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Prolonged QT syndrome

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https://www.readbyqxmd.com/read/29160150/electrocardiogram-screening-in-children-with-congenital-sensorineural-hearing-loss-prevalence-and-follow-up-of-abnormalities
#1
Zainab Farzal, Jonathan Walsh, Faisal I Ahmad, Jason Roberts, Sunita J Ferns, And Carlton J Zdanski
Objective The purpose is to determine the prevalence of electrocardiogram (ECG) abnormalities, including borderline and prolonged QT, among screened children with sensorineural hearing loss (SNHL) and to analyze their subsequent medical workup. Study Design Institutional Review Board-approved case series with chart review. Setting Tertiary academic center. Subjects and Methods Cases from 1996 to 2014 involving pediatric patients (N = 1994) with SNHL were analyzed. Abnormal ECGs were categorized as borderline/prolonged QT or other...
November 1, 2017: Otolaryngology—Head and Neck Surgery
https://www.readbyqxmd.com/read/29133340/effects-of-hawthorn-crataegus-pentagyna-leaf-extract-on-electrophysiologic-properties-of-cardiomyocytes-derived-from-human-cardiac-arrhythmia-specific-induced-pluripotent-stem-cells
#2
Sara Pahlavan, Marziyeh Shalchi Tousi, Mahdi Ayyari, Abolfazl Alirezalu, Hassan Ansari, Tomo Saric, Hossein Baharvand
Cardiac arrhythmias are major life-threatening conditions. The landmark discovery of induced pluripotent stem cells has provided a promising in vitro system for modeling hereditary cardiac arrhythmias as well as drug development and toxicity testing. Nowadays, nutraceuticals are frequently used as supplements for cardiovascular therapy. Here we studied the cardiac effects of hawthorn (Crataegus pentagyna) leaf extract using cardiomyocytes (CMs) differentiated from healthy human embryonic stem cells, long QT syndrome type 2 (LQTS2), and catecholaminergic polymorphic ventricular tachycardia type 1 (CPVT1) patient-specific induced pluripotent stem cells...
November 13, 2017: FASEB Journal: Official Publication of the Federation of American Societies for Experimental Biology
https://www.readbyqxmd.com/read/29133066/-qtc-interval-in-the-neonatal-period-in-a-mexican-population-a-pilot-study
#3
Rocio Alejandra Peña-Juárez, Adrián Garcia-Canales, Luis Martin Garrido-García, Cesar Augusto Valerio-Carballo
INTRODUCTION: QT interval prolongation is associated with ventricular arrhythmias and sudden death syndrome. OBJECTIVE: To determine the value of QTc interval in healthy newborns in a general hospital in Jalisco, Mexico, and their outcome during their first months of life. METHODS: The study included healthy newborns from March to November 2016, in the Hospital General of Occidente in Jalisco, Mexico. A 12-lead electrocardiogram was performed at a speed of 25mm/s during the first 48h of life...
November 10, 2017: Archivos de Cardiología de México
https://www.readbyqxmd.com/read/29121487/risk-of-cardiac-events-in-long-qt-syndrome-patients-when-taking-antiseizure-medications
#4
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/29118886/polymorphic-ventricular-tachycardia-secondary-to-subarachnoid-haemorrhage-a-rare-occurrence-in-the-setting-of-normal-qtc
#5
Siddharth Paresh Shah, Priyanka Pitroda, Kinner Patel, Rahul Chandak, Timothy Ford
Subarachnoid hemorrhage (SAH) is a neurologic emergency associated with high mortality rate. Polymorphic ventricular tachycardia (VT) is a rare arrhythmia. It can occur in any setting of a long QT interval and bradycardia. This may result from a cardiomyopathy (both ischemic and non-ischemic), acute coronary ischemia, congenital long QT syndrome, electrolyte disturbances and cerebrovascular diseases. We report a rare case of polymorphic VT of unclear etiology with a normal corrected QT, likely secondary to SAH...
