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Early repolarization

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https://www.readbyqxmd.com/read/29331839/functional-characterization-of-a-novel-herg-variant-in-a-family-with-recurrent-sudden-infant-death-syndrome-retracting-a-genetic-diagnosis
#1
Valentine Sergeev, Frances Perry, Thomas M Roston, Shubhayan Sanatani, Glen F Tibbits, Thomas W Claydon
Long QT syndrome (LQTS) is the most common cardiac ion channelopathy and has been found to be responsible for approximately 10% of sudden infant death syndrome (SIDS) cases. Despite increasing use of broad panels and now whole exome sequencing (WES) in the investigation of SIDS, the probability of identifying a pathogenic mutation in a SIDS victim is low. We report a family-based study who are afflicted by recurrent SIDS in which several members harbor a variant, p.Pro963Thr, in the C-terminal region of the human-ether-a-go-go (hERG) gene, published to be responsible for cases of LQTS type 2...
December 20, 2017: Forensic Science International
https://www.readbyqxmd.com/read/29314443/ryanodine-receptor-inhibition-by-dantrolene-effectively-suppresses-ventricular-arrhythmias-in-an-ex-vivo-model-of-long-qt-syndrome
#2
Gerrit Frommeyer, Julius Krawczyk, Christian Ellermann, Nils Bögeholz, Simon Kochhäuser, Dirk G Dechering, Michael Fehr, Lars Eckardt
AIMS: A significant antiarrhythmic potential of ryanodine receptor inhibition was reported in experimental studies. The aim of the present study was to assess potential antiarrhythmic effects of dantrolene in an experimental whole-heart model of drug-induced long-QT syndrome (LQTS). METHODS: In 12 isolated rabbit hearts long-QT-2-syndrome was simulated by infusion of erythromycin (300μM). 12 rabbit hearts were treated with veratridine (0.5μM) to mimic long-QT-3-syndrome...
January 4, 2018: Journal of Cardiovascular Electrophysiology
https://www.readbyqxmd.com/read/29297274/development-of-models-for-predicting-torsade-de-pointes-cardiac-arrhythmias-using-perceptron-neural-networks
#3
Mohsen Sharifi, Dan Buzatu, Stephen Harris, Jon Wilkes
BACKGROUND: Blockage of some ion channels and in particular, the hERG (human Ether-a'-go-go-Related Gene) cardiac potassium channel delays cardiac repolarization and can induce arrhythmia. In some cases it leads to a potentially life-threatening arrhythmia known as Torsade de Pointes (TdP). Therefore recognizing drugs with TdP risk is essential. Candidate drugs that are determined not to cause cardiac ion channel blockage are more likely to pass successfully through clinical phases II and III trials (and preclinical work) and not be withdrawn even later from the marketplace due to cardiotoxic effects...
December 28, 2017: BMC Bioinformatics
https://www.readbyqxmd.com/read/29296579/early-repolarization-syndrome-caused-by-de-novo-duplication-of-kcnd3-detected-by-next-generation-sequencing
#4
Samuel Chauveau, Alexandre Janin, Marianne Till, Elodie Morel, Philippe Chevalier, Gilles Millat
No abstract text is available yet for this article.
December 2017: HeartRhythm Case Reports
https://www.readbyqxmd.com/read/29274604/interictal-cardiac-repolarization-abnormalities-in-people-with-epilepsy
#5
Nirmeen A Kishk, Yasser Sharaf, Asmaa M Ebraheim, Yasser Baghdady, Nelly Alieldin, Ahmed Afify, Ahmed Eldamaty
BACKGROUND AND OBJECTIVE: The occurrence of cardiac electrical abnormalities such as repolarization disorders in patients with epilepsy was previously documented and may, in part, clarify the mechanism of sudden unexpected death in those patients. The aim of this study was to investigate the frequency of cardiac repolarization disorders among patients with epilepsy and whether specific demographic- or disease-related features were associated with their occurrence. SUBJECTS AND METHODS: This cross-sectional study was carried out on 1000 subjects with epilepsy who were compared with age- and sex-matched 2500 subjects without epilepsy...
December 20, 2017: Epilepsy & Behavior: E&B
https://www.readbyqxmd.com/read/29273208/malignant-early-repolarization
#6
Elias B Hanna, Paul A LeLorier, David M Tadin, Ronnie O Ortiz, D Luke Glancy
A 27-year-old man presents with successfully resuscitated ventricular fibrillation. Structural and electrical causes of ventricular fibrillation in the young are presented along with a diagnostic strategy. Electrocardiographic features of malignant early repolarization are discussed.
