9,971 results match your criteria Long QT Syndrome


Concealed congenital long QT syndrome during velopharyngeal dysfunction correction: a case report.

J Dent Anesth Pain Med 2020 Jun 24;20(3):165-171. Epub 2020 Jun 24.

Department of Anesthesia and Pain Medicine, School of Medicine, Pusan National University, Busan, Korea.

The congenital long QT syndrome (LQTS) is an inherited cardiac disorder characterized by increased QT intervals and a tendency to experience ventricular tachycardia, which can cause fainting, heart failure, or sudden death. A 4-year-old female patient undergoing velopharyngeal correction surgery under general anesthesia suddenly developed Torsades de pointes. Although the patient spontaneously resolved to sinus rhythm without treatment, subsequent QT prolongation persisted. Read More

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http://dx.doi.org/10.17245/jdapm.2020.20.3.165DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7321742PMC

Hydroxychloroquine efficacy and safety in preventing SARS-CoV-2 infection and COVID-19 disease severity during pregnancy (COVID-Preg): a structured summary of a study protocol for a randomised placebo controlled trial.

Trials 2020 Jul 2;21(1):607. Epub 2020 Jul 2.

ISGlobal, Hospital Clínic-Universitat de Barcelona, Barcelona, Spain.

Objectives: The primary objectives of the study are: 1. To assess the effect of hydroxychloroquine (HCQ) in reducing SARS-CoV-2 viral shedding by PCR in infected pregnant women with mild symptoms. 2. Read More

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http://dx.doi.org/10.1186/s13063-020-04557-yDOI Listing

Noninvasive Fetal Electrocardiography in the Diagnosis of Long QT Syndrome: A Case Series.

Fetal Diagn Ther 2020 Jul 2:1-6. Epub 2020 Jul 2.

Division of Cardiology, Children's National Hospital, Washington, District of Columbia, USA.

Introduction: Early detection and monitoring for malignant arrhythmias is fundamental to prenatal care in long QT syndrome (LQTS). Recently, we studied the feasibility of isolating the fetal electrocardiogram (fECG) and measuring electrocardiographic intervals with a noninvasive fECG device using blind source separation with reference signal. Our aim was to evaluate the ability of fECG to diagnose LQTS. Read More

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http://dx.doi.org/10.1159/000508043DOI Listing

Anticancer drugs and COVID-19 antiviral treatments in patients with cancer: What can we safely use?

Eur J Cancer 2020 Jun 10;136:1-3. Epub 2020 Jun 10.

Sorbonne Université, Assistance Publique - Hôpitaux de Paris, Pitié-Salpêtrière Hospital, Department of Oncology, F-75013 Paris, France; Institut Universitaire de Cancérologie. AP-HP Sorbonne Université, Paris, France; INSERM, Institut Pierre Louis d'Epidémiologie et de Santé Publique, équipe Theravir, France.

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http://dx.doi.org/10.1016/j.ejca.2020.05.027DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7284279PMC

Effects of Hydroxychloroquine Treatment on QT Interval.

Heart Rhythm 2020 Jun 28. Epub 2020 Jun 28.

Department of Medicine, University of Minnesota, Minneapolis, MN, USA; Division of Cardiology, Minneapolis VA Health Care System, Minneapolis, MN, USA. Electronic address:

Background: Hydroxychloroquine (HCQ) has been promoted as a potential treatment for COVID-19 but there are safety concerns.

Objectives: To determine the effect of HCQ treatment on QT interval METHODS: We retrospectively studied the electrocardiograms of 819 patients treated with HCQ for rheumatologic diseases from 2000 to 2020. The primary outcome was corrected QT (QTc) interval, by Bazett formula, during HCQ therapy. Read More

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http://dx.doi.org/10.1016/j.hrthm.2020.06.029DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7321659PMC

Trend survey on adverse event profiles of antipsychotic long-acting injections and oral agents using the Japanese adverse drug event report database.

Psychiatry Res 2020 Jun 23;291:113249. Epub 2020 Jun 23.

Department of Psychiatry, Fujita Health University School of Medicine, Toyoake, Aichi, Japan.

This study aims to assess the differences in adverse event profiles of long-acting injectable antipsychotics (LAIs) and oral antipsychotics (OAPs) using real-world data in the Japanese Adverse Drug Event Report database. Reporting odds ratios were determined using disproportionality analysis to estimate the risk of adverse events for LAIs and OAPs. Differences in adverse event profiles between formulations were determined after propensity score matching. Read More

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http://dx.doi.org/10.1016/j.psychres.2020.113249DOI Listing

Acquired Long QT Syndrome after Acute Myocardial Infarction: A Rare but Potentially Fatal Entity.

