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Long qt syndrome

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https://www.readbyqxmd.com/read/28346324/t-wave-alternans-and-long-qt-syndrome
#1
Rebecca S Isserman, Allan F Simpao, Alan Jay Schwartz, Matthew F Pearsall
No abstract text is available yet for this article.
March 27, 2017: Anesthesiology
https://www.readbyqxmd.com/read/28343764/channelopathies-genetic-testing-and-risk-stratification
#2
Arthur A M Wilde, Ahmad Amin
The cardiac channelopathies are a group of diseases with (disease-) specific electrocardiographic (ECG) characteristics and a disease-specific risk of sudden cardiac death (SCD). This group includes the Long QT Syndromes (LQTS), Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT), Brugada Syndrome (BrS), Short QT Syndromes (SQTS), and Early Repolarization Syndrome (ERS). In the past 2 decades the genetic basis for these disease entities has largely been unraveled and that, together with the identification of the genetic basis of the cardiomyopathies, has paved the way for the complete new field of Cardiogenetics...
March 18, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/28341588/targeted-next-generation-sequencing-of-51-genes-involved-in-primary-electrical-disease
#3
Dorien Proost, Johan Saenen, Geert Vandeweyer, Annelies Rotthier, Maaike Alaerts, Emeline M Van Craenenbroeck, Joachim Van Crombruggen, Geert Mortier, Wim Wuyts, Christiaan Vrints, Jurgen Del Favero, Bart Loeys, Lut Van Laer
Primary electrical disease (PED) is characterized by cardiac arrhythmias, which can lead to sudden cardiac death in the absence of detectable structural heart disease. PED encompasses a diversity of inherited syndromes, predominantly Brugada syndrome, early repolarization syndrome, long QT syndrome, short QT syndrome, arrhythmogenic right ventricular cardiomyopathy, and catecholaminergic polymorphic ventricular tachycardia. To overcome the diagnostic challenges imposed by the clinical and genetic heterogeneity of PED, we developed a targeted gene panel for next-generation sequencing of 51 PED genes...
March 21, 2017: Journal of Molecular Diagnostics: JMD
https://www.readbyqxmd.com/read/28340164/denervation-of-the-extrinsic-cardiac-sympathetic-nervous-system-as-a-treatment-modality-for-arrhythmia
#4
Chance M Witt, Luciana Bolona, Michelle O Kinney, Christopher Moir, Michael J Ackerman, Suraj Kapa, Samuel J Asirvatham, Christopher J McLeod
Denervation of the extrinsic cardiac sympathetic nervous system is a method of altering the autonomic tone experienced by the heart and vasculature. It has been studied and employed as a therapy for cardiac disease for decades. Currently, there is a high level of interest in using cardiac denervation for treatment of arrhythmias. This review describes the anatomy and physiology of the cardiac autonomic nervous system followed by a discussion of the mechanistic studies which provide a basis for the therapeutic use of sympathetic denervation...
March 10, 2017: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
https://www.readbyqxmd.com/read/28340026/high-rate-of-subcutaneous-implantable-cardioverter-defibrillator-sensing-screening-failure-in-patients-with-brugada-syndrome-a-comparison-with-other-inherited-primary-arrhythmia-syndromes
#5
Giulio Conte, Mihoko Kawabata, Carlo de Asmundis, Erika Taravelli, Francesco Petracca, Diego Ruggiero, Maria Luce Caputo, François Regoli, Gian-Battista Chierchia, Alessandra Chiodini, Alessandro Del Bufalo, Tiziano Moccetti, Masahiko Goya, Kenzo Hirao, Alessandro Vicentini, Gaetano M De Ferrari, Pedro Brugada, Angelo Auricchio
Aims: Subcutaneous implantable cardioverter-defibrillator (S-ICD) can avoid important complications associated with transvenous leads in patients with inherited primary arrhythmia syndromes, who do not need pacing therapy. Few data are available on the percentage of patients with inherited arrhythmia syndromes eligible for S-ICD implantation. Aim of this study was to analyse the eligibility for S-ICD in a series of patients with Brugada syndrome (BrS), and to compare it with patients with other channelopathies...