November 24, 2017: American Journal of Cardiology
https://www.readbyqxmd.com/read/29233644/looking-at-late-repolarization-to-see-early-disease
#7
Pedro Silva Cunha
No abstract text is available yet for this article.
December 9, 2017: Portuguese Journal of Cardiology: An Official Journal of the Portuguese Society of Cardiology
https://www.readbyqxmd.com/read/29225104/evaluation-of-qt-dispersion-and-t-peak-to-t-end-interval-in-patients-with-early-stage-sarcoidosis
#8
Hacı Ahmet Kasapkara, Ayşegül Şentürk, Emine Bilen, Hüseyin Ayhan, Bilge Duran Karaduman, Zeynep Şeyma Turinay, Murat Can Güney, Tahir Durmaz, Telat Keleş, Engin Bozkurt
INTRODUCTION AND AIM: Sarcoidosis increases inhomogeneity in ventricular repolarization due to the presence of sarcoid granuloma, which significantly correlates with ventricular fibrillation. Various studies have suggested that the interval from the peak to the end of the electrocardiographic T wave (T-peak to T-end [Tpe] interval) may correspond to the transmural dispersion of repolarization and that increased Tpe interval and Tpe/QT ratio are associated with malignant ventricular arrhythmias...
December 7, 2017: Portuguese Journal of Cardiology: An Official Journal of the Portuguese Society of Cardiology
https://www.readbyqxmd.com/read/29216239/effects-of-early-afterdepolarizations-on-excitation-patterns-in-an-accurate-model-of-the-human-ventricles
#9
Enid Van Nieuwenhuyse, Gunnar Seemann, Alexander V Panfilov, Nele Vandersickel
Early Afterdepolarizations, EADs, are defined as the reversal of the action potential before completion of the repolarization phase, which can result in ectopic beats. However, the series of mechanisms of EADs leading to these ectopic beats and related cardiac arrhythmias are not well understood. Therefore, we aimed to investigate the influence of this single cell behavior on the whole heart level. For this study we used a modified version of the Ten Tusscher-Panfilov model of human ventricular cells (TP06) which we implemented in a 3D ventricle model including realistic fiber orientations...
2017: PloS One
https://www.readbyqxmd.com/read/29205672/repetitive-incessant-electrical-storm-triggered-by-early-repolarization
#10
Sofia Chatzidou, Evangelos Repasos, Sotiris Plastiras, Christos Kontogiannis, Marinos Kosmopoulos, Diamantis I Tsilimigras, Ioannis Paraskevaidis, Stelios Rokas
Early repolarization syndrome (ERS) was originally considered a normal variant with benign outcome. However, recent studies have demonstrated that it can be linked to a considerable risk of life-threatening arrhythmias and sudden cardiac death. We report a case with an extraordinary, extremely malignant clinical expression of ERS refractory to all antiarrhythmic drugs including quinidine. This case demonstrates real-time changes of dynamic electrocardiogram (ECG) preceding a polymorphic ventricular tachycardia (VT)-ventricular fibrillation (VF) and possible external factors triggering arrhythmia onset...
December 5, 2017: Annals of Noninvasive Electrocardiology
https://www.readbyqxmd.com/read/29203397/pro-arrhythmic-effects-of-low-plasma-k-in-human-ventricle-an-illustrated-review
#11
REVIEW
Beatriz Trenor, Karen Cardona, Lucia Romero, Juan F Gomez, Javier Saiz, Sridharan Rajamani, Luiz Belardinelli, Wayne Giles
Potassium levels in the plasma, [K+]o, are regulated precisely under physiological conditions. However, increases (from approx. 4.5 to 8.0mM) can occur as a consequence of, e.g., endurance exercise, ischemic insult or kidney failure. This hyperkalemic modulation of ventricular electrophysiology has been studied extensively. Hypokalemia is also common. It can occur in response to diuretic therapy, following renal dialysis, or during recovery from endurance exercise. In the human ventricle, clinical hypokalemia (e...
