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J wave syndrome

Mouna Ben Kilani, Soufia Naccache, Rami Tlili, Dorra Mbarek, Salma Longo, Youssef Ben Ameur, Mohamed Rachid Boujnah
Class Ic antiarrythmic overdose is associated with a relatively high mortality. We presenta case report regarding a suicidal intoxication of an 18-year old female with a medical history of Wolff-Parkinson-White syndrome. The preliminary examination highlighted a profound cardiovascular collapse. The electrocardiogram showed a PR interval extended to 360 ms. The QRS complexes were enlarged to 360 ms with a right bundle brunch block appearance associated with left posterior hemibloc. There were repolarization abnormalities such as elevation of the J-point, convex ST segment and biphasic T wave in the right precordial leads ("Brugada-Like ECG pattern")...
June 2017: La Tunisie Médicale
Matthieu Raveau, Denis Polygalov, Roman Boehringer, Kenji Amano, Kazuhiro Yamakawa, Thomas J McHugh
Down syndrome, the leading genetic cause of intellectual disability, results from an extra-copy of chromosome 21. Mice engineered to model this aneuploidy exhibit Down syndrome-like memory deficits in spatial and contextual tasks. While abnormal neuronal function has been identified in these models, most studies have relied on in vitro measures. Here, using in vivo recording in the Dp(16)1Yey model, we find alterations in the organization of spiking of hippocampal CA1 pyramidal neurons, including deficits in the generation of complex spikes...
February 27, 2018: ELife
Andrés Ricardo Pérez-Riera, Raimundo Barbosa-Barros, Wallam Lima Aragão, Rodrigo Daminello-Raimundo, Luiz Carlos de Abreu, Joseane Elza Tonussi Mendes Rossette do Valle, Isabel Cristina Esposito Sorpreso, Kjell Nikus
We report a case of acute coronary syndrome with transient prominent anterior QRS forces (PAF) caused by proximal subocclusion of the left anterior descending (LAD) coronary artery before the first septal perforator branch. The ECG change indicates left septal fascicular block (LSFB) with associated slurring-type giant J-wave. Currently, this J-wave variant is considered as a lambda-like wave or QRS-ST-T "triangulation". Its presence is indicative of poor prognosis because of the risk for cardiac arrest as a consequence of ventricular tachycardia/ventricular fibrillation (VT/VF)...
February 24, 2018: Annals of Noninvasive Electrocardiology
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
BACKGROUND: Previously described patients with early repolarization syndrome (ERS) may have experienced silent coronary artery spasm (CAS) because the diagnosis of CAS was mainly based on symptoms or coronary angiography findings, without performing a spasm provocation test. This study investigated the significance of CAS diagnosis and evaluated the incidence of silent CAS in patients with possible ERS (ie, idiopathic ventricular fibrillation [VF] and inferolateral J wave). METHODS AND RESULTS: The study included 34 patients with idiopathic VF and inferolateral J wave...
February 7, 2018: Journal of the American Heart Association
Cindy Meun, Oscar H Franco, Klodian Dhana, Loes Jaspers, Taulant Muka, Yvonne Louwers, M Arfan Ikram, Bart C J M Fauser, Maryam Kavousi, Joop S E Laven
Context: Polycystic ovary syndrome (PCOS) is closely linked to hyperandrogenism. In PCOS, hyperandrogenism has been associated with metabolic disturbances which increase the risk for cardiovascular disease (CVD). Objective: To assess the association of high serum androgen levels, as a postmenopausal remnant of PCOS, with the prevalence of atherosclerosis and incidence of CVD in postmenopausal women. Design: The Rotterdam Study, a prospective population-based cohort study...
February 1, 2018: Journal of Clinical Endocrinology and Metabolism
James Winter, Michael Tipton, Michael J Shattock
This study tested the hypothesis that concomitant sympathetic and parasympathetic stimulation ("autonomic conflict") may act as a trigger for arrhythmia in long QT syndrome (LQTS). Studies were performed in isolated innervated rabbit hearts treated with clofilium (100 nmol/L); a potassium channel blocker. The influence of vagus nerve stimulation (VNS) on spontaneous ventricular arrhythmia was assessed in the absence/presence of sustained noradrenaline perfusion (100 nmol/L) and with sudden adrenergic stress (injections of noradrenaline into the perfusion line)...
