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https://www.readbyqxmd.com/read/28776165/prolongation-of-the-qtc-interval-in-hiv-infected-individuals-compared-to-the-general-population
#1
Nico Reinsch, Marina Arendt, Marie Henrike Geisel, Christina Schulze, Volker Holzendorf, Anna Warnke, Till Neumann, Norbert H Brockmeyer, Dirk Schadendorf, Lewin Eisele, Raimund Erbel, Susanne Moebus, Karl-Heinz Jöckel, Stefan Esser
OBJECTIVES: Prolonged QT interval is associated with arrhythmias and sudden death. An increased prevalence of QT interval prolongation in human immunodeficiency virus-infected (HIV) subjects was previously described. The impact of different medications and HIV infection itself on the QT interval is rarely investigated in large HIV+ cohorts. METHODS: We compared QT interval measurement in 496 HIV(+) patients of the HIV-HEART study (HIVH) and 992 sex- and age-matched controls of the population-based German Heinz Nixdorf Recall study (HNR)...
August 3, 2017: Infection
https://www.readbyqxmd.com/read/28407228/deleterious-protein-altering-mutations-in-the-scn10a-voltage-gated-sodium-channel-gene-are-associated-with-prolonged-qt
#2
Maen D Abou Ziki, Sara B Seidelmann, Emily Smith, Gourg Atteya, Yuexin Jiang, Rodolfo Gil Fernandes, Mark A Marieb, Joseph G Akar, Arya Mani
Long QT syndrome (LQT) is a pro-arrhythmogenic condition with life threatening complications. Fifteen genes have been associated with congenital LQT however, the genetic causes remain unknown in more than 20% of cases. Eighteen patients with history of palpitations, presyncope, syncope and prolonged QT were referred to the Yale Cardiovascular Genetics Program. All subjects underwent whole exome sequencing (WES) followed by confirmatory Sanger sequencing. Mutation burden analysis was carried out using WES data from sixteen subjects with no identifiable cause of LQT...
April 13, 2017: Clinical Genetics
https://www.readbyqxmd.com/read/28315637/trpm4-non-selective-cation-channel-variants-in-long-qt-syndrome
#3
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
#4
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)...
September 2017: Heart: Official Journal of the British Cardiac Society
https://www.readbyqxmd.com/read/28216547/mid-term-follow-up-of-school-aged-children-with-borderline-long-qt-interval
#5
Aya Miyazaki, Heima Sakaguchi, Yu Matsumura, Yosuke Hayama, Kanae Noritake, Jun Negishi, Etsuko Tsuda, Yoshihiro Miyamoto, Takeshi Aiba, Wataru Shimizu, Kengo Kusano, Isao Shiraishi, Hideo Ohuchi
BACKGROUND: There are no definitive diagnostic criteria or follow-up strategies for long QT syndrome (LQTS) in children with a borderline long QT interval (b-LQT).Methods and Results:We retrospectively evaluated the clinical course, genetic testing results, corrected QT interval (QTc), and LQTS score of 59 school-aged children (5-18 years old) with a b-LQT (400≤QTc<500 ms). Syncope, but neither aborted cardiac arrest nor sudden cardiac death, occurred in 2 patients during the follow-up (6±3...
April 25, 2017: Circulation Journal: Official Journal of the Japanese Circulation Society
https://www.readbyqxmd.com/read/28150739/alterations-in-the-carnitine-cycle-in-a-mouse-model-of-rett-syndrome
#6
Sabrina Mucerino, Anna Di Salle, Nicola Alessio, Sabrina Margarucci, Raffaella Nicolai, Mariarosa A B Melone, Umberto Galderisi, Gianfranco Peluso
Rett syndrome (RTT) is a neurodevelopmental disease that leads to intellectual deficit, motor disability, epilepsy and increased risk of sudden death. Although in up to 95% of cases this disease is caused by de novo loss-of-function mutations in the X-linked methyl-CpG binding protein 2 gene, it is a multisystem disease associated also with mitochondrial metabolic imbalance. In addition, the presence of long QT intervals (LQT) on the patients' electrocardiograms has been associated with the development of ventricular tachyarrhythmias and sudden death...