Tex Heart Inst J 2020 Apr;47(2):163-164

Division of Cardiology, David Geffen School of Medicine at UCLA, Los Angeles, California 90025.

Acquired long QT syndrome is typically caused by medications, electrolyte disturbances, bradycardia, or catastrophic central nervous system events. We report a case of myocardial infarction-related acquired long QT syndrome in a 58-year-old woman that had no clear cause and progressed to torsades de pointes requiring treatment with isoproterenol and magnesium. Despite negative results of DNA testing against a known panel of genetic mutations and polymorphisms associated with long QT syndrome, the patient's family history of fatal cardiac disease suggests a predisposing genetic component. Read More

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http://dx.doi.org/10.14503/THIJ-18-6872DOI Listing

The Citrus Flavonoid Hesperetin Has an Inadequate Anti-Arrhythmic Profile in the ΔKPQ Na1.5 Mutant of the Long QT Type 3 Syndrome.

Biomolecules 2020 Jun 24;10(6). Epub 2020 Jun 24.

Laboratory of Ion Channel Research, Department of Cellular and Molecular Medicine, KU Leuven, VIB Center for Brain & Disease Research, 3000 Leuven, Belgium.

Type 3 long QT syndromes (LQT3) are associated with arrhythmogenic gain-of-function mutations in the cardiac voltage-gated Na channel (hNa1.5). The citrus flavanone hesperetin (HSP) was previously suggested as a template molecule to develop new anti-arrhythmic drugs, as it blocks slowly-inactivating currents carried by the LQT3-associated hNa1. Read More

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http://dx.doi.org/10.3390/biom10060952DOI Listing

Prevalence of sudden arrhythmic death syndrome-related genetic mutations in an Asian cohort of whole genome sequence.

Europace 2020 Jun 28. Epub 2020 Jun 28.

Division of Cardiology, Department of Internal Medicine and Cardiovascular center, National Taiwan University College of Medicine and Hospital, No. 7, Chung-Shan South Road, Taipei 100, Taiwan.

Aims: Recently, the spectrum of background mutation in the genes implicated in sudden arrhythmic death syndrome (SADS), has been elucidated in the Caucasian populations. However, this information is largely unknown in the Asian populations.

Methods And Results: We assessed the background rare variants (minor allele frequency < 0. Read More

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http://dx.doi.org/10.1093/europace/euaa092DOI Listing

'Social distancing' of the neuronal nitric oxide synthase from its adaptor protein causes arrhythmogenic trigger-substrate interactions in Long QT Syndrome.

Cardiovasc Res 2020 Jun 26. Epub 2020 Jun 26.

Department of Internal Medicine, Section of Cardiovascular Medicine, Yale New Haven Hospital.

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http://dx.doi.org/10.1093/cvr/cvaa179DOI Listing

Transgenic Rabbit Models in Proarrhythmia Research.

Front Pharmacol 2020 5;11:853. Epub 2020 Jun 5.

Department of Cardiology and Angiology I, Heart Center, University of Freiburg, Freiburg, Germany.

Drug-induced proarrhythmia constitutes a potentially lethal side effect of various drugs. Most often, this proarrhythmia is mechanistically linked to the drug's potential to interact with repolarizing cardiac ion channels causing a prolongation of the QT interval in the ECG. Despite sophisticated screening approaches during drug development, reliable prediction of proarrhythmia remains very challenging. Read More

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http://dx.doi.org/10.3389/fphar.2020.00853DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7291951PMC

Left ventricular myocardial deformation pattern, mechanical dispersion, and their relation with electrocardiogram markers in the large population-based STANISLAS cohort: insights into electromechanical coupling.

Eur Heart J Cardiovasc Imaging 2020 Jun 24. Epub 2020 Jun 24.

Université de Lorraine, INSERM, Centre d'Investigations Cliniques Plurithématique 1433, CHRU de Nancy, Inserm U1116, Rue du Morvan, 54500 Vandœuvre-lès-Nancy, France.

Aims : Mechanical alterations in patients with electrical conduction abnormalities are reported to have prognostic value in patients with left ventricular asynchrony or long QT syndrome beyond electrocardiogram (ECG) variables. Whether conduction and repolarization patterns derived from ECG are associated with speckle tracking echocardiography parameters in subjects without overt cardiac disease is yet to be investigated. To report ranges of longitudinal deformation according to conduction and repolarization values in a population-based cohort. Read More

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http://dx.doi.org/10.1093/ehjci/jeaa148DOI Listing

E1784K, the most common Brugada syndrome and long-QT syndrome type 3 mutant, disrupts sodium channel inactivation through two separate mechanisms.