November 24, 2017: Trends in Cardiovascular Medicine
https://www.readbyqxmd.com/read/29191564/dynamic-st-segment-abnormality
#12
Elias B Hanna, Paul A LeLorier, D Luke Glancy
A 37-year-old man came to the emergency department because of several days of intermittent chest pain. An electrocardiogram (ECG) showed sinus rhythm, left atrial and left ventricular enlargement, and an early repolarization pattern. A second ECG recorded 10 minutes later was strikingly different, with ST-segment elevation and large upright T waves in the anterior precordial leads, interpreted as evidence of an ST-segment elevation myocardial infarction, and the cardiac catheterization team was activated. Closer inspection of the ECG, however, disclosed that the changes were because of intermittent ventricular pre-excitation of the Wolff-Parkinson-White type, and no electrocardiographic, echocardiographic, or serum markers of myocardial infarction were found...
February 1, 2018: American Journal of Cardiology
https://www.readbyqxmd.com/read/29186409/dynamic-j-point-elevation-associated-with-bigeminy-in-a-patient-with-early-repolarization-phenotype
#13
Thomas Andrew Nelson, Justin Lee
No abstract text is available yet for this article.
November 27, 2017: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
https://www.readbyqxmd.com/read/29183619/non-sustained-microvolt-level-t-wave-alternans-in-congenital-long-qt-syndrome-types-1-and-2
#14
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
https://www.readbyqxmd.com/read/29157021/gastroparesis-pharmacotherapy-and-cardiac-risk
#15
Per M Hellström, Ahmad Al-Saffar
BACKGROUND: Gastroparesis is characterized by abnormal gastric motility and delayed emptying with symptoms of early satiety, postprandial fullness, bloating, nausea, vomiting and abdominal pain. Pharmacological discovery has been lagging because potential drugs often are associated with abnormalities of electrical conduction of the myocardium due to interaction with cardiac ion channels leading to limited pharmaceutical options for development of new drugs. OBJECTIVE: Addresses the safety of drugs for gastroparesis in terms of cardiotoxicity related to the clinical use of prokinetics and antiemetics...
November 20, 2017: Scandinavian Journal of Gastroenterology
https://www.readbyqxmd.com/read/29148067/should-theophylline-be-added-to-the-j-wave-syndrome-therapeutic-armamentarium
#16
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/29148059/theophylline%C3%A2-as-an-adjunct-to-control-malignant-ventricular-arrhythmia-associated-with-early-repolarization
#17
Tilman Perrin, Regis Guieu, Linda Koutbi, Frédéric Franceschi, Jérôme Hourdain, Michele Brignole, Jean-Claude Deharo
Early repolarization (ER) has been associated with an increased risk of sudden cardiac arrest. Interestingly, ventricular arrhythmias seem to be triggered by parasympathetic stimulation. In the present case report, we describe complete control of highly frequent malignant ventricular arrhythmias after adding theophylline to ineffective oral hydroquinidine and high-rate pacing in a patient suffering from malignant ER. We hypothesize that the theophylline-mediated enhanced beta-adrenergic stimulation could reduce the transmural myocardial voltage discrepancy by increasing the inward ICa,L current...
November 16, 2017: Pacing and Clinical Electrophysiology: PACE
https://www.readbyqxmd.com/read/29148055/theophylline-the-forgotten-antiarrhythmic-drug%C3%A2-now-for-malignant-early-repolarization
#18
EDITORIAL
Sami Viskin, Ofer Havakuk, Charles Antzelevitch, Raphael Rosso
No abstract text is available yet for this article.
November 16, 2017: Pacing and Clinical Electrophysiology: PACE
https://www.readbyqxmd.com/read/29141592/case-report-electrical-storm-during-induced-hypothermia-in-a-patient-with-early-repolarization
#19
Patrick Badertscher, Michael Kuehne, Beat Schaer, Christian Sticherling, Stefan Osswald, Tobias Reichlin
BACKGROUND: Population based studies showed an association of early repolarization in the electrocardiogram (ECG) and a higher rate of sudden cardiac death presumably due to ventricular fibrillation. The triggers for ventricular fibrillation in patients with early repolarization are not fully understood. CASE PRESENTATION: We describe the case of a young patient with a survived ventricular fibrillation arrest while asleep followed by multiple episodes of recurrent ventricular fibrillation...
November 15, 2017: BMC Cardiovascular Disorders
https://www.readbyqxmd.com/read/29130071/the-electrophysiological-substrate-of-early-repolarization-syndrome-noninvasive-mapping-in-patients
#20
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
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