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https://www.readbyqxmd.com/read/29784533/long-qt-syndrome-a-comprehensive-review-of-the-literature-and-current-evidence
#1
REVIEW
Syed Raza Shah, Ki Park
Long QT syndrome (LQT) represents a heterogeneous family of cardiac electrophysiologic disorders characterized by QT prolongation and T-wave abnormalities on the electrocardiogram. It is commonly associated with syncope, however, sudden cardiac death can occur due to torsades de pointes. LQT is a clinical diagnosis and should be suspected in individuals on the basis of clinical presentation, family history and electrocardiogram characteristics. Management is focused on the prevention of syncope and ultimately sudden death...
May 10, 2018: Current Problems in Cardiology
https://www.readbyqxmd.com/read/29766267/-syncopes-and-channelopathies
#2
REVIEW
Johanna Müller-Leisse, Christos Zormpas, Thorben König, David Duncker, Christian Veltmann
Syncope can be the first manifestation of cardiac channelopathies, namely Brugada syndrome, long QT syndrome, short QT syndrome and catecholaminergic polymorphic ventricular tachycardia (CPVT). Patients affected by these rare diseases are at increased risk for sudden cardiac death due to ventricular tachyarrhythmias and require specific therapy and follow-up. As syncope is common in the general population, only few cases are caused by an underlying channelopathy. Nevertheless, the diagnosis should be considered in young patients with structurally normal hearts, especially if the history of syncope is typical for an arrhythmogenic cause, in the presence of characteristic echocardiogram (ECG) patterns, and if there is a family history of channelopathies or sudden cardiac death...
May 15, 2018: Herzschrittmachertherapie & Elektrophysiologie
https://www.readbyqxmd.com/read/29759629/circadian-variation-of-ventricular-arrhythmias-in-catecholaminergic-polymorphic-ventricular-tachycardia
#3
Christina Y Miyake, S Yukiko Asaki, Gregory Webster, Richard J Czosek, Joseph Atallah, Kishor Avasarala, Sri O Rao, Patricia E Thomas, Jeffrey J Kim, Santiago O Valdes, Caridad de la Uz, Yunfei Wang, Xander H T Wehrens, Dominic Abrams
OBJECTIVES: The aim of this paper was to investigate whether ventricular arrhythmias in children with catecholaminergic polymorphic ventricular tachycardia (CPVT) show circadian patterns. BACKGROUND: Circadian arrhythmic patterns have been established in long QT, Brugada, and early repolarization, but have not been investigated in CPVT. METHODS: This is a multicenter, retrospective review of pediatric CPVT patients, age <21 years at diagnosis...
November 2017: JACC. Clinical Electrophysiology
https://www.readbyqxmd.com/read/29758301/lipid-roles-in-herg-function-and-interactions-with-drugs
#4
REVIEW
Meruyert Kudaibergenova, Laura L Perissinotti, Sergei Y Noskov
Human-ether-a-go-go-related channel (hERG) is a voltage gated potassium channel (Kv 11.1) abundantly expressed in heart and brain tissues. In addition to playing an important role in mediation of repolarizing K+ currents (IKr ) in Action Potential (AP), hERG is notorious for its propensity to interact with various medications. The drug-induced block of K+ currents across hERG channel are strongly associated with dysrhythmic conditions collectively known as drug-induced long-QT-syndrome. The recent availability of the high-resolution Cryo-EM structures for the hERG channel has provided unique opportunity to resolve structural mechanisms involved into the process of voltage-gating of hERG channels, map various roles played by components of ventricular and neuronal membranes and then to connect it to cellular pathways through which diverse chemical compounds might be affecting function of the channel...
May 11, 2018: Neuroscience Letters
https://www.readbyqxmd.com/read/29758173/lack-of-genotype-phenotype-correlation-in-families-who-had-brugada-syndrome-and-sudden-arrhythmic-death-syndrome-with-reported-pathogenic-scn1b-variants
#5
Belinda Gray, Can Hasdemir, Jodie Ingles, Takeshi Aiba, Naomasa Makita, Vincent Probst, Arthur A M Wilde, Ruth Newbury-Ecob, Mary N Sheppard, Christopher Semsarian, Raymond W Sy, Elijah R Behr
BACKGROUND: There is limited evidence that Brugada syndrome (BrS) is due to SCN1B variants (BrS5). This gene may be inappropriately included in routine genetic testing panels for BrS or sudden arrhythmic death syndrome (SADS). OBJECTIVE: We sought to characterize the genotype-phenotype correlation in families who had BrS and SADS with reportedly pathogenic SCN1B variants and to review their pathogenicity. METHODS: Families with BrS and SADS were assessed from 6 inherited arrhythmia centers worldwide, and a comprehensive literature review was performed...
