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J wave syndrome

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https://www.readbyqxmd.com/read/29148067/should-theophylline-be-added-to-the-j-wave-syndrome-therapeutic-armamentarium
#1
EDITORIAL
Charles Antzelevitch, Sami Viskin
The substrate for both early repolarization syndrome (ERS) and Brugada syndrome (BrS), the so-called J wave syndromes, has been shown to develop as a consequence of genetic variants causing a loss of function of inward currents such sodium (INa ) and calcium (ICa ) channel currents or a gain of function in outward currents such as the transient outward current (Ito ). These changes in ion channel function result in an outward shift of the balance of currents active during the early phases of the epicardial action potential in the right ventricular outflow tract (RVOT) of the heart in the case BrS and in the inferior left ventricle in the case of ERS...
November 16, 2017: Pacing and Clinical Electrophysiology: PACE
https://www.readbyqxmd.com/read/29141844/architectural-t-wave-analysis-and-identification-of-on-therapy-breakthrough-arrhythmic-risk-in-type-1-and-type-2-long-qt-syndrome
#2
Alan Sugrue, Ram K Rohatgi, Peter A Noseworthy, Vaclav Kremen, J Martijn Bos, Bo Qiang, Yehu Sapir, Zachi I Attia, Christopher G Scott, Peter Brady, Samuel J Asirvatham, Paul A Friedman, Michael J Ackerman
BACKGROUND: Although the hallmark of long-QT syndrome (LQTS) is abnormal cardiac repolarization, there are varying degrees of phenotypic expression and arrhythmic risk. Our aim was to evaluate the performance of a morphological T-wave analysis program in defining breakthrough LQTS arrhythmic risk beyond the QTc value. METHODS AND RESULTS: We analyzed 407 genetically confirmed patients with LQT1 (n=246; 43% men) and LQT2 (n=161; 41% men) over the mean follow-up period of 6...
November 2017: Circulation. Arrhythmia and Electrophysiology
https://www.readbyqxmd.com/read/29130071/the-electrophysiological-substrate-of-early-repolarization-syndrome-noninvasive-mapping-in-patients
#3
Junjie Zhang, Mélèze Hocini, Maria Strom, Phillip S Cuculich, Daniel H Cooper, Frédéric Sacher, Michel Haïssaguerre, Yoram Rudy
Background: The early repolarization (ER) pattern is a common ECG finding. Recent studies established a definitive clinical association between ER and fatal ventricular arrhythmias. However, the arrhythmogenic substrate of ER in the intact human heart has not been characterized. Objectives: To map the epicardial electrophysiological (EP) substrate in ER syndrome patients using noninvasive Electrocardiographic Imaging (ECGI), and to characterize substrate properties that support arrhythmogenicity...
August 2017: JACC. Clinical Electrophysiology
https://www.readbyqxmd.com/read/29128194/treatment-of-electrical-status-epilepticus-in-sleep-clinical-and-eeg-characteristics-and-response-to-147-treatments-in-47-patients
#4
Bart van den Munckhof, Christian Alderweireld, Susanne Davelaar, Heleen C van Teeseling, Stavros Nikolakopoulos, Kees P J Braun, Floor E Jansen
OBJECTIVE: Electrical status epilepticus in sleep (ESES) syndrome is characterized by near-continuous sleep-induced epileptiform activity and acquired cognitive deficits. Treatment is assumed mandatory to improve cognitive outcome. We aimed to compare EEG characteristics, subjective evaluation and objective neuropsychological assessment as measures to evaluate treatment efficacy, and to analyze possible predictors. METHODS: We retrospectively included patients with ESES syndrome treated in our center...
October 26, 2017: European Journal of Paediatric Neurology: EJPN
https://www.readbyqxmd.com/read/29067908/evaluation-of-baseline-ecg-in-patients-undergoing-oral-flecainide-challenge-test-for-suspected-brugada-syndrome-an-analysis-of-lead-ii
#5
Maneesh K Rai, Mukund A Prabhu, Jayaprakash Shenthar, Natarajan Kumaraswamy U, Ritesh Vekariya, Padmanabh Kamath, Narasimha Pai, Ramanath L Kamath, Vivek Pillai
BACKGROUND AND OBJECTIVES: We analyzed Lead II in patients undergoing an Oral Flecainide Challenge test (FCT), to identify any pointers that could predict a positive FCT and thereby help in recognition of latent BS. METHODS: The following parameters in lead II were retrospectively analyzed from the pre-test ECG in 62 patients undergoing FCT for suspected BS: The presence or absence of S waves, S wave amplitude, duration and upslope duration; J point parameters- Early repolarization, QRS notch, and QRS Slur; ST segment parameters-lack of isoelectric ST segment, ST duration and QT interval...