October 2017: Cardiology Research
https://www.readbyqxmd.com/read/29101013/the-congenital-long-qt-syndrome-type-3-an-update
#6
REVIEW
Andrés Ricardo Pérez-Riera, Raimundo Barbosa-Barros, Rodrigo Daminello Raimundo, Marianne Penachini da Costa de Rezende Barbosa, Isabel Cristina Esposito Sorpreso, Luiz Carlos de Abreu
Congenital long QT syndrome type 3 (LQT3) is the third in frequency compared to the 15 forms known currently of congenital long QT syndrome (LQTS). Cardiac events are less frequent in LQT3 when compared with LQT1 and LQT2, but more likely to be lethal; the likelihood of dying during a cardiac event is 20% in families with an LQT3 mutation and 4% with either an LQT1 or an LQT2 mutation. LQT3 is consequence of mutation of gene SCN5A which codes for the Nav1.5 Na(+) channel α-subunit and electrocardiographically characterized by a tendency to bradycardia related to age, prolonged QT/QTc interval (mean QTc value 478 ± 52 ms), accentuated QT dispersion consequence of prolonged ST segment, late onset of T wave and frequent prominent U wave because of longer repolarization of the M cell across left ventricular wall...
October 31, 2017: Indian Pacing and Electrophysiology Journal
https://www.readbyqxmd.com/read/29071820/application-of-multigene-panel-sequencing-in-patients-with-prolonged-rate-corrected-qt-interval-and-no-pathogenic-variants-detected-in-kcnq1-kcnh2-and-scn5a
#7
Soo Hyun Seo, So Yeon Kim, Sung Im Cho, Hyunwoong Park, Seungjun Lee, Jong Moon Choi, Man Jin Kim, Jee Soo Lee, Kyung Jin Ahn, Mi Kyoung Song, Eun Jung Bae, Sung Sup Park, Moon Woo Seong
Long QT syndrome (LQTS) is an inherited cardiac disease characterized by a prolonged heart rate-corrected QT (QTc) interval. We investigated the genetic causes in patients with prolonged QTc intervals who were negative for pathogenic variants in three major LQTS-related genes (KCNQ1, KCNH2, and SCN5A). Molecular genetic testing was performed using a panel including 13 LQTS-related genes and 67 additional genes implicated in other cardiac diseases. Overall, putative genetic causes of prolonged QTc interval were identified in three of the 30 patients (10%)...
January 2018: Annals of Laboratory Medicine
https://www.readbyqxmd.com/read/29060841/modeling-the-effects-of-amiodarone-on-short-qt-syndrome-variant-2-in-the-human-ventricles
#8
Cunjin Luo, Kuanquan Wang, Henggui Zhang
AIMS: The short QT syndrome (SQTS) is a new genetic disorder associated with atrial and ventricular arrhythmias and sudden death. The SQT2, SQTS variant, results from a gain-of-function mutation (V307L) in the KCNQ1-encoded potassium channel. Although pro-arrhythmogenic effects of SQTS have been characterized, less is known about the pharmacology of SQTS. Therefore, this study aims to assess the effects of amiodarone on SQT2. METHODS AND RESULTS: The ten Tusscher et al...
July 2017: Conference Proceedings: Annual International Conference of the IEEE Engineering in Medicine and Biology Society
https://www.readbyqxmd.com/read/29057193/long-qt-syndrome-unveiled-by-a-fatal-combination-of-medications-and-electrolyte-abnormalities
#9
Pooja Sethi, Jennifer Treece, Vandana Pai, Chidinma Onweni
Long QT syndrome (LQTS) can present with syncope and seizure-like activity in the setting of torsades de pointes (TdP) with hemodynamic instability. Electrolyte abnormalities and medications can predispose to TdP in the setting of latent LQTS. An implantable cardioverter defibrillator (ICD) is needed if patients with TdP continue to be symptomatic despite medical treatment. We report a case of a patient who presented with seizures and was found to have prolonged corrected QT interval (QTc). During her admission, she was treated with ondansetron...