March 7, 2017: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
https://www.readbyqxmd.com/read/28339995/long-term-flecainide-therapy-in-type-3-long-qt-syndrome
#6
Ehud Chorin, Rivki Taub, Aron Medina, Nir Flint, Sami Viskin, Jesaia Benhorin
Aims: Type 3 long QT syndrome (LQT3) is caused by gain-of-function mutations in the cardiac sodium channel gene (SCN5A). Previous reports on the long-term use of sodium channel blockers in LQT3 are sparse. The objective of the current study was to evaluate the long-term safety and efficacy of flecainide therapy in patients with LQT3 who carry the D1790G SCN5A mutation. Methods and results: The study population comprised 30 D1790G carriers who were treated with flecainide and followed for 1-215 months (mean 145 ± 54 months, median 140 months)...
February 28, 2017: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
https://www.readbyqxmd.com/read/28336914/inhibition-of-serum-and-glucocorticoid-regulated-kinase-1-as-novel-therapy-for-cardiac-arrhythmia-disorders
#7
Vassilios J Bezzerides, Aifeng Zhang, Ling Xiao, Bridget Simonson, Santosh A Khedkar, Shiro Baba, Filomena Ottaviano, Stacey Lynch, Katherine Hessler, Alan C Rigby, David Milan, Saumya Das, Anthony Rosenzweig
Alterations in sodium flux (INa) play an important role in the pathogenesis of cardiac arrhythmias and may also contribute to the development of cardiomyopathies. We have recently demonstrated a critical role for the regulation of the voltage-gated sodium channel NaV1.5 in the heart by the serum and glucocorticoid regulated kinase-1 (SGK1). Activation of SGK1 in the heart causes a marked increase in both the peak and late sodium currents leading to prolongation of the action potential duration and an increased propensity to arrhythmia...
March 23, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28335032/allele-specific-ablation-rescues-electrophysiological-abnormalities-in-a-human-ips-cell-model-of-long-qt-syndrome-with-a-calm2-mutation
#8
Yuta Yamamoto, Takeru Makiyama, Takeshi Harita, Kenichi Sasaki, Yimin Wuriyanghai, Mamoru Hayano, Suguru Nishiuchi, Hirohiko Kohjitani, Sayako Hirose, Jiarong Chen, Fumika Yokoi, Taisuke Ishikawa, Seiko Ohno, Kazuhisa Chonabayashi, Hideki Motomura, Yoshinori Yoshida, Minoru Horie, Naomasa Makita, Takeshi Kimura
Background: Calmodulin is a ubiquitous Ca 2+ sensor molecule encoded by three distinct calmodulin genes, CALM1-3 . Recently, mutations in CALM1-3 have been reported to be associated with severe early-onset long-QT syndrome (LQTS). However, the underlying mechanism through which heterozygous calmodulin mutations lead to severe LQTS remains unknown, particularly in human cardiomyocytes. Objectives: We aimed to establish an LQTS disease model associated with a CALM2 mutation (LQT15) using human induced pluripotent stem cells (hiPSCs) and to assess mutant allele-specific ablation by genome editing for the treatment of LQT15...
March 1, 2017: Human Molecular Genetics
https://www.readbyqxmd.com/read/28323172/can-long-qt-syndrome-be-diagnosed-by-vectorcardiography-when-the-qtc-on-the-resting-12-lead-ecg-is-of-normal-duration
#9
EDITORIAL
Arthur J Moss
No abstract text is available yet for this article.
March 16, 2017: Heart Rhythm: the Official Journal of the Heart Rhythm Society
https://www.readbyqxmd.com/read/28323171/acquired-long-qt-syndrome-in-hospitalized-patients
#10
Haixu Yu, Li Zhang, Jinqiu Liu, Ying Liu, Peter R Kowey, Yanli Zhang, Yue Chen, Yushan Wei, Lianjun Gao, Huihua Li, Jie Du, Yunlong Xia
BACKGROUND: Acquired long QT syndrome (ALQTS) has long been overlooked in clinical practice. Recent studies reported that severe ALQTS (QTc ≥500 ms) in hospitalized patients is associated with increased all-cause mortality. OBJECTIVE: We aimed to determine the role of ALQTS in the clinical outcomes of hospitalized patients. METHODS: Electronic medical records were reviewed to identify severe ALQTS in hospitalized patients in a single study center from 9-1-2013 to 2-28-2014...