July 2017: Indian Pacing and Electrophysiology Journal
https://www.readbyqxmd.com/read/29050732/-epidemiology-of-early-repolarization-pattern-in-maghreb
#6
W Amara, S A Bouallouche, A Rezoug, A El Hraiech, A Iusuf, N Hammoudi
An early repolarization variant (ERV) in inferolateral leads has recently been associated with vulnerability to ventricular fibrillation. These studies have been conducted in the occidental countries. The prevalence of ERV in the population of the Maghreb is unknown. The aim of this study was to evaluate the prevalence and risk factors of ERV in a young population from Algeria. METHODS: We assessed the prevalence of ERV within a population of 441 healthy subjects (mean age 25 years) using 12-lead electrocardiography...
November 2017: Annales de Cardiologie et D'angéiologie
https://www.readbyqxmd.com/read/29050557/agreement-of-the-resting-distal-to%C3%A2-aortic%C3%A2-coronary-pressure-with-the%C3%A2-instantaneous-wave-free-ratio
#7
Yuhei Kobayashi, Nils P Johnson, Frederik M Zimmermann, Nils Witt, Colin Berry, Allen Jeremias, Bon-Kwon Koo, Giovanni Esposito, Gilles Rioufol, Seung-Jung Park, Takeshi Nishi, Dong-Hyun Choi, Keith G Oldroyd, Emanuele Barbato, Nico H J Pijls, Bernard De Bruyne, William F Fearon
BACKGROUND: Recently, 2 randomized controlled trials showed that the instantaneous wave-free ratio (iFR), a resting coronary physiological index, is noninferior to fractional flow reserve for guiding revascularization. The resting distal to aortic coronary pressure (Pd/Pa) measured at rest is another adenosine-free index widely available in the cardiac catheterization laboratory; however, little is known about the agreement of Pd/Pa using iFR as a reference standard. OBJECTIVES: The goal of this study was to investigate the agreement of Pd/Pa with iFR...
October 24, 2017: Journal of the American College of Cardiology
https://www.readbyqxmd.com/read/29021844/intracoronary-acetylcholine-application-as-a-possible-probe-inducing-j-waves-in-patients-with-early-repolarization-syndrome
#8
REVIEW
Toru Maruyama, Kazumasa Fujita, Kei Irie, Shouhei Moriyama, Mitsuhiro Fukata
Acetylcholine is widely used for a diagnostic provocation test of coronary spasm in patients with vasospastic angina. Acetylcholine usually induces coronary vasodilatation mediated by muscarinic receptor activation, but sometimes it evokes vasoconstriction of coronary arteries where the endothelium is damaged. Early repolarization syndrome is characterized by a J wave observed at the end of the QRS complex in a surface electrocardiogram. The J wave is attributed to the transmural voltage gradient at the early repolarization phase across the ventricular wall, which stems mainly from prominent transient outward current in the epicardium, but not in the endocardium...
October 2017: Journal of Arrhythmia
https://www.readbyqxmd.com/read/28993753/electroencephalography-in-the-diagnosis-of-genetic-generalized-epilepsy-syndromes
#9
REVIEW
Udaya Seneviratne, Mark J Cook, Wendyl Jude D'Souza
Genetic generalized epilepsy (GGE) consists of several syndromes diagnosed and classified on the basis of clinical features and electroencephalographic (EEG) abnormalities. The main EEG feature of GGE is bilateral, synchronous, symmetric, and generalized spike-wave complex. Other classic EEG abnormalities are polyspikes, epileptiform K-complexes and sleep spindles, polyspike-wave discharges, occipital intermittent rhythmic delta activity, eye-closure sensitivity, fixation-off sensitivity, and photoparoxysmal response...
