterozygous mutations typically lead to the Romano-Ward type of LQTS, We have provided a further evidence for the recessive transmission of a novel KCNQ1 gene mutation in two consanguineous families for the first time in Iran. METHODS: Next generation sequencing, DNA Sanger sequencing and haplotype analysis were performed for genotype determination...
July 12, 2017: Journal of Electrocardiology
https://www.readbyqxmd.com/read/29032483/regulation-of-cardiac-voltage-gated-sodium-channel-by-kinases-roles-of-protein-kinases-a-and-c
#7
Ademuyiwa S Aromolaran, Mohamed Chahine, Mohamed Boutjdir
In the heart, voltage-gated sodium (Nav) channel (Nav1.5) is defined by its pore-forming α-subunit and its auxiliary β-subunits, both of which are important for its critical contribution to the initiation and maintenance of the cardiac action potential (AP) that underlie normal heart rhythm. The physiological relevance of Nav1.5 is further marked by the fact that inherited or congenital mutations in Nav1.5 channel gene SCN5A lead to altered functional expression (including expression, trafficking, and current density), and are generally manifested in the form of distinct cardiac arrhythmic events, epilepsy, neuropathic pain, migraine, and neuromuscular disorders...
October 15, 2017: Handbook of Experimental Pharmacology
https://www.readbyqxmd.com/read/29031413/experiences-in-digitizing-and-digitally-measuring-a-paper-based-ecg-archive
#8
Arttu Holkeri, Antti Eranti, Tuomas V Kenttä, Kai Noponen, M Anette E Haukilahti, Tapio Seppänen, M Juhani Junttila, Tuomas Kerola, Harri Rissanen, Markku Heliövaara, Paul Knekt, Aapo L Aro, Heikki V Huikuri
BACKGROUND: No established method for digitizing and digital measuring of paper electrocardiograms (ECG) exists. We describe a paper ECG digitizing and digital measuring process, and report comparability to manual measurements. METHODS: A paper ECG was recorded from 7203 health survey participants in 1978-1980. With specific software, the ECGs were digitized (ECG Trace Tool), and measured digitally (EASE). A sub-sample of 100 ECGs was selected for manual measurements...
September 27, 2017: Journal of Electrocardiology
https://www.readbyqxmd.com/read/29030380/association-between-qt-interval-components-and-sudden-cardiac-death-the-aric-study-atherosclerosis-risk-in-communities
#9
Wesley T O'Neal, Matthew J Singleton, Jason D Roberts, Larisa G Tereshchenko, Nona Sotoodehnia, Lin Y Chen, Gregory M Marcus, Elsayed Z Soliman
BACKGROUND: Several reports have demonstrated that prolongation of the QT interval is associated with sudden cardiac death (SCD). However, it is unknown whether any of the components within the QT interval are responsible for its association with SCD. METHODS AND RESULTS: We examined the association of the individual QT-interval components (R-wave onset to R-peak, R-peak to R-wave end, ST-segment, T-wave onset to T-peak, and T-peak to T-wave end) with SCD in 12 241 participants (54±5...
October 2017: Circulation. Arrhythmia and Electrophysiology
https://www.readbyqxmd.com/read/29029798/effect-of-intravenous-ondansetron-on-qtc-interval-in-children-with-gastroenteritis
#10
Robert J Hoffman, Khalid Alansari
BACKGROUND: The potential for ondansetron to cause QT prolongation and fatal dysrhythmia is well-reported, including a 2011 FDA report on the topic. Few clinical trials evaluating this phenomenon in the ED setting exist, and only one is pediatric. OBJECTIVE: We have sought to determine the effect of a standardized dose of intravenous ondansetron on the QTc duration of children under 14years of age treated for gastroenteritis-associated vomiting in a pediatric ED...
