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early repolarization syndrome

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https://www.readbyqxmd.com/read/29205672/repetitive-incessant-electrical-storm-triggered-by-early-repolarization
#1
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/29183619/non-sustained-microvolt-level-t-wave-alternans-in-congenital-long-qt-syndrome-types-1-and-2
#2
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/29148067/should-theophylline-be-added-to-the-j-wave-syndrome-therapeutic-armamentarium
#3
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/29130071/the-electrophysiological-substrate-of-early-repolarization-syndrome-noninvasive-mapping-in-patients
#4
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/29127493/late-sodium-current-associated-cardiac-electrophysiological-and-mechanical-dysfunction
#5
REVIEW
Shandong Yu, Gang Li, Christopher L-H Huang, Ming Lei, Lin Wu
Late sodium current (INaL) is a small sustained inward current observed during the cardiac action potential plateau phase following decay of the early peak INa. The endogenous INaL is relatively small in normal hearts but exerts functionally significant effects on cardiomyocyte repolarization with potentially pro-arrhythmic effects in hearts with reduced repolarization reserve. Enhanced INa,L occurs in long QT syndrome 3 (LQTS 3) patients, and under a number of pathological and pharmacological cardiovascular conditions, including bradycardia, myocardial ischemia, reperfusion injury, and heart failure...
November 10, 2017: Pflügers Archiv: European Journal of Physiology
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
#6
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
#7
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/29033520/author-reply-to-letter-to-the-editor-silent-and-malignant-early-repolarization-syndrome-mimicking-hyper-acute-st-elevation-myocardial-infarction
#8
Weng-Chio Tam, Ming-Hsiung Hsieh, Yung-Kuo Lin, Jong-Shiuan Yeh
No abstract text is available yet for this article.
July 2017: Acta Cardiologica Sinica
https://www.readbyqxmd.com/read/29021844/intracoronary-acetylcholine-application-as-a-possible-probe-inducing-j-waves-in-patients-with-early-repolarization-syndrome
#9
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/28940628/identification%C3%A2-of-electrocardiographic-risk-markers-for-the-initial-and-recurrent-episodes-of-ventricular-fibrillation-in-patients-with-brugada-syndrome
#10
Hiroshi Morita, Atsuyuki Watanabe, Satoshi Kawada, Masakazu Miyamoto, Yoshimasa Morimoto, Koji Nakagawa, Nobuhiro Nishii, Kazufumi Nakamura, Hiroshi Ito
INTRODUCTION: New onset of ventricular fibrillation (VF) in asymptomatic patients with Brugada-type ECG is not frequent, but it cannot be negligible. Risk markers for predicting VF are usually based on results of analysis in symptomatic patients, and they have not been determined for asymptomatic patients. We analyzed ECG markers in patients with Brugada syndrome to differentiate the risk factors for VF in both symptomatic and asymptomatic patients. METHODS: The subjects were 471 patients with Brugada syndrome and we divided the subjects into two groups: Asymptomatic group (n = 326) and Symptomatic group (syncope: n = 122, VF: n = 23)...
September 22, 2017: Journal of Cardiovascular Electrophysiology
https://www.readbyqxmd.com/read/28931785/ventricular-fibrillation-due-to-early-repolarization-syndrome-in-the-wake-of-hypothermia-due-to-fulminant-acute-disseminated-encephalomyelitis
#11
Kyoichiro Yazaki, Yoichi Ajiro, Kunihiko Shimizu, Fumiaki Mori, Nobue Yagihara, Akinori Sato, Tatsuya Takahashi, Kazunori Iwade
No abstract text is available yet for this article.
September 20, 2017: Circulation Journal: Official Journal of the Japanese Circulation Society
https://www.readbyqxmd.com/read/28930854/a-female-patient-with-hypokalaemia-induced-j-wave-syndrome-an-unusual-case-report
#12
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/28842979/epicardial-ablation-utilizing-remote-magnetic-navigation-in-a-patient-with-brugada-syndrome-and-inferior-early-repolarization
#13
Paul Chun Yih Lim, Koonlawee Nademanee, Edward Chun Yi Lee, Wee Siong Teo
We report a case of epicardial ablation in a combined Brugada and inferior early repolarization syndrome patient with recurrent defibrillator therapy for spontaneous ventricular fibrillation. Electroanatomic mapping and ablation were achieved with remote magnetic navigation. Highly fractionated electrograms were seen epicardially in the anterior right ventricular outflow tract (RVOT) and at the anterior-inferior right ventricle. Ablation of the RVOT region resulted in resolution Brugada pattern electrocardiogram...