February 2, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28096388/competition-of-calcified-calmodulin-n-lobe-and-pip2-to-an-lqt-mutation-site-in-kv7-1-channel
#7
William Sam Tobelaim, Meidan Dvir, Guy Lebel, Meng Cui, Tal Buki, Asher Peretz, Milit Marom, Yoni Haitin, Diomedes E Logothetis, Joel Alan Hirsch, Bernard Attali
Voltage-gated potassium 7.1 (Kv7.1) channel and KCNE1 protein coassembly forms the slow potassium current IKS that repolarizes the cardiac action potential. The physiological importance of the IKS channel is underscored by the existence of mutations in human Kv7.1 and KCNE1 genes, which cause cardiac arrhythmias, such as the long-QT syndrome (LQT) and atrial fibrillation. The proximal Kv7.1 C terminus (CT) binds calmodulin (CaM) and phosphatidylinositol-4,5-bisphosphate (PIP2), but the role of CaM in channel function is still unclear, and its possible interaction with PIP2 is unknown...
January 31, 2017: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/27690427/discovery-of-dihydrobenzoxazepinone-gs-6615-late-sodium-current-inhibitor-late-inai-a-phase-ii-agent-with-demonstrated-preclinical-anti-ischemic-and-antiarrhythmic-properties
#8
Jeff A Zablocki, Elfatih Elzein, Xiaofen Li, Dmitry O Koltun, Eric Q Parkhill, Tetsuya Kobayashi, Ruben Martinez, Britton Corkey, Haibo Jiang, Thao Perry, Rao Kalla, Gregory T Notte, Oliver Saunders, Michael Graupe, Yafan Lu, Chandru Venkataramani, Juan Guerrero, Jason Perry, Mark Osier, Robert Strickley, Gongxin Liu, Wei-Qun Wang, Lufei Hu, Xiao-Jun Li, Nesrine El-Bizri, Ryoko Hirakawa, Kris Kahlig, Cheng Xie, Cindy Hong Li, Arvinder K Dhalla, Sridharan Rajamani, Nevena Mollova, Daniel Soohoo, Eve-Irene Lepist, Bernard Murray, Gerry Rhodes, Luiz Belardinelli, Manoj C Desai
Late sodium current (late INa) is enhanced during ischemia by reactive oxygen species (ROS) modifying the Nav 1.5 channel, resulting in incomplete inactivation. Compound 4 (GS-6615, eleclazine) a novel, potent, and selective inhibitor of late INa, is currently in clinical development for treatment of long QT-3 syndrome (LQT-3), hypertrophic cardiomyopathy (HCM), and ventricular tachycardia-ventricular fibrillation (VT-VF). We will describe structure-activity relationship (SAR) leading to the discovery of 4 that is vastly improved from the first generation late INa inhibitor 1 (ranolazine)...
October 3, 2016: Journal of Medicinal Chemistry
https://www.readbyqxmd.com/read/27646833/the-effects-of-pharmacological-compounds-on-beat-rate-variations-in-human-long-qt-syndrome-cardiomyocytes
#9
Jukka Kuusela, Jiyeong Kim, Esa Räsänen, Katriina Aalto-Setälä
Healthy human heart rate fluctuates overtime showing long-range fractal correlations. In contrast, various cardiac diseases and normal aging show the breakdown of fractal complexity. Recently, it was shown that human induced pluripotent stem cell-derived cardiomyocytes (hiPSC-CMs) intrinsically exhibit fractal behavior as in humans. Here, we investigated the fractal complexity of hiPSC-derived long QT-cardiomyocytes (LQT-CMs). We recorded extracellular field potentials from hiPSC-CMs at baseline and under the effect of various compounds including β-blocker bisoprolol, ML277, a specific and potent IKs current activator, as well as JNJ303, a specific IKs blocker...
December 2016: Stem Cell Reviews
https://www.readbyqxmd.com/read/27586292/current-voltage-relationship-for-late-na-current-in-adult-rat-ventricular-myocytes
#10
R B Clark, W R Giles
It is now well established that the slowly inactivating component of the Na(+) current (INa-L) in the mammalian heart is a significant regulator of the action potential waveform. This insight has led to detailed studies of the role of INa-L in a number of important and challenging pathophysiological settings. These include genetically based ventricular arrhythmias (LQT 1, 2, and 3), ventricular arrhythmias arising from progressive cardiomyopathies (including diabetic), and proarrhythmic abnormalities that develop during local or global ventricular ischemia...
2016: Current Topics in Membranes
https://www.readbyqxmd.com/read/27401738/prominent-qtc-prolongation-in-a-patient-with-a-rare-variant-in-the-cardiac-ryanodine-receptor-gene
#11
Yuki Taniguchi, Aya Miyazaki, Heima Sakaguchi, Yousuke Hayama, Norihiro Ebishima, Jun Negishi, Kanae Noritake, Yoshihiro Miyamoto, Wataru Shimizu, Takeshi Aiba, Hideo Ohuchi
We report the case of a 12-year-old female patient with a history of four syncopal episodes related to exercise over 2 years and who showed prominent QTc prolongation on electrocardiogram; therefore, she was clinically diagnosed with long QT syndrome type-1. However, genetic analysis did not identify any LQT-related genes but showed a rare missense variant in the cardiac ryanodine receptor gene. From the results of drug-loading tests, administration of oral propranolol was initiated; thereafter, she experienced no syncopal episodes...