J Gen Physiol 2020 Sep;152(9)

Department of Biomedical Physiology and Kinesiology, Simon Fraser University, Burnaby, BC, Canada.

Inheritable and de novo variants in the cardiac voltage-gated sodium channel, Nav1.5, are responsible for both long-QT syndrome type 3 (LQT3) and Brugada syndrome type 1 (BrS1). Interestingly, a subset of Nav1. Read More

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http://dx.doi.org/10.1085/jgp.202012595DOI Listing
September 2020

The hERG channel activator, RPR260243, enhances protective I current in early refractory reducing arrhythmogenicity in zebrafish hearts.

Am J Physiol Heart Circ Physiol 2020 Jun 19. Epub 2020 Jun 19.

Biomedical Physiology and Kinesiology, Simon Fraser University, Canada.

hERG K channels are important in cardiac repolarization and their dysfunction causes prolongation of the ventricular action potential, Long QT Syndrome, and arrhythmia. As such, approaches to augment hERG channel function, such as activator compounds, have been of significant interest due to their marked therapeutic potential. Activator compounds that hinder channel inactivation abbreviate action potential duration (APD), but carry risk of over-correction leading to Short QT Syndrome. Read More

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http://dx.doi.org/10.1152/ajpheart.00038.2020DOI Listing

If case reports be the food of knowledge, write on: our Cases of the Quarter.

Authors:
Stefan Slater

J R Coll Physicians Edinb 2020 Mar;50(1):75-79

Royal College of Physicians of Edinburgh, Edinburgh, UK, Email:

The value of publishing case reports has long been debated and the arguments are summarised. Last year, to encourage trainees, the Royal College of Physicians of Edinburgh's Senior Fellows Club inaugurated an annual prize for the best case report or case series published in the Journal of the Royal College of Physicians of Edinburgh by a doctor in training. Some of the highlights of last year's entries are reviewed, commented on and developed. Read More

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http://dx.doi.org/10.4997/JRCPE.2020.120DOI Listing

Estimates of population-based incidence of malignant arrhythmias associated with medication use-a narrative review.

Fundam Clin Pharmacol 2020 Jun 15. Epub 2020 Jun 15.

School of Pharmacy, Faculty of Science, University of Waterloo, Room 3006, 10A Victoria St. S., Kitchener, ON, Canada.

Certain medications are reported to be associated with acquired long-QT syndrome (ALQTS), which can degenerate into a potentially severe 'malignant' arrhythmia known as torsades de pointes (TdP). However, population-based estimations of the incidence of medication-associated malignant arrhythmia are limited. The purpose of this article is to review the clinical symptoms, cellular mechanism, categorization, and risk factors of these malignant arrhythmias, as well as illustrate results and methodological limitations of epidemiological literature which have previously estimated population-based incidence of ALQTS and malignant arrhythmia. Read More

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http://dx.doi.org/10.1111/fcp.12578DOI Listing

Prolonged left ventricular contraction duration in apical segments as a marker of arrhythmic risk in patients with long QT syndrome.

Europace 2020 Jun 12. Epub 2020 Jun 12.

Department of Congenital Heart Diseases, Institute of Cardiology, Alpejska 42, 04-628 Warsaw, Poland.

Aims: Long QT syndrome (LQTS) is an inherited cardiac ion channelopathy predisposing to life-threatening ventricular arrhythmias and sudden cardiac death. The aim of this study was to investigate left ventricular mechanical abnormalities in LQTS patients and establish a potential role of strain as a marker of arrhythmic risk.

Methods And Results: We included 47 patients with genetically confirmed LQTS (22 LQT1, 20 LQT2, 3 LQT3, and 2 SCN3B) and 25 healthy controls. Read More

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http://dx.doi.org/10.1093/europace/euaa107DOI Listing

Genetic Basis and Prognostic Value of Exercise QT Dynamics.

Circ Genom Precis Med 2020 Jun 11. Epub 2020 Jun 11.

Institute of Cardiovascular Science, University College London & Barts Heart Centre, St Bartholomew's Hospital, London, United Kingdom.

- Abnormal QT interval responses to heart rate (QT dynamics) is an independent risk predictor for cardiovascular disease in patients, but its genetic basis and prognostic value in a population-based cohort have not been investigated. - QT dynamics during exercise and recovery were derived in 56,643 individuals from UK Biobank without a history of cardiovascular events. Genome-wide association studies (GWAS) were conducted to identify genetic variants and bioinformatics analyses were performed to prioritize candidate genes. Read More

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http://dx.doi.org/10.1161/CIRCGEN.119.002774DOI Listing

Potential pro-arrhythmic effects of pharmacotherapy against SARS-CoV-2.