May 3, 2018: Heart Rhythm: the Official Journal of the Heart Rhythm Society
https://www.readbyqxmd.com/read/29756694/effects-of-inhalation-and-intravenous-anaesthesia-on-intraoperative-cardiopulmonary-function-and-postoperative-complications-in-patients-undergoing-thoracic-surgery-a-systematic-review-and-meta-analysis
#6
Qian-Yun Pang, Ran An, Hong-Liang Liu
INTRODUCTION: There is a high incidence of postoperative cardiopulmonary complications after thoracic surgery with one lung ventilation (OLV), the effect of general anaesthetics on intraoperative cardiopulmonary function and postoperative complications is still unclear. EVIDENCE ACQUISITION: We searched the Embase, Pubmed, Cochrane Library, Springer, Wiley, CNKI, VIP and Wanfang databases for randomized controlled trials (RCTs) in which inhalation anaesthesia and intravenous anaesthesia were compared; intraoperative cardiopulmonary function and postoperative complications were assessed in patients undergoing thoracic surgery with intraoperative one-lung ventilation (OLV)...
May 11, 2018: Minerva Anestesiologica
https://www.readbyqxmd.com/read/29744527/inherited-primary-arrhythmia-disorders-cardiac-channelopathies-and-sports-activity
#7
REVIEW
S Marrakchi, I Kammoun, E Bennour, L Laroussi, M Ben Miled, S Kachboura
Sudden cardiac death (SCD) in an apparently healthy individual is a tragedy. It is important to identify the cause of death and to prevent SCD in potentially at-risk family members. Inherited primary arrhythmia disorders are associated with exercise-related SCD. Despite the well-known benefits of exercise, exercise restriction has been a historical mainstay of therapy for these conditions. However, since familiarity with inherited arrhythmia conditions has increased and patients are often children and young adults, it is necessary to reassess the treatment guidelines regarding exercise constraints...
May 9, 2018: Herz
https://www.readbyqxmd.com/read/29740400/the-role-of-kcnq1-mutations-and-maternal-beta-blocker-use-during-pregnancy-in-the-growth-of-children-with-long-qt-syndrome
#8
Heta Huttunen, Matti Hero, Mitja Lääperi, Johanna Känsäkoski, Heikki Swan, Joel A Hirsch, Päivi J Miettinen, Taneli Raivio
Objective: Two missense mutations in KCNQ1 , an imprinted gene that encodes the alpha subunit of the voltage-gated potassium channel Kv7.1, cause autosomal dominant growth hormone deficiency and maternally inherited gingival fibromatosis. We evaluated endocrine features, birth size, and subsequent somatic growth of patients with long QT syndrome 1 (LQT1) due to loss-of-function mutations in KCNQ1 . Design: Medical records of 104 patients with LQT1 in a single tertiary care center between 1995 and 2015 were retrospectively reviewed...
2018: Frontiers in Endocrinology
https://www.readbyqxmd.com/read/29728395/-scn5a-na-v-1-5-variant-functional-perturbation-and-clinical-presentation-variants-of-a-certain-significance
#9
Brett M Kroncke, Andrew M Glazer, Derek K Smith, Jeffrey D Blume, Dan M Roden
BACKGROUND: Accurately predicting the impact of rare nonsynonymous variants on disease risk is an important goal in precision medicine. Variants in the cardiac sodium channel SCN5A (protein NaV 1.5; voltage-dependent cardiac Na+ channel) are associated with multiple arrhythmia disorders, including Brugada syndrome and long QT syndrome. Rare SCN5A variants also occur in ≈1% of unaffected individuals. We hypothesized that in vitro electrophysiological functional parameters explain a statistically significant portion of the variability in disease penetrance...
May 2018: Circulation. Genomic and precision medicine
https://www.readbyqxmd.com/read/29723683/beyond-the-length-and-look-of-repolarization-defining-the-non-qtc-electrocardiographic-profiles-of-patients-with-congenital-long-qt-syndrome
#10
Conor M Lane, J Martijn Bos, Ram K Rohatgi, Michael J Ackerman
BACKGROUND: Little is known about the spectrum and prevalence of ECG features beyond the length and morphology of repolarization in patients with congenital long QT syndrome (LQTS). OBJECTIVE: To characterize the full ECG phenotype of LQTS patients and evaluate differences by age and LQTS genotype. METHODS: Retrospective review of 943 patients with LQTS (57% female, median age 25 years; IQR 9 - 34 years) was performed. Comprehensive analysis of their initial evaluation ECG was performed using definitions outlined in Heart Rhythm Society guidelines...