2017: Frontiers in Neurology
https://www.readbyqxmd.com/read/28974566/cardiovascular-structure-and-function-in-children-with-middle-aortic-syndrome-and-renal-artery-stenosis
#10
Rawan K Rumman, Cameron Slorach, Wei Hui, Mina Matsuda-Abedini, Valerie Langlois, Seetha Radhakrishnan, Armando J Lorenzo, Joao Amaral, Luc Mertens, Rulan S Parekh
Middle aortic syndrome (MAS) is a narrowing of the abdominal aorta, often in conjunction with renal artery stenosis (RAS). Structure and function of the cardiovascular system are not well understood. In a prospective cross-sectional study, 35 children with MAS or RAS or both (MAS/RAS) were compared with 140 age-, sex-, and body surface area-matched healthy children. Vascular assessment included carotid intima-media thickness and carotid distensibility using B-mode ultrasound and central and peripheral pulse wave velocities using applanation tonometry...
October 3, 2017: Hypertension
https://www.readbyqxmd.com/read/28965522/apical-hypertrophic-cardiomyopathy-treated-as-st-elevation-myocardial-infarction
#11
Christopher J Parr, Rajat Sharma, Philip J Garber
Electrocardiographic changes resulting from apical hypertrophic cardiomyopathy may mimic an acute coronary syndrome. A 67-year-old Sudanese male without cardiac risk factors presented to hospital with chest pain and electrocardiographic findings of septal ST-segment elevation, ST-segment depression in V4-V6, and diffuse T-wave inversion. He was treated as an acute ST-elevation myocardial infarction with thrombolytics. There was no cardiac biomarker rise and coronary angiography did not reveal evidence of significant coronary arterial disease...
October 2, 2017: CJEM
https://www.readbyqxmd.com/read/28954894/enhanced-interlaminar-excitation-or-reduced-superficial-layer-inhibition-in-neocortex-generates-different-spike-and-wave-like-electrographic-events-in-vitro
#12
Stephen P Hall, Roger D Traub, Natalie E Adams, Mark O Cunningham, Ian Schofield, Alistair J Jenkins, Miles A Whittington
Acute In vitro models have revealed a great deal of information about mechanisms underlying many types of epileptiform activity. However, few examples exist that shed light on spike and wave (SpW) patterns of pathological activity. SpW are seen in many epilepsy syndromes, both generalised and focal, and manifest across the entire age spectrum. They are heterogeneous in terms of their severity, symptom burden and apparent anatomical origin (thalamic, neocortical or both), but any relationship between this heterogeneity and underlying pathology remains elusive...
September 27, 2017: Journal of Neurophysiology
https://www.readbyqxmd.com/read/28942095/changes-in-truncal-obesity-and-fat-distribution-predict-arterial-health
#13
Frank E Corrigan, Heval Mohamed Kelli, Devinder S Dhindsa, Robert E Heinl, Ibhar Al Mheid, Muhammad Hammadah, Salim S Hayek, Salman Sher, Danny J Eapen, Greg S Martin, Arshed A Quyyumi
BACKGROUND: Truncal obesity is associated with metabolic syndrome and cardiovascular risk. Although vascular health is influenced by weight, it is not known whether changes in fat distribution modulate arterial function. OBJECTIVE: We assessed how changes in truncal (android) fat at 1 year affect arterial stiffness and endothelial function. METHODS: We recruited 711 healthy volunteers (235 males, age 48 ± 11 years) into the Emory Predictive Health Study; 498 returned at 1 year...
September 1, 2017: Journal of Clinical Lipidology
https://www.readbyqxmd.com/read/28930854/a-female-patient-with-hypokalaemia-induced-j-wave-syndrome-an-unusual-case-report
#14
Aqian Wang, Xiang Li, Muhammad Nabeel Dookhun, Tiancheng Zhang, Ping Xie, Yunshan Cao
RATIONALE: Prominent J waves can be seen in life-threatening cardiac arrhythmias such as Brugada syndrome, early repolarization syndrome, and ventricular fibrillation. We herein present an unusual case report of hypokalemia-induced J wave syndrome and ST (a part of ECG) segment elevation. PATIENTS CONCERNS: A 52-year-old woman with chief complaints of chest pain for 2 hours and diarrhea showed a marked hypokalemia (2.8 mmol/L) and slightly elevated creatine kinase-MB (CK-MB) (57...
