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https://www.readbyqxmd.com/read/28213505/revealing-the-concealed-nature-of-long-qt-type-3-syndrome
#1
Amara Greer-Short, Sharon A George, Steven Poelzing, Seth H Weinberg
BACKGROUND: Gain-of-function mutations in the voltage-gated sodium channel (Nav1.5) are associated with the long-QT-3 (LQT3) syndrome. Nav1.5 is densely expressed at the intercalated disk, and narrow intercellular separation can modulate cell-to-cell coupling via extracellular electric fields and depletion of local sodium ion nanodomains. Models predict that significantly decreasing intercellular cleft widths slows conduction because of reduced sodium current driving force, termed "self-attenuation...
February 2017: Circulation. Arrhythmia and Electrophysiology
https://www.readbyqxmd.com/read/28165781/short-qt-syndrome-and-ventricular-tachycardia
#2
Oscar Mp Jolobe
No abstract text is available yet for this article.
February 2, 2017: British Journal of Hospital Medicine
https://www.readbyqxmd.com/read/28146053/cardiac-channelopathies-and-sudden-death-recent-clinical-and-genetic-advances
#3
REVIEW
Anna Fernández-Falgueras, Georgia Sarquella-Brugada, Josep Brugada, Ramon Brugada, Oscar Campuzano
Sudden cardiac death poses a unique challenge to clinicians because it may be the only symptom of an inherited heart condition. Indeed, inherited heart diseases can cause sudden cardiac death in older and younger individuals. Two groups of familial diseases are responsible for sudden cardiac death: cardiomyopathies (mainly hypertrophic cardiomyopathy, dilated cardiomyopathy, and arrhythmogenic cardiomyopathy) and channelopathies (mainly long QT syndrome, Brugada syndrome, short QT syndrome, and catecholaminergic polymorphic ventricular tachycardia)...
January 29, 2017: Biology
https://www.readbyqxmd.com/read/28110572/-cardiomyopathy-and-ion-channel-diseases-registry-the-szeged-cardiogen-registry
#4
Péter Blazsó, Kornél Kákonyi, Tamás Forster, Róbert Sepp
The Szeged cardiomyopathy and ion channel diseases registry aims to establish a representative disease-specific registry based on the recruitment of patients with different cardiomyopathies and ion channel diseases followed at the Cardiology Center, University of Szeged. The registry collects patient data on the main forms of primary cardiomyopathies (hypertrophic, dilated, restrictive, arrhythmogenic right ventricular, left ventricular non-compact, tako-tsubo cardiomyopathy) and ion channel diseases (long QT syndrome, short QT syndrome, Brugada syndrome, catecholaminergic polymorphic ventricular tachycardia)...
January 2017: Orvosi Hetilap
https://www.readbyqxmd.com/read/28012188/video-assisted-thoracoscopic-left-cardiac-sympathetic-denervation-in-patients-with-hereditary-ventricular-arrhythmias
#5
Se Yong Jang, Yongkeun Cho, Nam Kyun Kim, Chang-Yeon Kim, Jihyun Sohn, Jae-Hyung Roh, Myung Hwan Bae, Jang Hoon Lee, Dong Heon Yang, Hun Sik Park, Shung Chull Chae, Tak-Hyuk Oh, Gun Jik Kim
BACKGROUND: Left cardiac sympathetic denervation (LCSD) has been underutilized in patients with hereditary ventricular arrhythmia syndromes such as congenital long QT syndrome (LQTS) and catecholaminergic polymorphic ventricular tachycardia (CPVT). The purpose of this study was to investigate the safety and efficacy of video-assisted thoracoscopic (VATS) LCSD in such patients. METHODS: Fifteen patients (4 men, 24.6 ± 10.5 years old) who underwent VATS-LCSD between November 2010 and January 2015 for hereditary ventricular arrhythmia syndromes at Kyungpook National University Hospital were enrolled in this study...