August 18, 2017: Curēus
https://www.readbyqxmd.com/read/29046645/computational-cardiac-modeling-reveals-mechanisms-of-ventricular-arrhythmogenesis-in-long-qt-syndrome-type-8-cacna1c-r858h-mutation-linked-to-ventricular-fibrillation
#10
Jieyun Bai, Kuanquan Wang, Yashu Liu, Yacong Li, Cuiping Liang, Gongning Luo, Suyu Dong, Yongfeng Yuan, Henggui Zhang
Functional analysis of the L-type calcium channel has shown that the CACNA1C R858H mutation associated with severe QT interval prolongation may lead to ventricular fibrillation (VF). This study investigated multiple potential mechanisms by which the CACNA1C R858H mutation facilitates and perpetuates VF. The Ten Tusscher-Panfilov (TP06) human ventricular cell models incorporating the experimental data on the kinetic properties of L-type calcium channels were integrated into one-dimensional (1D) fiber, 2D sheet, and 3D ventricular models to investigate the pro-arrhythmic effects of CACNA1C mutations by quantifying changes in intracellular calcium handling, action potential profiles, action potential duration restitution (APDR) curves, dispersion of repolarization (DOR), QT interval and spiral wave dynamics...
2017: Frontiers in Physiology
https://www.readbyqxmd.com/read/29037160/-homozygous-and-compound-heterozygous-mutation-in-3-turkish-family-with-jervell-and-lange-nielsen-syndrome-case-reports
#11
Fahrettin Uysal, Burcu Turkgenc, Guven Toksoy, Ozlem M Bostan, Elif Evke, Oya Uyguner, Cengiz Yakicier, Hulya Kayserili, Ergun Cil, Sehime G Temel
BACKGROUND: Jervell and Lange-Nielsen syndrome (JLNS) isa recessive model of long QT syndrome which might also be related to possible hearing loss. Although the syndrome has been demonstrated to be originated from homozygous or compound heterozygous mutations in either the KCNQ1 or KCNE1 genes, additional mutations in other genetic loci should be considered, particularly in malignant course patients. CASE PRESENTATIONS: Three patients were admitted into hospital due to recurrent seizures/syncope, intrauterine and postnatal bradycardia respectively; moreover all three patients had congenital sensorineural hearing-loss...
October 16, 2017: BMC Medical Genetics
https://www.readbyqxmd.com/read/29036559/the-brisk-standing-test-for-long-qt-syndrome-in-prepubertal-school-children-defining-normal
#12
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/29021306/characterization-of-a-human-induced-pluripotent-stem-cell-derived-cardiomyocyte-model-for-the-study-of-variant-pathogenicity-validation-of-a-kcnj2-mutation
#13
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/29020304/identification-of-a-targeted-and-testable-antiarrhythmic-therapy-for-long-qt-syndrome-type-2-using-a-patient-specific-cellular-model
#14
Ashish Mehta, Chrishan J A Ramachandra, Pritpal Singh, Anuja Chitre, Chong Hui Lua, Manuela Mura, Lia Crotti, Philip Wong, Peter J Schwartz, Massimiliano Gnecchi, Winston Shim
Aims: Loss-of-function mutations in the hERG gene causes long-QT syndrome type 2 (LQT2), a condition associated with reduced IKr current. Four different mutation classes define the molecular mechanisms impairing hERG. Among them, Class 2 mutations determine hERG trafficking defects. Lumacaftor (LUM) is a drug acting on channel trafficking already successfully tested for cystic fibrosis and its safety profile is well known. We hypothesize that LUM might rescue also hERG trafficking defects in LQT2 and exert anti-arrhythmic effects...