October 4, 2017: American Journal of Emergency Medicine
https://www.readbyqxmd.com/read/29029613/right-precordial-directed-electrocardiographical-markers-identify-arrhythmogenic-right-ventricular-cardiomyopathy-in-the-absence-of-conventional-depolarization-or-repolarization-abnormalities
#11
Daniel Cortez, Anneli Svensson, Jonas Carlson, Sharon Graw, Nandita Sharma, Francesca Brun, Anita Spezzacatene, Luisa Mestroni, Pyotr G Platonov
BACKGROUND: Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) carries a risk of sudden death. We aimed to assess whether vectorcardiographic (VCG) parameters directed toward the right heart and a measured angle of the S-wave would help differentiate ARVD/C with otherwise normal electrocardiograms from controls. METHODS: Task Force 2010 definite ARVD/C criteria were met for all patients. Those who did not fulfill Task Force depolarization or repolarization criteria (-ECG) were compared with age and gender-matched control subjects...
October 13, 2017: BMC Cardiovascular Disorders
https://www.readbyqxmd.com/read/29024579/impact-of-the-norepinephrine-prodrug-droxidopa-on-the-qtc-interval-in-healthy-individuals
#12
William B White, L Arthur Hewitt, Ali A Mehdirad
A double-blind, 4-period crossover study (NCT01327066) was conducted to assess the effect of the novel norepinephrine prodrug droxidopa on the QT interval in in healthy subjects. Subjects were randomized to receive a single dose of droxidopa 600 mg (maximal dose) and 2000 mg (supratherapeutic dose) compared with the positive control, moxifloxacin 400 mg, and placebo, each separated by a 3-day washout period. Patients were monitored by continuous Holter monitoring, and electrocardiograms (ECGs) were extracted 0...
October 11, 2017: Clinical Pharmacology in Drug Development
https://www.readbyqxmd.com/read/29023952/pharmacokinetics-and-electrophysiological-effects-of-sotalol-hydrochloride-in-horses
#13
B Broux, D De Clercq, A Decloedt, L Vera, M Devreese, R Gehring, S Croubels, G van Loon
BACKGROUND: Arrhythmias in horses may require long term anti-arrhythmic therapy. Unfortunately, oral anti-arrhythmic drugs for use in horses are currently scarce. In human patients and small animals, sotalol, a β-blocker with class III anti-arrhythmic properties, is often used for long-term treatment. OBJECTIVES: To determine the pharmacokinetics of sotalol at multiple oral dosages in unfasted horses, as well as the effects on electro- and echocardiographic measurements, right atrial and ventricular monophasic action potential (MAP) and effective refractory period (ERP)...
October 10, 2017: Equine Veterinary Journal
https://www.readbyqxmd.com/read/29021868/management-of-patients-with-long-qt-syndrome
#14
Serkan Cay, Ozcan Ozeke, Firat Ozcan, Serhat Koca, Aysenur Pac, Dursun Aras, Serkan Topaloglu
No abstract text is available yet for this article.
October 2017: Journal of Arrhythmia
https://www.readbyqxmd.com/read/29021866/criteria-for-short-qt-interval-based-on-a-new-qt-heart-rate-adjustment-formula
#15
Simon W Rabkin
BACKGROUND: A short QT interval, within which an increased risk for atrial fibrillation and/or fatal cardiac arrhythmias occurs, has been difficult to define. METHODS: The lower percentiles of a new QTc formula were determined, using a precise mathematical fitting of the QT-heart rate relationship from the ECGs of 13,600 individuals from the NHANES II and III surveys. RESULTS: The QTc interval for persons in the lower fifth percentile, second (2...
October 2017: Journal of Arrhythmia
https://www.readbyqxmd.com/read/29021858/landiolol-suppression-of-electrical-storm-of-torsades-de-pointes-in-patients-with-congenital-long-qt-syndrome-type-2-and-myocardial-ischemia
#16
Ryota Kitajima, Takeshi Aiba, Tsukasa Kamakura, Kohei Ishibashi, Mitsuru Wada, Yuko Inoue, Koji Miyamoto, Hideo Okamura, Takashi Noda, Satoshi Nagase, Yu Kataoka, Yasuhide Asaumi, Teruo Noguchi, Satoshi Yasuda, Kengo Kusano
A 76-year-old man who had been diagnosed with long-QT syndrome type 2 had frequent syncopal attacks. The electrocardiogram was monitored, and frequent torsades de pointes (TdP) was detected despite administration of conventional medications: oral propranolol, verapamil, intravenous magnesium sulfate, verapamil, and lidocaine. In contrast, 2 μg/kg/min landiolol could completely suppress TdP. Subsequently, an implantable cardioverter defibrillator was placed, and he was diagnosed with silent myocardial ischemia using myocardial perfusion scintigraphy and coronary angiography...