December 24, 2016: Pacing and Clinical Electrophysiology: PACE
https://www.readbyqxmd.com/read/27805004/mir-19b-regulates-ventricular-action-potential-duration-in-zebrafish
#6
Alexander Benz, Mandy Kossack, Dominik Auth, Claudia Seyler, Edgar Zitron, Lonny Juergensen, Hugo A Katus, David Hassel
Sudden cardiac death due to ventricular arrhythmias often caused by action potential duration (APD) prolongation is a common mode of death in heart failure (HF). microRNAs, noncoding RNAs that fine tune gene expression, are frequently dysregulated during HF, suggesting a potential involvement in the electrical remodeling process accompanying HF progression. Here, we identified miR-19b as an important regulator of heart function. Zebrafish lacking miR-19b developed severe bradycardia and reduced cardiac contractility...
November 2, 2016: Scientific Reports
https://www.readbyqxmd.com/read/27771688/bystander-cardio-pulmonary-resuscitation-saves-life-in-a-patient-with-short-qt-syndrome
#7
Pradeep Kumar Sharma, Neeraj Awasthy
No abstract text is available yet for this article.
October 8, 2016: Indian Pediatrics
https://www.readbyqxmd.com/read/27761160/atrial-arrhythmias-in-inherited-arrhythmogenic-disorders
#8
Can Hasdemir
Atrial arrhythmias are being increasingly recognized in inherited arrhythmogenic disorders particularly in patients with Brugada syndrome and short QT syndrome. Atrial arrhythmias in inherited arrhythmogenic disorders have significant epidemiologic, clinical, and prognostic implications. There has been progress in the understanding of underlying genetic characteristics and the mechanistic link between atrial arrhythmias and inherited arrhythmogenic disorders. Appropriate management of these patients is of paramount importance...
October 2016: Journal of Arrhythmia
https://www.readbyqxmd.com/read/27761156/left-cardiac-sympathetic-denervation-an-important-treatment-option-for-patients-with-hereditary-ventricular-arrhythmias
#9
REVIEW
Yongkeun Cho
Medications such as ß-blockers are currently the primary treatment for patients with hereditary arrhythmia syndromes such as long QT syndrome (LQTS) and catecholaminergic polymorphic ventricular tachycardia (CPVT). However, these drugs are ineffective in some patients, and the other treatment option, that is implantable cardioverter defibrillator (ICD) implantation, is associated with significant complications in young and active patients. Left cardiac sympathetic denervation (LCSD) may reduce the wide gap between life-long ß-blocker medication and ICD implantation...
October 2016: Journal of Arrhythmia
https://www.readbyqxmd.com/read/27729057/takotsubo-like-syndrome-triggered-by-fludrocortisone-overdose-for-addison-s-disease-a-case-report
#10
Radu Campean, Matthias Hasun, Claudia Stöllberger, Johannes Bucher, Josef Finsterer, Christoph Schnack, Franz Weidinger
BACKGROUND: Reversible left ventricular dysfunction, also termed Takotsubo cardiomyopathy, is rarely reported in Addison's disease after initiation of hormone replacement therapy. The pathogenesis of this cardiomyopathy is unknown. CASE PRESENTATION: A 41-year-old white woman with a history of autoimmune Hashimoto thyroiditis diagnosed 3 years earlier and acute adrenal insufficiency diagnosed 3 weeks earlier presented with new onset of heart failure New York Heart Association class IV, which had started shortly after initiation of hormone replacement therapy with hydrocortisone 20 mg/day and fludrocortisone 0...
October 12, 2016: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/27646833/the-effects-of-pharmacological-compounds-on-beat-rate-variations-in-human-long-qt-syndrome-cardiomyocytes
#11
Jukka Kuusela, Jiyeong Kim, Esa Räsänen, Katriina Aalto-Setälä
Healthy human heart rate fluctuates overtime showing long-range fractal correlations. In contrast, various cardiac diseases and normal aging show the breakdown of fractal complexity. Recently, it was shown that human induced pluripotent stem cell-derived cardiomyocytes (hiPSC-CMs) intrinsically exhibit fractal behavior as in humans. Here, we investigated the fractal complexity of hiPSC-derived long QT-cardiomyocytes (LQT-CMs). We recorded extracellular field potentials from hiPSC-CMs at baseline and under the effect of various compounds including β-blocker bisoprolol, ML277, a specific and potent IKs current activator, as well as JNJ303, a specific IKs blocker...
