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short qt syndrome

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
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
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
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...
September 19, 2016: Stem Cell Reviews
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
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...
August 17, 2016: Journal of Electrocardiology
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...
August 12, 2016: Channels
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...
July 28, 2016: Journal of Electrocardiology
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
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...
2016: EMBO Molecular Medicine
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
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
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
Nianhang Chen, Simon Zhou, Maria Palmisano
Lenalidomide is a lead therapeutic in multiple myeloma and deletion 5q myelodysplastic syndromes and shows promising activities in other hematologic malignancies. This article presents a comprehensive review of the clinical pharmacokinetics and pharmacodynamics of lenalidomide. Oral lenalidomide is rapidly and highly absorbed (>90 % of dose) under fasting conditions. Food affects oral absorption, reducing area under the concentration-time curve (AUC) by 20 % and maximum concentration (C max) by 50 %. The increase in AUC and C max is dose proportional, and interindividual variability in plasma exposure is low to moderate...
June 28, 2016: Clinical Pharmacokinetics
Gerrit Frommeyer, Christian Ellermann, Dirk G Dechering, Simon Kochhäuser, Nils Bögeholz, Fatih Güner, Patrick Leitz, Christian Pott, Lars Eckardt
BACKGROUND: Ranolazine has been reported to have an antiarrhythmic potential. The aim of this study was to assess the electrophysiologic effects of ranolazine and to compare its effects to vernakalant in an experimental whole-heart model of short-QT syndrome. METHODS: Rabbit hearts were isolated and Langendorff-perfused. After obtaining baseline data, pinacidil, an IKATP channel opener, was administered (1 μM). RESULTS: Endo- and epicardial monophasic action potentials and a 12-lead ECG showed a significant abbreviation of QT interval (- 34 milliseconds, P < 0...
June 10, 2016: Journal of Cardiovascular Electrophysiology
Ljubica Georgijević, Lana Andrić
Electrocardiography (ECG) is especially significant in pre-participation screening due to its ability to discover or to rise a suspicion for certain cardiovascular diseases and conditions that represent a serious health risk in athletes. Common, conditionally benign and training related ECG changes are sinus bradycardia and sinus arrhythmia, first degree atrioventricular block, incomplete right bundle branch block, benign early repolarization, and isolated QRS voltage criteria for left ventricular enlargement...
January 2016: Srpski Arhiv za Celokupno Lekarstvo
Arnon Adler, Sami Viskin
Genetic cardiac diseases related to potassium channelopathies are a group of relatively rare syndromes that includes long QT syndrome, short QT syndrome, Brugada syndrome, and early repolarization syndrome. Patients with these syndromes share a propensity for the development of life-threatening ventricular arrhythmias in the absence of significant cardiac structural abnormalities. Familial atrial fibrillation has also been associated with potassium channel dysfunction but differs from the other syndromes by being a rare cause of a common condition...
June 2016: Cardiac Electrophysiology Clinics
Lei Chen, Kevin J Sampson, Robert S Kass
Cardiac delayed rectifier potassium channels conduct outward potassium currents during the plateau phase of action potentials and play pivotal roles in cardiac repolarization. These include IKs, IKr and the atrial specific IKur channels. In this article, we will review their molecular identities and biophysical properties. Mutations in the genes encoding delayed rectifiers lead to loss- or gain-of-function phenotypes, disrupt normal cardiac repolarization and result in various cardiac rhythm disorders, including congenital Long QT Syndrome, Short QT Syndrome and familial atrial fibrillation...
June 2016: Cardiac Electrophysiology Clinics
Esther S Kim, Emma D Deeks
Gabapentin enacarbil is an extended-release prodrug of gabapentin that is approved in the USA (Horizant(®)) and Japan (Regnite(®)) for the treatment of moderate to severe primary restless legs syndrome (RLS) in adults [featured indication]. This article summarizes pharmacological, efficacy and tolerability data relevant to the use of oral gabapentin enacarbil in this indication. In double-blind, multicentre trials, treatment with gabapentin enacarbil 600 mg/day for 12 weeks significantly improved the symptoms of moderate to severe primary RLS in adults...
May 2016: Drugs
Federico Migliore, Maria Silvano, Alessandro Zorzi, Emanuele Bertaglia, Mariachiara Siciliano, Loira Leoni, Pietro De Franceschi, Sabino Iliceto, Domenico Corrado
AIMS: This study was designed to prospectively evaluate the risk-benefit ratio of implantable cardioverter defibrillator (ICD) therapy in young patients with cardiomyopathies and channelopathies. METHODS AND RESULTS: The study population included 96 consecutive patients [68 men, median age 27 (22-32) years] with cardiomyopathies, such as arrhythmogenic right ventricular cardiomyopathy (n = 35), dilated cardiomyopathy (n = 17), hypertrophic cardiomyopathy (n = 15), Brugada syndrome (n = 14), idiopathic ventricular fibrillation (n = 5), left ventricular noncompaction (n = 4), long-QT syndrome (n = 4) and short-QT syndrome (n = 2), who were 18-35 years old at the time of ICD implantation...
July 2016: Journal of Cardiovascular Medicine
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