March 16, 2017: Heart Rhythm: the Official Journal of the Heart Rhythm Society
https://www.readbyqxmd.com/read/28320606/autoantibodies-with-beta-adrenergic-activity-from-chronic-chagasic-patients-induce-cardiac-arrhythmias-and-early-afterdepolarization-in-a-drug-induced-lqt2-rabbit-hearts
#11
Marco Antonio Vidal Jiménez, José H M Nascimento, Gustavo Monnerat, Leonardo Maciel, Claudia N Paiva, Roberto Coury Pedrosa, Antonio C Campos de Carvalho, Emiliano Medei
BACKGROUND: Cardiac arrhythmias are one of the main causes of death in ChCP and other dilated cardiomyopathies. Previous studies demonstrated that ventricular arrhythmias are associated with the presence of autoantibodies with beta-adrenergic activity, Ab-β. OBJECTIVES: The aim of this study was to investigate whether Ab-β, present in chronic chagasic patients (ChCP), induce cardiac arrhythmias in the pharmacological type-2 long QT syndrome model (LQTS-2). METHODS/RESULTS: The LQTS2 was established by perfusion of Tyrode saline solution with a potassium channel blocker E-4031 (5μM) in isolated rabbit hearts or in rabbit cardiac strips, in order to record ECG or action potential, respectively...
March 1, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/28318666/cardiac-sympathetic-denervation-100years-later-jonnesco-would-have-never-believed-it
#12
Peter J Schwartz, Gaetano M De Ferrari, Luigi Pugliese
One hundred years have elapsed since Thomas Jonnesco performed the first left cardiac sympathetic denervation (LCSD) in a patient with unmanageable angina pectoris and ventricular tachyarrhythmias, and the progress in the field has surpassed imagination. Here we will review the historic basis of cardiac sympathectomy for the management of life-threatening arrhythmias and will then discuss the often forgotten critical experimental studies that provided the rationale for the amazing growth of its role in clinical management...
March 8, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/28316956/sudden-infant-death-syndrome-due-to-long-qt-syndrome-a-brief-review-of-the-genetic-substrate-and-prevalence
#13
REVIEW
Nikolaos S Ioakeimidis, Theodora Papamitsou, Soultana Meditskou, Zafiroula Iakovidou-Kritsi
The pathophysiological mechanisms which lead to sudden infant death syndrome (SIDS) are not completely understood. Cardiac channelopathies are a well-established causative factor with long QT syndrome (LQTS) being the most frequent one, accounting for approximately 12% of SIDS cases. The genetic substrate of the above arrhythmogenic syndrome has been thoroughly described but only specific gene mutations or polymorphisms have been identified as SIDS causative. The review will focus on the prevalence of LQTS-induced SIDS or near-SIDS cases and the mutations held responsible...
December 2017: Journal of Biological Research
https://www.readbyqxmd.com/read/28315637/trpm4-non-selective-cation-channel-variants-in-long-qt-syndrome
#14
Thomas Hof, Hui Liu, Laurent Sallé, Jean-Jacques Schott, Corinne Ducreux, Gilles Millat, Philippe Chevalier, Vincent Probst, Romain Guinamard, Patrice Bouvagnet
BACKGROUND: Long QT syndrome (LQTS) is an inherited arrhythmic disorder characterized by prolongation of the QT interval, a risk of syncope, and sudden death. There are already a number of causal genes in LQTS, but not all LQTS patients have an identified mutation, which suggests LQTS unknown genes. METHODS: A cohort of 178 LQTS patients, with no mutations in the 3 major LQTS genes (KCNQ1, KCNH2, and SCN5A), was screened for mutations in the transient potential melastatin 4 gene (TRPM4)...
March 18, 2017: BMC Medical Genetics
https://www.readbyqxmd.com/read/28292826/arrhythmia-risk-and-%C3%AE-blocker-therapy-in-pregnant-women-with-long-qt-syndrome
#15
Kohei Ishibashi, Takeshi Aiba, Chizuko Kamiya, Aya Miyazaki, Heima Sakaguchi, Mitsuru Wada, Ikutaro Nakajima, Koji Miyamoto, Hideo Okamura, Takashi Noda, Toshifumi Yamauchi, Hideki Itoh, Seiko Ohno, Hideki Motomura, Yoshiharu Ogawa, Hiroko Goto, Takaomi Minami, Nobue Yagihara, Hiroshi Watanabe, Kanae Hasegawa, Akihiro Terasawa, Hitoshi Mikami, Kayo Ogino, Yukiko Nakano, Sato Imashiro, Yosuke Fukushima, Yoshimitsu Tsuzuki, Koko Asakura, Jun Yoshimatsu, Isao Shiraishi, Shiro Kamakura, Yoshihiro Miyamoto, Satoshi Yasuda, Takashi Akasaka, Minoru Horie, Wataru Shimizu, Kengo Kusano
BACKGROUND: Pregnancy is one of the biggest concerns for women with long QT syndrome (LQTS). OBJECTIVES: This study investigated pregnancy-related arrhythmic risk and the efficacy and safety of β-blocker therapy for lethal ventricular arrhythmias in pregnant women with LQTS (LQT-P) and their babies. METHODS: 136 pregnancies in 76 LQT-P (29±5 years old; 22 LQT1, 36 LQT2, one LQT3, and 17 genotype-unknown) were enrolled. We retrospectively analysed their clinical and electrophysiological characteristics and pregnancy outcomes in the presence (BB group: n=42) or absence of β-blocker therapy (non-BB group: n=94)...