December 2016: Stem Cell Reviews
https://www.readbyqxmd.com/read/27642736/-consciousness-disorders-from-cardiological-view
#12
Hans-Joachim Trappe
Consciousness disorders may have many causes, mainly cardiac arrhythmias. The incidence of bradyarrhythmias (BA) in patients with acute coronary syndrome (ACS) is 0.3-18 % and caused by sinus node dysfunction (SND), high degree atrioventricular (AV) block or bundle branch blocks. SND are sinus bradycardia or sinus arrest. 1st degree AV-block occurs in 4-13 % of patients with ACS caused by rhythm disturbances in atrium, AV node, bundle of His or the Tawara system. 1st or 2nd degree AV block is seen very frequently within 24 hours after beginning of ACS and these arrhythmias are frequently transient and no more present after 72 hours...
September 2016: Deutsche Medizinische Wochenschrift
https://www.readbyqxmd.com/read/27600096/nonalternans-repolarization-variability-and-arrhythmia-the-calcium-connection
#13
Jan Němec
Repolarization alternans precedes certain types of ventricular arrhythmias and its presence may be prognostically useful, but some ventricular arrhythmias are not preceded by repolarization alternans. Nonalternans changes of ventricular repolarization occur in patients with coronary artery disease and in subjects with congenital long QT syndrome. In animal experiments, nonalternans repolarization lability occurs in a canine model of chronic AV block with propensity to polymorphic ventricular tachycardia, in the perfused rabbit heart exposed to IKr block, and in a murine model of catecholaminergic polymorphic ventricular tachycardia...
November 2016: Journal of Electrocardiology
https://www.readbyqxmd.com/read/27540685/styrax-blocks-inward-and-outward-current-of-kir2-1-channel
#14
Shuxi Ren, Chunli Pang, Junwei Li, Yayue Huang, Suhua Zhang, Yong Zhan, Hailong An
Kir2.1 plays key roles in setting rest membrane potential and modulation of cell excitability. Mutations of Kir2.1, such as D172N or E299V, inducing gain-of-function, can cause type3 short QT syndrome (SQT3) due to the enlarged outward currents. So far, there is no clinical drug target to block the currents of Kir2.1. Here, we identified a novel blocker of Kir2.1, styrax, which is a kind of natural compound selected from traditional Chinese medicine. Our data show that styrax can abolish the inward and outward currents of Kir2...
January 2, 2017: Channels
https://www.readbyqxmd.com/read/27539165/the-natural-history-of-fetal-long-qt-syndrome
#15
Bettina F Cuneo, Janette F Strasburger, Ronald T Wakai
INTRODUCTION: Fetal magnetocardiography (fMCG), the magnetic analog of ECG, has provided invaluable insight into the mechanisms of fetal arrhythmias. In the past 15years, we have evaluated over 300 fetuses with arrhythmia by fMCG. We review the unique characteristics and natural history of the long QT syndrome (LQTS) rhythms. METHODS: We reviewed the fMCGs of subjects referred with suspected LQTS based on either a positive family history or echo diagnosis of the LQTS rhythms (sinus bradycardia, ventricular tachycardia, or 2:1 AV conduction) to the Biomagnetism laboratory in the Department of Medical Physics, UW-Madison...
November 2016: Journal of Electrocardiology
https://www.readbyqxmd.com/read/27521091/polymorphic-ventricular-tachycardia-ventricular-fibrillation-and-sudden-cardiac-death-in-the-normal-heart
#16
REVIEW
Ashok J Shah, Meleze Hocini, Arnaud Denis, Nicolas Derval, Frederic Sacher, Pierre Jais, Michel Haissaguerre
Primary electrical diseases manifest with polymorphic ventricular tachycardia (PMVT) and ventricular fibrillation (VF) and along with idiopathic VF contribute to about 10% of sudden cardiac deaths (SCDs) overall. These disorders include long QT syndrome, Brugada syndrome, catecholaminergic polymorphic ventricular tachycardia, short QT syndrome, and early repolarization syndrome. This article reviews the clinical electrophysiological management of PMVT/VF in a structurally normal heart affected with these disorders...