February 2, 2018: Journal of Molecular and Cellular Cardiology
Karin W Littink, Patricia T Y Stappers, Frans C C Riemslag, Herman E Talsma, Maria M van Genderen, Frans P M Cremers, Rob W J Collin, L Ingeborgh van den Born
Enhanced S-cone syndrome (ESCS) is mainly associated with mutations in the NR2E3 gene. However, rare mutations in the NRL gene have been reported in patients with ESCS. We report on an ESCS phenotype in additional patients with autosomal recessive NRL (arNRL) mutations. Three Moroccan patients of two different families with arNRL mutations were enrolled in this study. The mutation in the DNA of one patient, from a consanguineous marriage, was detected by homozygosity mapping. The mutation in the DNA of two siblings from a second family was detected in a targeted next-generation sequencing project...
January 30, 2018: Genes
Shigehiro Karashima, Toyonobu Tsuda, Yusuke Wakabayashi, Mitsuhiro Kometani, Masashi Demura, Taro Ichise, Masa-Aki Kawashiri, Yoshiyu Takeda, Kenshi Hayashi, Takashi Yoneda
A J wave is a common electrocardiographic finding in the general population. Individuals with prominent J waves in multiple electrocardiogram (ECG) leads have a higher risk of lethal arrhythmias than those with low-amplitude J waves. There are few reports about the relationship between thyroid function and J-wave amplitude. We report the case of a 45-year-old man who had unexpected ventricular fibrillation (VF). He had dynamic J-point elevation in multiple ECG leads. Possible early repolarization syndrome was diagnosed...
February 1, 2018: Journal of the Endocrine Society
Naresh Reddivalla, Amie L Robinson, Kimberly J Reid, Mohamed A Radhi, Jignesh Dalal, Erin K Opfer, Sherwin S Chan
Sinusoidal obstruction syndrome (SOS) is a potentially fatal complication of hematopoietic stem cell transplantation (HSCT). Traditional ultrasound (US) has poor sensitivity and specificity. US shear wave elastography (SWE) is a newer technology that measures liver stiffness. This is a single-institution, prospective cohort study evaluating SWE in patients younger than 21 years who received HSCT from December 2015 through June 2017. SOS was defined using the modified Seattle criteria. Subjects had US with SWE at three scheduled time points...
January 15, 2018: Bone Marrow Transplantation
Jovil A Kannampuzha, Prasanna Sengodan, Sravani Avula, Bartholomew White, Stephen J Ganocy, Peter J Leo, Elizabeth S Kaufman
BACKGROUND: Patients with long QT syndrome (LQTS) are predisposed to polymorphic ventricular tachycardia (VT) during adrenergic stimulation. Microvolt T-wave alternans (MTWA) is linked to vulnerability to VT in structural heart disease. The prevalence of non-sustained MTWA (NS-MTWA) in LQTS is unknown. METHODS: 31 LQT1, 42 LQT2, and 80 controls underwent MTWA testing during exercise. MTWA tests were classified per standardized criteria, and re-analyzed according to the modified criteria to account for NS-MTWA...
November 21, 2017: Journal of Electrocardiology
Peter R Strege, Amelia Mazzone, Cheryl E Bernard, Leila Neshatian, Simon J Gibbons, Yuri A Saito, David J Tester, Melissa L Calvert, Emeran A Mayer, Lin Chang, Michael J Ackerman, Arthur Beyder, Gianrico Farrugia
The SCN5A-encoded voltage-gated mechanosensitive sodium (Na(+)) channel NaV1.5 is expressed in human GI smooth muscle cells and interstitial cells of Cajal. NaV1.5 contributes to smooth muscle electrical slow waves and mechanical sensitivity. In predominately Caucasian IBS patient cohorts, 2-3% have SCN5A missense mutations which alter NaV1.5 function and may contribute to IBS pathophysiology. In this study examined a racially and ethnically diverse cohort of IBS patients for SCN5A missense mutations, and compared them to IBS negative controls, and determined the resulting NaV1...
November 22, 2017: American Journal of Physiology. Gastrointestinal and Liver Physiology
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
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
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
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
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
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
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
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
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
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