August 26, 2017: Pacing and Clinical Electrophysiology: PACE
https://www.readbyqxmd.com/read/28815597/cardiac-action-potential-repolarization-revisited-early-repolarization-shows-all-or-none-behaviour
#14
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/28797161/masked-inherited-primary-arrhythmia-syndromes-in-sudden-cardiac-death-patients-accompanied-by-coronary-vasospasm
#15
Ki Hong Lee, Hyung Wook Park, Jeong Nam Eun, Jeong Gwan Cho, Nam Sik Yoon, Mi Ran Kim, Yo Han Ku, Hyukjin Park, Seung Hun Lee, Jeong Han Kim, Min Chul Kim, Woo Jin Kim, Hyun Kuk Kim, Jae Yeong Cho, Keun-Ho Park, Doo Sun Sim, Hyun Ju Yoon, Kye Hun Kim, Young Joon Hong, Ju Han Kim, Youngkeun Ahn, Myung Ho Jeong, Jong Chun Park
BACKGROUND/AIMS: Coronary vasospasms are one of the important causes of sudden cardiac death (SCD). Provocation of coronary vasospasms can be useful, though some results may lead to false positives, with patients potentially experiencing recurrent SCD despite appropriate medical treatments. We hypothesized that it is not coronary vasospasms but inherited primary arrhythmia syndromes (IPAS) that underlie the development of SCD. METHODS: We analyzed 74 consecutive patients (3...
September 2017: Korean Journal of Internal Medicine
https://www.readbyqxmd.com/read/28685701/the-role-of-quinidine-in-the-pharmacological-therapy-of-ventricular-arrhythmias-quinidine
#16
Bojana Bozic, Teodora Vidonja Uzelac, Aleksandra Kezic, Milica Bajcetic
BACKGROUND: Historically, quinidine was the first medicine used in the therapy of heart arrhythmias. Studies in the early 20th century identified quinidine, a diastereomer of the antimalarial quinine, as the most potent of the antiarrhythmic substances extracted from the cinchona plant. Quinidine is used by the 1920s, as an antiarrhythmic agent to maintain sinus rhythm after conversion from atrial flutter or atrial fibrillation and to prevent recurrence of ventricular tachycardia or ventricular fibrillation...
July 7, 2017: Mini Reviews in Medicinal Chemistry
https://www.readbyqxmd.com/read/28685698/medical-therapy-for-long-qt-syndrome
#17
George Adamos, Nicoletta Iacovidou, Theodoros Xanthos
Long QT syndrome (LQTS) is an arrhythmogenic disorder characterized by repolarization abnormalities with a propensity to cause life threatening cardiac events. The first manifestation of the syndrome may be sudden death, therefore, early diagnosis and therapy is of great importance. LQTS can be both congenital and acquired. The latter is most commonly seen in hospitalized patients and such individuals have an easily recognizable and reversible precipitating factor (electrolyte disturbances, certain drugs etc...
July 7, 2017: Mini Reviews in Medicinal Chemistry
https://www.readbyqxmd.com/read/28619993/low-extracellular-potassium-prolongs-repolarization-and-evokes-early-afterdepolarization-in-human-induced-pluripotent-stem-cell-derived-cardiomyocytes
#18
Jukka Kuusela, Kim Larsson, Disheet Shah, Chandra Prajapati, Katriina Aalto-Setälä
Long QT syndrome (LQTS) is characterized by a prolonged QT-interval on electrocardiogram and by increased risk of sudden death. One of the most common and potentially life-threatening electrolyte disturbances is hypokalemia, characterized by low concentrations of K(+) Using a multielectrode array platform and current clamp technique, we investigated the effect of low extracellular K(+) concentration ([K(+)]Ex) on the electrophysiological properties of hiPSC-derived cardiomyocytes (CMs) generated from a healthy control subject (WT) and from two symptomatic patients with type 1 of LQTS carrying G589D (LQT1A) or IVS7-2A>G mutation (LQT1B) in KCNQ1 The baseline prolongations of field potential durations (FPDs) and action potential durations (APDs) were longer in LQT1-CMs than in WT-CMs...
June 15, 2017: Biology Open
https://www.readbyqxmd.com/read/28606656/divergent-antiarrhythmic-effects-of-resveratrol-and-piceatannol-in-a-whole-heart-model-of-long-qt-syndrome
#19
Christian Ellermann, Julian Wolfes, Simon Kochhäuser, Dirk G Dechering, Florian Reinke, Kristina Wasmer, Lars Eckardt, Gerrit Frommeyer
BACKGROUND: The polyphenol resveratrol and its metabolite piceatannol have beneficial health effects including antiarrhythmic properties in ischemia/reperfusion. The objective of this study was to determine potential antiarrhythmic effects in acquired long-QT-syndrome (LQTS). METHODS AND RESULTS: 26 rabbit hearts were isolated and Langendorff-perfused. The IKr-blocker sotalol (100μM) was infused to mimic LQTS-2. Hearts were assigned to two groups. Sotalol significantly prolonged action potential duration (APD90) and QT-interval in both groups (group 1:APD90: +18ms, p<0...
September 15, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/28569435/the-new-kids-on-the-block-of-arrhythmogenic-disorders-short-qt-syndrome-and-early-repolarization
#20
REVIEW
Andrea Mazzanti, Katherine Underwood, Dmitriy Nevelev, Shanna Kofman, Silvia G Priori
Short QT syndrome (SQTS) is one of the rarest inheritable cardiac channelopathies, characterized by an accelerated cardiac repolarization, which is also the substrate for the development of life-threatening ventricular arrhythmias. Up to this date, fewer than 200 SQTS cases have been reported in the literature worldwide. Patients with SQTS may experience a cardiac arrest as early as in the neonatal period or as late as 80 years old. The cumulative probability of experiencing a cardiac arrest by the fifth decade of life approaches 40%, highlighting the importance of early recognition and management...
October 2017: Journal of Cardiovascular Electrophysiology
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