September 2016: Cardiac Electrophysiology Clinics
https://www.readbyqxmd.com/read/27470144/a-new-herg-allosteric-modulator-rescues-genetic-and-drug-induced-long-qt-syndrome-phenotypes-in-cardiomyocytes-from-isogenic-pairs-of-patient-induced-pluripotent-stem-cells
#17
Luca Sala, Zhiyi Yu, Dorien Ward-van Oostwaard, Jacobus Pd van Veldhoven, Alessandra Moretti, Karl-Ludwig Laugwitz, Christine L Mummery, Adriaan P IJzerman, Milena Bellin
Long-QT syndrome (LQTS) is an arrhythmogenic disorder characterised by prolongation of the QT interval in the electrocardiogram, which can lead to sudden cardiac death. Pharmacological treatments are far from optimal for congenital forms of LQTS, while the acquired form, often triggered by drugs that (sometimes inadvertently) target the cardiac hERG channel, is still a challenge in drug development because of cardiotoxicity. Current experimental models in vitro fall short in predicting proarrhythmic properties of new drugs in humans...
September 1, 2016: EMBO Molecular Medicine
https://www.readbyqxmd.com/read/27446933/clinical-genetic-testing-for-the-cardiomyopathies-and-arrhythmias-a-systematic-framework-for-establishing-clinical-validity-and-addressing-genotypic-and-phenotypic-heterogeneity
#18
John Garcia, Jackie Tahiliani, Nicole Marie Johnson, Sienna Aguilar, Daniel Beltran, Amy Daly, Emily Decker, Eden Haverfield, Blanca Herrera, Laura Murillo, Keith Nykamp, Scott Topper
Advances in DNA sequencing have made large, diagnostic gene panels affordable and efficient. Broad adoption of such panels has begun to deliver on the promises of personalized medicine, but has also brought new challenges such as the presence of unexpected results, or results of uncertain clinical significance. Genetic analysis of inherited cardiac conditions is particularly challenging due to the extensive genetic heterogeneity underlying cardiac phenotypes, and the overlapping, variable, and incompletely penetrant nature of their clinical presentations...
2016: Frontiers in Cardiovascular Medicine
https://www.readbyqxmd.com/read/27437140/monogenec-arrhythmic-syndromes-from-molecular-and-genetic-aspects-to-bedside
#19
Golukhova E Z, Gromova O I, Shomahov R A, Bulaeva N I, Bockeria L A
The abrupt cessation of effective cardiac function that is generally due to heart rhythm disorders can cause sudden and unexpected death at any age and is referred to as a syndrome called "sudden cardiac death" (SCD). Annually, about 400,000 cases of SCD occur in the United States alone. Less than 5% of the resuscitation techniques are effective. The prevalence of SCD in a population rises with age according to the prevalence of coronary artery disease, which is the most common cause of sudden cardiac arrest...
April 2016: Acta Naturae
https://www.readbyqxmd.com/read/27382819/a-case-of-granisetron-associated-intraoperative-cardiac-arrest
#20
Mohammed Al Harbi, Derar Al Rifai, Hassan Al Habeeb, Freddie Wambi, Georges Geldhof, Vassilios Dimitriou
We report a case of intraoperative severe bradycardia that resulted in asystole and cardiac arrest shortly after (<2 min) intravenous granisetron 1mg for postoperative nausea and vomiting prophylaxis, that occurred in a female patient who underwent an elective total thyroidectomy. After two cycles of cardiopulmonary resuscitation and defibrillation, spontaneous circulation and sinus rhythm returned successfully. Postoperatively, the patient was diagnosed with a drug-induced long QT syndrome. At the time of the event, granisetron was the only medication administered...
February 2016: Middle East Journal of Anesthesiology
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