October 2017: Journal of Arrhythmia
https://www.readbyqxmd.com/read/29021308/machine-learning-and-rare-variant-adjudication-in-type-1-long-qt-syndrome
#17
EDITORIAL
John R Giudicessi
No abstract text is available yet for this article.
October 2017: Circulation. Cardiovascular Genetics
https://www.readbyqxmd.com/read/29021306/characterization-of-a-human-induced-pluripotent-stem-cell-derived-cardiomyocyte-model-for-the-study-of-variant-pathogenicity-validation-of-a-kcnj2-mutation
#18
Roselle Gélinas, Nabil El Khoury, Marie-A Chaix, Claudine Beauchamp, Azadeh Alikashani, Nathalie Ethier, Gabrielle Boucher, Louis Villeneuve, Laura Robb, Frédéric Latour, Blandine Mondesert, Lena Rivard, Philippe Goyette, Mario Talajic, Céline Fiset, John David Rioux
BACKGROUND: Long-QT syndrome is a potentially fatal condition for which 30% of patients are without a genetically confirmed diagnosis. Rapid identification of causal mutations is thus a priority to avoid at-risk situations that can lead to fatal cardiac events. Massively parallel sequencing technologies are useful for the identification of sequence variants; however, electrophysiological testing of newly identified variants is crucial to demonstrate causality. Long-QT syndrome could, therefore, benefit from having a standardized platform for functional characterization of candidate variants in the physiological context of human cardiomyocytes...
October 2017: Circulation. Cardiovascular Genetics
https://www.readbyqxmd.com/read/29021305/predicting-the-functional-impact-of-kcnq1-variants-of-unknown-significance
#19
Bian Li, Jeffrey L Mendenhall, Brett M Kroncke, Keenan C Taylor, Hui Huang, Derek K Smith, Carlos G Vanoye, Jeffrey D Blume, Alfred L George, Charles R Sanders, Jens Meiler
BACKGROUND: An emerging standard-of-care for long-QT syndrome uses clinical genetic testing to identify genetic variants of the KCNQ1 potassium channel. However, interpreting results from genetic testing is confounded by the presence of variants of unknown significance for which there is inadequate evidence of pathogenicity. METHODS AND RESULTS: In this study, we curated from the literature a high-quality set of 107 functionally characterized KCNQ1 variants. Based on this data set, we completed a detailed quantitative analysis on the sequence conservation patterns of subdomains of KCNQ1 and the distribution of pathogenic variants therein...
October 2017: Circulation. Cardiovascular Genetics
https://www.readbyqxmd.com/read/29020391/association-between-mitochondrial-dna-copy-number-and-sudden-cardiac-death-findings-from-the-atherosclerosis-risk-in-communities-study-aric
#20
Yiyi Zhang, Eliseo Guallar, Foram N Ashar, Ryan J Longchamps, Christina A Castellani, John Lane, Megan L Grove, Josef Coresh, Nona Sotoodehnia, Leonard Ilkhanoff, Eric Boerwinkle, Nathan Pankratz, Dan E Arking
Aims: Sudden cardiac death (SCD) is a major public health burden. Mitochondrial dysfunction has been implicated in a wide range of cardiovascular diseases including cardiomyopathy, heart failure, and arrhythmias, but it is unknown if it also contributes to SCD risk. We sought to examine the prospective association between mtDNA copy number (mtDNA-CN), a surrogate marker of mitochondrial function, and SCD risk. Methods and results: We measured baseline mtDNA-CN in 11 093 participants from the Atherosclerosis Risk in Communities (ARIC) study...
June 30, 2017: European Heart Journal
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