March 14, 2017: Heart: Official Journal of the British Cardiac Society
https://www.readbyqxmd.com/read/28291620/pacing-in-congenital-heart-disease-a-four-decade-experience-in-a-single-tertiary-centre
#16
Disha Midha, Zhong Chen, David G Jones, Howell J Williams, Karen Lascelles, Julian Jarman, Jonathan Clague, Janice Till, Konstatinos Dimopoulos, Sonya V Babu-Narayan, Vias Markides, Michael A Gatzoulis, Tom Wong
BACKGROUND: The increased risk of brady- and tachy-arrhythmias in the congenital heart disease (CHD) population means that cardiac rhythm management devices are often required at an early age and expose patients to device-related complications. The present study drew upon four decades of experience at a tertiary adult congenital heart disease ACHD center and aimed to investigate the indication for cardiac implantable electronic devices (CIEDs) and predictors of late device-related complication requiring re-intervention...
March 6, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/28290961/kcnq1-gene-variants-in-large-asymptomatic-populations-considerations-for-genomic-screening-of-military-cohorts
#17
Paul Kruszka, Karin Weiss, Donald W Hadley
INTRODUCTION: The advances in genomic technology of large populations make the potential for genomic screening of military cohorts and recruits feasible, affording the potential to identify at-risk individuals before occurrence of potentially life-threatening events. Exploring sudden cardiac death, known to cause significant morbidity and mortality in young military service members, we focused on the most common gene associated with long QT syndrome (LQTS), KCNQ1. MATERIALS AND METHODS: Using the publicly available database Exome Aggregation Consortium as a surrogate for a military population, variants in KCNQ1 were filtered on the basis of population prevalence, classification as a disease mutation in the Human Gene Mutation database, and classification as pathogenic or likely pathogenic in the ClinVar database...
March 2017: Military Medicine
https://www.readbyqxmd.com/read/28287284/long-qt-syndrome-in-pregnancy-a-successful-case-of-icd-implantation-during-the-prenatal-period
#18
Mitsutake Yano, Yoshihiro Nishida, Kentaro Kai, Terukazu Ishii, Naohiko Takahashi, Hisashi Narahara
No abstract text is available yet for this article.
March 13, 2017: Journal of Obstetrics and Gynaecology: the Journal of the Institute of Obstetrics and Gynaecology
https://www.readbyqxmd.com/read/28279743/vectrocardiography-identifies-patients-with-electrocardiographically-concealed-long-qt-syndrome-eclqts
#19
Daniel Cortez, J Martijn Bos, Michael J Ackerman
BACKGROUND: Long QT syndrome (LQTS) and genotypic subtypes are associated with distinctive T wave patterns, arrhythmogenic triggers, and corrected QT interval (QTc) risk associations. Twenty percent of patients with LQTS have normal QTc values, electrographically concealed LQTS (ecLQTS). Vectorcardiography (VCG) has value for sudden cardiac death risk assessment. OBJECTIVE: To determine the utility of VCG to identify patients with ecLQTS. METHODS: We performed a retrospective review on patients with ecLQTS, defined as resting QTc values < 440 ms...
March 6, 2017: Heart Rhythm: the Official Journal of the Heart Rhythm Society
https://www.readbyqxmd.com/read/28275941/what-is-the-role-of-cardiac-sympathetic-denervation-for-recurrent-ventricular-tachycardia
#20
REVIEW
Jonathan C Hong, Todd Crawford, Harikrishna Tandri, Kaushik Mandal
There is a subset of patients who have recurrent ventricular tachycardia despite optimal medical management with pharmacologic therapy and catheter ablation. The cardiac sympathetic nervous system is responsible for triggering and perpetuating ventricular arrhythmias, and surgery can reduce the sympathetic stimulation to the heart. Evidence supports the use of left cardiac sympathetic denervation in recurrent ventricular arrhythmias for long QT syndrome and catecholaminergic polymorphic ventricular tachycardia...
February 2017: Current Treatment Options in Cardiovascular Medicine
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