September 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28928055/electrophysiological-analyses-of-transgenic-mice-overexpressing-kcnj8-with-s422l-mutation-in-cardiomyocytes
#15
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
https://www.readbyqxmd.com/read/28863167/the-development-and-validation-of-an-easy-to-use-automatic-qt-interval-algorithm
#16
Ben J M Hermans, Arja S Vink, Frank C Bennis, Luc H Filippini, Veronique M F Meijborg, Arthur A M Wilde, Laurent Pison, Pieter G Postema, Tammo Delhaas
BACKGROUND: To evaluate QT-interval dynamics in patients and in drug safety analysis, beat-to-beat QT-interval measurements are increasingly used. However, interobserver differences, aberrant T-wave morphologies and changes in heart axis might hamper accurate QT-interval measurements. OBJECTIVE: To develop and validate a QT-interval algorithm robust to heart axis orientation and T-wave morphology that can be applied on a beat-to-beat basis. METHODS: Additionally to standard ECG leads, the root mean square (ECGRMS), standard deviation and vectorcardiogram were used...
2017: PloS One
https://www.readbyqxmd.com/read/28851305/nephrolithiasis-and-osteomalacia-associated-with-adefovir-induced-fanconi-syndrome-in-a-patient-with-hepatitis-b
#17
Jueying Lin, Yufeng Zhuo, Dongdong Zhang
BACKGROUND: An increasing number of case reports suggest that acquired renal Fanconi syndrome may be associated with prolonged use of adefovir against hepatitis B virus. Renal Fanconi syndrome is an uncommon disease, and its complication with nephrolithiasis is quite rare. Herein, we report a rare coexistence of nephrolithiasis and acquired renal Fanconi syndrome in a chronic hepatitis B-positive patient with prolonged adefovir therapy. CASE PRESENTATION: The patient presented with osteomalacia and nephrolithiasis...
August 29, 2017: BMC Nephrology
https://www.readbyqxmd.com/read/28841780/evaluating-the-mutual-pathways-among-electronic-cigarette-use-conventional-smoking-and-nicotine-dependence
#18
Arielle S Selya, Jennifer S Rose, Lisa Dierker, Donald Hedeker, Robin J Mermelstein
BACKGROUND AND AIMS: The implications of the rapid rise in electronic cigarette (e-cigarette) use remain unknown. We examined mutual associations between e-cigarette use, conventional cigarette use, and nicotine dependence over time to 1) test the association between e-cigarette use and later conventional smoking (both direct and via nicotine dependence), 2) test the converse associations, and 3) determine the strongest pathways predicting each product's use. DESIGN: Data from 4 annual waves of a prospective cohort study were analyzed...
August 25, 2017: Addiction
https://www.readbyqxmd.com/read/28815597/cardiac-action-potential-repolarization-revisited-early-repolarization-shows-all-or-none-behaviour
#19
Beatriz Trenor, Karen Cardona, Javier Saiz, Denis Noble, Wayne Giles
In healthy mammalian hearts the action potential (AP) waveform initiates and modulates each contraction, or heartbeat. As a result, AP height and duration are key physiological variables. In addition, rate-dependent changes in ventricular AP duration (APD), and variations in APD at a fixed heart rate are both reliable biomarkers of electrophysiological stability. Present guidelines for the likelihood that candidate drugs will increase arrhythmias rely on small changes in APD and Q-T intervals as criteria for safety pharmacology decisions...
November 1, 2017: Journal of Physiology
https://www.readbyqxmd.com/read/28637782/the-brugada-syndrome-susceptibility-gene-hey2-modulates-cardiac-transmural-ion-channel-patterning-and-electrical-heterogeneity
#20
Christiaan C Veerman, Svitlana Podliesna, Rafik Tadros, Elisabeth M Lodder, Isabella Mengarelli, Berend de Jonge, Leander Beekman, Julien Barc, Ronald Wilders, Arthur A M Wilde, Bastiaan J Boukens, Ruben Coronel, Arie O Verkerk, Carol Ann Remme, Connie R Bezzina
RATIONALE: Genome-wide association studies previously identified an association of rs9388451 at chromosome 6q22.3 (near HEY2) with Brugada syndrome. The causal gene and underlying mechanism remain unresolved. OBJECTIVE: We used an integrative approach entailing transcriptomic studies in human hearts and electrophysiological studies in Hey2(+/-) (Hey2 heterozygous knockout) mice to dissect the underpinnings of the 6q22.31 association with Brugada syndrome. METHODS AND RESULTS: We queried expression quantitative trait locus data acquired in 190 human left ventricular samples from the genotype-tissue expression consortium for cis-expression quantitative trait locus effects of rs9388451, which revealed an association between Brugada syndrome risk allele dosage and HEY2 expression (β=+0...
August 18, 2017: Circulation Research
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