April 30, 2018: Heart Rhythm: the Official Journal of the Heart Rhythm Society
https://www.readbyqxmd.com/read/29713203/evaluating-cardiac-risk-exposure-response-analysis-in-early-clinical-drug-development
#11
REVIEW
Julie Grenier, Sabina Paglialunga, Bruce H Morimoto, Robert M Lester
The assessment of a drug's cardiac liability has undergone considerable metamorphosis by regulators since International Council for Harmonization of Technical Requirement for Pharmaceuticals for Human Use E14 guideline was introduced in 2005. Drug developers now have a choice in how proarrhythmia risk can be evaluated; the options include a dedicated thorough QT (TQT) study or exposure response (ER) modeling of intensive electrocardiogram (ECG) captured in early clinical development. The alternative approach of ER modeling was incorporated into a guidance document in 2015 as a primary analysis tool which could be utilized in early phase dose escalation studies as an option to perform a dedicated TQT trial...
2018: Drug, Healthcare and Patient Safety
https://www.readbyqxmd.com/read/29706106/the-impact-of-pazopanib-on-the-cardiovascular-system
#12
Cody N Justice, Mohamed H Derbala, Tesla M Baich, Amber N Kempton, Aaron S Guo, Thai H Ho, Sakima A Smith
Pazopanib is an approved treatment for renal cell carcinoma and a second-line treatment for nonadipocytic soft-tissue sarcoma. However, its clinical efficacy is limited by its cardiovascular side effects. Pazopanib and other vascular endothelial growth factor receptor tyrosine kinase inhibitors have been associated with the development of hypertension, QT interval prolongation, and other cardiovascular events; however, these mechanisms are largely unknown. Gaining a deeper understanding of these mechanisms is essential for the development of appropriate surveillance strategies and possible diagnostic biomarkers to allow us to monitor patients and modulate therapy prior to significant cardiac insult...
January 1, 2018: Journal of Cardiovascular Pharmacology and Therapeutics
https://www.readbyqxmd.com/read/29704693/cardiac-arrest-during-spine-surgery-in-the-prone-position-case-report-and-review-of-the-literature
#13
REVIEW
Victor E Staartjes, Shiva A Schillevoort, Patricia G Blum, J Peter van Tintelen, Wouter E Kok, Marc L Schröder
OBJECTIVE: Intraoperative cardiac arrest (CA) is usually attributable to pre-existing disease, or to intraoperative complications. In rare cases, intraoperative stress can demask certain genetic diseases, such as Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT). It is essential that neurosurgeons are aware of the etiologies, risk factors, and initial management of CA during surgery in the prone position. METHODS: We present a case of CA directly after spinal fusion for lumbar spondylolisthesis, and review the literature on cardiac arrests during spinal neurosurgery in the prone position...
April 26, 2018: World Neurosurgery
https://www.readbyqxmd.com/read/29698642/obesity-and-ventricular-repolarization-a-comprehensive-review
#14
REVIEW
Jad Omran, Brian P Bostick, Albert K Chan, Martin A Alpert
Obesity is known to be a strong predictor of sudden cardiac death. For this reason, concern exists that this association may be related to delayed ventricular repolarization (VR), which has been extensively studied in overweight and obese patients. The corrected QT interval (QTc) and QT or QTc dispersion have been the most commonly-used electrocardiographic methods for assessing VR. Multiple controlled studies demonstrated that QTc and QT or QTc dispersion were significantly longer/greater in overweight and obese subjects than in normal weight controls...
April 23, 2018: Progress in Cardiovascular Diseases
https://www.readbyqxmd.com/read/29688407/mental-stress-test-a-rapid-simple-and-efficient-test-to-unmask-long-qt-syndrome
#15
Pauline Etienne, François Huchet, Nathalie Gaborit, Julien Barc, Aurélie Thollet, Florence Kyndt, Béatrice Guyomarch, Hervé Le Marec, Flavien Charpentier, Jean-Jacques Schott, Richard Redon, Vincent Probst, Jean-Baptiste Gourraud
Aims: QT prolongation during mental stress test (MST) has been associated with familial idiopathic ventricular fibrillation. In long QT syndrome (LQTS), up to 30% of mutation carriers have normal QT duration. Our aim was to assess the QT response during MST, and its accuracy in the diagnosis of concealed LQTS. Methods and results: All patients who are carrier of a KCNQ1 or KCNH2 mutations without QT prolongation were enrolled. A control group was constituted of patients with negative exercise and epinephrine tests...