February 2017: Heart and Vessels
https://www.readbyqxmd.com/read/27394160/experience-with-bisoprolol-in-long-qt1-and-long-qt2-syndrome
#12
MULTICENTER STUDY
Christian Steinberg, Gareth J Padfield, Basil Al-Sabeq, Arnon Adler, John A Yeung-Lai-Wah, Charles R Kerr, Marc W Deyell, Jason G Andrade, Matthew T Bennett, Raymond Yee, George J Klein, Martin Green, Zachary W M Laksman, Andrew D Krahn, Santabhanu Chakrabarti
BACKGROUND: The protective effect of beta-blockers in patients with inherited Long-QT syndrome is well established. Recent reports have suggested that beta-blockers are not equally effective in Long-QT (LQT). Bisoprolol is an attractive candidate for use in LQT because of its cardioselective properties and favorable side-effect profile. METHODS: We performed a retrospective cohort study of 114 consecutive patients with gene-positive Long-QT syndrome type 1 (LQT1) or Long-QT syndrome type 2 (LQT2) treated with bisoprolol, nadolol or atenolol with a total of 580 person-years of follow-up...
November 2016: Journal of Interventional Cardiac Electrophysiology: An International Journal of Arrhythmias and Pacing
https://www.readbyqxmd.com/read/27054604/influence-of-pregnancy-in-patients-with-congenital-long-qt-syndrome
#13
Lohit Garg, Jalaj Garg, Parasuram Krishnamoorthy, Amy Ahnert, Neeraj Shah, Raman S Dusaj, Babak Bozorgnia
Congenital long QT syndrome (LQTS) is a disorder of myocardial repolarization and is characterized by a prolonged QT interval on an electrocardiogram. A prolonged QT predisposes patients to an increased risk of syncope and sudden cardiac death secondary to polymorphic ventricular tachycardia. Several mutations linked to the LQTS have been identified, the most common of which have been found in the potassium channel KCNQ1 (LQT1) and hERG (LQT2) genes and in the sodium channel SCN5A (LQT3) gene. Female sex is an independent risk factor for the development of torsades de pointes in LQTS...
July 2017: Cardiology in Review
https://www.readbyqxmd.com/read/26632536/pronounced-shortening-of-qt-interval-with-mexiletine-infusion-test-in-patients-with-type-3-congenital-long-qt-syndrome
#14
Moritoshi Funasako, Takeshi Aiba, Kohei Ishibashi, Ikutaro Nakajima, Koji Miyamoto, Yuko Inoue, Hideo Okamura, Takashi Noda, Shiro Kamakura, Toshihisa Anzai, Teruo Noguchi, Satoshi Yasuda, Yoshihiro Miyamoto, Kengo Fukushima Kusano, Hisao Ogawa, Wataru Shimizu
BACKGROUND: Mexiletine is often used for medical therapy in LQT3 patients, however, the usefulness of mexiletine infusion test for LQT3 patients has not been reported. The aim of this study was to evaluate the usefulness of mexiletine infusion test for detecting LQT3 patients. METHODS AND RESULTS: We analyzed response in 12-lead electrocardiogram parameters measured in II or V5 to i.v. mexiletine infusion (2 mg/kg) during sinus rhythm among 31 genotype-positive LQT patients (29 ± 18 years, 12 male)...
2016: Circulation Journal: Official Journal of the Japanese Circulation Society
https://www.readbyqxmd.com/read/26548443/two-cases-of-lqt-syndrome-with-malignant-syncope-after-switch-from-propranolol-to-bisoprolol
#15
Milos Kesek, Annika Rydberg, Steen M Jensen
Propranolol in slow-release form has been the first-line treatment in long QT (LQT) until it was withdrawn from the market. We describe two cases where a switch to bisoprolol resulted in worsening of arrhythmia control: A man with LQT2, asymptomatic on propranolol, experienced syncope after switching to bisoprolol 5 mg daily. He switched back to propranolol and has remained asymptomatic during subsequent 12 months. A man with classical Jervell Lange-Nielsen syndrome, previous gangliectomy, and ICD implantation, switched to bisoprolol 5 mg daily...
