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early repolarization syndrome

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https://www.readbyqxmd.com/read/29436392/significance-of-coronary-artery-spasm-diagnosis-in-patients-with-early-repolarization-syndrome
#1
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
https://www.readbyqxmd.com/read/29407007/new-insights-into-the-use-of-the-12-lead-electrocardiogram-for-diagnosing-acute-myocardial-infarction-in-the-emergency-department
#2
REVIEW
David F Miranda, Angie S Lobo, Brooks Walsh, Yader Sandoval, Stephen W Smith
The 12-lead electrocardiogram (ECG) remains the most immediately accessible and widely used initial diagnostic tool for guiding management in patients with suspected myocardial infarction (MI). Although the development of high-sensitivity cardiac troponin assays has improved the rule-in and rule-out and risk stratification of acute MI without ST elevation, the immediate management of the subset of acute MI with acute coronary occlusion depends on integrating clinical presentation and ECG findings. Careful interpretation of the ECG might yield subtle features suggestive of ischemia that might facilitate more rapid triage of patients with subtle acute coronary occlusion or, conversely, in identification of ST-elevation MI mimics (pseudo ST-elevation MI patterns)...
February 2018: Canadian Journal of Cardiology
https://www.readbyqxmd.com/read/29395134/mutation-in-kcne1-associated-to-early-repolarization-syndrome-by-modulation-of-slowly-activating-delayed-rectifier-k-current
#3
Hao Yao, Cheng-Cheng Ji, Yun-Jiu Cheng, Xu-Miao Chen, Li-Juan Liu, Jun Fan, Su-Hua Wu
BACKGROUND: Recent studies have revealed that mutation in KCNE1, β-subunits of cardiac potassium channel, involved in ventricular fibrillation. Whereas its role in early repolarization syndrome (ERS) is less well understood. OBJECTIVE: To study whether mutant in KCNE1 is associated with ERS and explore the possible underlying molecular mechanisms. METHODS: Whole genome from four unrelated families with ERS was amplified and sequenced. Wild-type (WT) KCNE1 and/or KCNE1-S38G (S38G) were expressed in HEK293 cells with KCNQ1...
January 29, 2018: Experimental Cell Research
https://www.readbyqxmd.com/read/29383332/ventricular-fibrillation-associated-with-dynamic-changes-in-j-point-elevation-in-a-patient-with-silent-thyroiditis
#4
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
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
#5
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
#6
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/29296579/early-repolarization-syndrome-caused-by-de-novo-duplication-of-kcnd3-detected-by-next-generation-sequencing
#7
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/29205672/repetitive-incessant-electrical-storm-triggered-by-early-repolarization
#8
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
#9
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
#10
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
#11
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
#12
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
#13
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
#14
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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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