April 20, 2018: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
https://www.readbyqxmd.com/read/29672845/drug-induced-proarrhythmia-and-torsade-de-pointes-a-primer-for-students-and-practitioners-of-medicine-and-pharmacy
#16
REVIEW
J Rick Turner, Ignacio Rodriguez, Emily Mantovani, Gary Gintant, Peter R Kowey, Ralph J Klotzbaugh, Krishna Prasad, Philip T Sager, Norman Stockbridge, Colette Strnadova
Multiple marketing withdrawals due to proarrhythmic concerns occurred in the United States, Canada, and the United Kingdom in the late 1980s to early 2000s. This primer reviews the clinical implications of a drug's identified proarrhythmic liability, the issues associated with these safety-related withdrawals, and the actions taken by the International Council for Harmonisation of Technical Requirements for Pharmaceuticals for Human Use (ICH) and by regulatory agencies in terms of changing drug development practices and introducing new nonclinical and clinical tests to asses proarrhythmic liability...
April 19, 2018: Journal of Clinical Pharmacology
https://www.readbyqxmd.com/read/29672598/long-qt-molecular-autopsy-in-sudden-unexplained-death-in-the-young-1-40-years-old-lessons-learnt-from-an-eight-year-experience-in-new-zealand
#17
Luciana Marcondes, Jackie Crawford, Nikki Earle, Warren Smith, Ian Hayes, Paul Morrow, Tom Donoghue, Amanda Graham, Donald Love, Jonathan R Skinner
BACKGROUND: To review long QT syndrome molecular autopsy results in sudden unexplained death in young (SUDY) between 2006 and 2013 in New Zealand. METHODS: Audit of the LQTS molecular autopsy results, cardiac investigations and family screening data from gene-positive families. RESULTS: During the study period, 365 SUDY cases were referred for molecular autopsy. 128 cases (35%) underwent LQTS genetic testing. 31 likely pathogenic variants were identified in 27 cases (21%); SCN5A (14/31, 45%), KCNH2 (7/31, 22%), KCNQ1 (4/31, 13%), KCNE2 (3/31, 10%), KCNE1 (2/31, 7%), KCNJ2 (1/31, 3%)...
2018: PloS One
https://www.readbyqxmd.com/read/29661707/the-genetic-architecture-of-long-qt-syndrome-a-critical-reappraisal
#18
REVIEW
John R Giudicessi, Arthur A M Wilde, Michael J Ackerman
Collectively, the completion of the Human Genome Project and subsequent development of high-throughput next-generation sequencing methodologies have revolutionized genomic research. However, the rapid sequencing and analysis of thousands upon thousands of human exomes and genomes has taught us that most genes, including those known to cause heritable cardiovascular disorders such as long QT syndrome, harbor an unexpected background rate of rare, and presumably innocuous, non-synonymous genetic variation. In this Review, we aim to reappraise the genetic architecture underlying both the acquired and congenital forms of long QT syndrome by examining how the clinical phenotype associated with and background genetic variation in long QT syndrome-susceptibility genes impacts the clinical validity of existing gene-disease associations and the variant classification and reporting strategies that serve as the foundation for diagnostic long QT syndrome genetic testing...
March 30, 2018: Trends in Cardiovascular Medicine
https://www.readbyqxmd.com/read/29643708/evidence-based-review-and-appraisal-of-the-use-of-droperidol-in-the-emergency-department
#19
REVIEW
Pei-Chun Lai, Yen-Ta Huang
Droperidol is a short-acting, potent dopamine D2 antagonist that can pass through the blood-brain barrier. A black box warning was issued for droperidol by the United States Food and Drug Administration in 2001 because of a risk of development of torsades de pointes induced by QT prolongation. Many experts feel that the incidence of arrhythmia is overestimated, and low-dose droperidol is almost always used by anesthesiologists for postoperative nausea and vomiting. In this review, we used evidence-based analysis to appraise high-quality studies with a low risk of bias published after 2001 on the use of droperidol in the emergency department (ED)...
January 2018: Ci Ji Yi Xue za Zhi, Tzu-chi Medical Journal
https://www.readbyqxmd.com/read/29628380/quadriceps-tendon-autograft-in-anterior-cruciate-ligament-reconstruction-a-systematic-review
#20
REVIEW
Eoghan T Hurley, Manuel Calvo-Gurry, Dan Withers, Shane K Farrington, Ray Moran, Cathal J Moran
PURPOSE: To systematically review the current evidence to ascertain whether quadriceps tendon autograft (QT) is a viable option in anterior cruciate ligament reconstruction. METHODS: A literature review was conducted in accordance with Preferred Reporting Items for Systematic Reviews and Meta-analyses (PRISMA) guidelines. Cohort studies comparing QT with bone-patellar tendon-bone autograft (BPTB) or hamstring tendon autograft (HT) were included. Clinical outcomes were compared, with all statistical analyses performed using IBM SPSS Statistics for Windows, version 22...
April 5, 2018: Arthroscopy: the Journal of Arthroscopic & related Surgery
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