July 21, 2017: European Heart Journal
https://www.readbyqxmd.com/read/29016765/broad-antiarrhythmic-effect-of-mexiletine-in-different-arrhythmia-models
#15
Gerrit Frommeyer, Jonas Garthmann, Christian Ellermann, Dirk G Dechering, Simon Kochhäuser, Florian Reinke, Julia Köbe, Kristina Wasmer, Lars Eckardt
Aims: Experimental studies and clinical reports suggest antiarrhythmic properties of mexiletine in different arrhythmias. We aimed at investigating mexiletine in experimental models of atrial fibrillation (AF) as well as in long-QT- (LQTS) and short-QT-syndrome (SQTS). Methods and results: In 15 isolated rabbit hearts, erythromycin (300 µM) was infused for simulation of long-QT-2-syndrome. In further 13 hearts, veratridine was administered to simulate long-QT-3-syndrome...
August 1, 2017: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
https://www.readbyqxmd.com/read/28992755/drug-mediated-shortening-of-action-potentials-in-lqts2-human-induced-pluripotent-stem-cell-cardiomyocytes
#16
Gary Duncan, Karl Firth, Vinoj George, Minh Duc Hoang, Andrew Staniforth, Godfrey Smith, Chris Denning
Cardiomyocytes (CMs) derived from human induced pluripotent stem cells (hiPSCs) are now a well-established modality for modeling genetic disorders of the heart. This is especially so for long QT syndrome (LQTS), which is caused by perturbation of ion channel function, and can lead to fainting, malignant arrhythmias and sudden cardiac death. LQTS2 is caused by mutations in KCNH2, a gene whose protein product contributes to IKr (also known as HERG), which is the predominant repolarizing potassium current in CMs...
October 9, 2017: Stem Cells and Development
https://www.readbyqxmd.com/read/28944242/autosomal-recessive-long-qt-syndrome-type-1-in-eight-families-from-saudi-arabia
#17
Amnah Y Bdier, Saleh Al-Ghamdi, Prashant K Verma, Khalid Dagriri, Bandar Alshehri, Omamah A Jiman, Sherif E Ahmed, Arthur A M Wilde, Zahurul A Bhuiyan, Jumana Y Al-Aama
BACKGROUND: One of the most common primary cardiac arrhythmia syndromes is autosomal dominant long QT syndrome, type 1 (LQT1), chiefly caused by mono-allelic mutations in the KCNQ1 gene. Bi-allelic mutations in the KCNQ1 gene are causal to Jervell and Lange-Nielsen syndrome (JLNS), characterized by severe and early-onset arrhythmias with prolonged QTc interval on surface ECG and sensorineural deafness. Occasionally, bi-allelic mutations in KCNQ1 are also found in patients without any deafness, referred to as autosomal recessive long QT syndrome, type 1 (AR LQT1)...
September 2017: Molecular Genetics & Genomic Medicine
https://www.readbyqxmd.com/read/28944149/a-case-of-leprosy-erythema-nodosum-leprosum-and-hemophagocytic-syndrome-a-continuum-of-manifestations-of-same-agent-host-interactions
#18
Prasan Kumar Panda, Ramjas Prajapati, Arvind Kumar, Manisha Jana, Pradeep Immanuel, Pranay Tanwar, Naveet Wig
A young adult man with 4-years history of lepromatous leprosy (received irregularly multidrug therapy) presented with two and half years history of symptoms suggestive of chronic erythema nodosum leprosum (ENL), initially responded to steroids and thalidomide, but later on failed. During the last 2-months, he developed fever, vomiting, and subsequently altered sensorium. On evaluation, he had hepatosplenomegaly, hyponatremia, hyperferritinemia, hemophagocytosis in bone marrow aspiration, lobular panniculitis in skin biopsy, and multiple parenchymal nodules in chest imaging...
August 2017: Intractable & Rare Diseases Research
https://www.readbyqxmd.com/read/28935680/cardiac-events-during-competitive-recreational-and-daily-activities-in-children-and-adolescents-with-long-qt-syndrome
#19
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
#20
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
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