March 2016: Pacing and Clinical Electrophysiology: PACE
https://www.readbyqxmd.com/read/26545904/bag3-related-myopathy-polyneuropathy-and-cardiomyopathy-with-long-qt-syndrome
#16
Anna Kostera-Pruszczyk, Małgorzata Suszek, Rafał Płoski, Maria Franaszczyk, Anna Potulska-Chromik, Piotr Pruszczyk, Elżbieta Sadurska, Justyna Karolczak, Anna M Kamińska, Maria Jolanta Rędowicz
BAG3 belongs to BAG family of molecular chaperone regulators interacting with HSP70 and anti-apoptotic protein Bcl-2. It is ubiquitously expressed with strong expression in skeletal and cardiac muscle, and is involved in a panoply of cellular processes. Mutations in BAG3 and aberrations in its expression cause fulminant myopathies, presenting with progressive limb and axial muscle weakness, and respiratory insufficiency and neuropathy. Herein, we report a sporadic case of a 15-years old girl with symptoms of myopathy, demyelinating polyneuropathy and asymptomatic long QT syndrome...
December 2015: Journal of Muscle Research and Cell Motility
https://www.readbyqxmd.com/read/26301350/a-rare-association-with-suffered-cardiac-arrest-long-qt-interval-and-syndactyly-timothy-syndrome-lqt-8
#17
Yakup Ergül, İsa Özyılmaz, Sertaç Haydın, Alper Güzeltaş, Volkan Tuzcu
No abstract text is available yet for this article.
August 2015: Anatolian Journal of Cardiology
https://www.readbyqxmd.com/read/26271031/estradiol-regulates-human-qt-interval-acceleration-of-cardiac-repolarization-by-enhanced-kcnh2-membrane-trafficking
#18
Lars Anneken, Stefan Baumann, Patrick Vigneault, Peter Biliczki, Corinna Friedrich, Ling Xiao, Zenawit Girmatsion, Ina Takac, Ralf P Brandes, Stefan Kissler, Inka Wiegratz, Sven Zumhagen, Birgit Stallmeyer, Stefan H Hohnloser, Thomas Klingenheben, Eric Schulze-Bahr, Stanley Nattel, Joachim R Ehrlich
BACKGROUND: Modulation of cardiac repolarization by sexual hormones is controversial and hormonal effects on ion channels remain largely unknown. In the present translational study, we therefore assessed the relationship between QTc duration and gonadal hormones and studied underlying mechanisms. METHODS AND RESULTS: We measured hormone levels and QTc intervals in women during clomiphene stimulation for infertility and women before, during, and after pregnancy. Three heterozygous LQT-2 patients (KCNH2-p...
February 14, 2016: European Heart Journal
https://www.readbyqxmd.com/read/26118557/obstructive-sleep-apnea-in-patients-with-congenital-long-qt-syndrome-implications-for-increased-risk-of-sudden-cardiac-death
#19
Abu S Shamsuzzaman, Virend K Somers, Timothy K Knilans, Michael J Ackerman, Yu Wang, Raouf S Amin
BACKGROUND: Congenital long QT syndrome (LQTS) is a familial arrhythmogenic cardiac channelopathy characterized by prolonged ventricular repolarization and increased risk of torsades de pointes-mediated syncope, seizures, and sudden cardiac death (SCD). QT prolongation corrected for heart rate (QTc) is an important diagnostic and prognostic feature in LQTS. Obstructive sleep apnea (OSA) has been increasingly implicated in the pathogenesis of cardiovascular disease, including arrhythmias and SCD...
July 1, 2015: Sleep
https://www.readbyqxmd.com/read/26070071/electrocardiographic-screening-for-prolonged-qt-interval-to-reduce-sudden-cardiac-death-in-psychiatric-patients-a-cost-effectiveness-analysis
#20
Antoine Poncet, Baris Gencer, Marc Blondon, Marianne Gex-Fabry, Christophe Combescure, Dipen Shah, Peter J Schwartz, Marie Besson, François R Girardin
IMPORTANCE: Sudden cardiac death is a leading cause of mortality in psychiatric patients. Long QT (LQT) is common in this population and predisposes to Torsades-de-Pointes (TdP) and subsequent mortality. OBJECTIVE: To estimate the cost-effectiveness of electrocardiographic screening to detect LQT in psychiatric inpatients. DESIGN, SETTING, AND PARTICIPANTS: We built a decision analytic model based on a decision tree to evaluate the cost-effectiveness and utility of LQT screening from a health care perspective...
2015: PloS One
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