Arch Cardiol Mex 2020 ;90(Supl):36-40

Laboratorio de Arritmias. Departamento de Cardiología. Hospital Central Sur de Alta Especialidad Pemex. HCSAE, Ciudad de México/México.

The pandemic caused by the SARS-COV-2 or COVID-19 virus has been a global challenge given its high rate of transmission and lack of effective therapy or vaccine. This scenario has led to the use of various drugs that have demonstrated a potential effect against the virus in vitro. However, time has not been enough to properly evaluate their clinical effectiveness. Read More

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http://dx.doi.org/10.24875/ACM.M20000061DOI Listing

High-throughput discovery of trafficking-deficient variants in the cardiac potassium channel K11.1.

Heart Rhythm 2020 Jun 6. Epub 2020 Jun 6.

Vanderbilt Center for Arrhythmia Research and Therapeutics, Division of Clinical Pharmacology, Department of Medicine, Vanderbilt University Medical Center, Nashville, TN, USA. Electronic address:

Background: KCHN2 encodes the K11.1 potassium channel responsible for I, a major repolarization current during the cardiomyocyte action potential. Variants in KCNH2 that lead to decreased I have been associated with Type 2 Long QT syndrome (LQT2). Read More

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http://dx.doi.org/10.1016/j.hrthm.2020.05.041DOI Listing

The importance of the epinephrine provocation test for the hidden type-1 congenital long QT syndrome.

Turk Kardiyol Dern Ars 2020 Jun;48(4):434-438

Department of Cardiology, Bağcılar Training and Research Hospital, İstanbul, Turkey.

Congenital long QT syndrome (LQTS) is a genetic channelopathy associated with a high incidence of sudden cardiac death in children and young adults. QT interval prolongation is typically the primary finding on the electrocardiography (ECG) recordings, but a normal QT interval may be seen in as many as 40% of patients with LQTS due to incomplete penetrance. A normal QT interval on ECG in patients with LQTS is known as hidden LQTS. Read More

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http://dx.doi.org/10.5543/tkda.2020.40480DOI Listing

Editorial commentary: A question on proarrhythmic food: Is grapefruit "the forbidden fruit" for patients with long QT syndrome?

Trends Cardiovasc Med 2020 Jul;30(5):313-314

Tel Aviv Sourasky Medical Center and Sackler School of Medicine, Tel Aviv University, Israel. Electronic address:

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http://dx.doi.org/10.1016/j.tcm.2020.05.007DOI Listing

Bilateral cardiac sympathetic denervation in children with long-QT syndrome and catecholaminergic polymorphic ventricular tachycardia.

J Electrocardiol 2020 May 22;61:32-36. Epub 2020 May 22.

Department of Pediatric Cardiology, Saglik Bilimleri University, Mehmet Akif Ersoy Thoracic and Cardiovascular Surgery Training and Research Hospital, Istanbul, Turkey.

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http://dx.doi.org/10.1016/j.jelectrocard.2020.05.014DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7243752PMC

SCN5a overlap syndromes. This episode: long QT syndrome type 3 meets multifocal ectopic Purkinje-related premature contractions.

Authors:
Ahmad S Amin

Heart Rhythm 2020 Jun 1. Epub 2020 Jun 1.

Amsterdam University Medical Centers, Academic Medical Center, Heart Center, Department of Cardiology, Amsterdam, the Netherlands. Electronic address:

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http://dx.doi.org/10.1016/j.hrthm.2020.05.033DOI Listing

[Sinus node dysfunction, Brugada syndrome and long QT syndrome affecting the same patient : when genetics can't make head or tail of it].

Rev Med Suisse 2020 Jun;16(696):1148-1152

Service de cardiologie, Département cœur-vaisseaux, CHUV, 1011 Lausanne.

The gene SCN5A encodes the cardiac sodium channel which, through the conduction of Na+ current into the cell, generates the fast upstroke of the action potential of cardiomyocytes. Pathogenic variants of SCN5A have been causally associated to several hereditary cardiac diseases including, among others, Brugada syndrome, congenital long QT syndrome and sinus node dysfunction. Recently, overlap syndromes have been described that are characterized by the simultaneous expression of mixed clinical phenotypes among two or more hereditary cardiac diseases associated to the gene SCN5A (HCD-SCN5A). Read More

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Efficacy of hydroxychloroquine for post-exposure prophylaxis to prevent severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection among adults exposed to coronavirus disease (COVID-19): a structured summary of a study protocol for a randomised controlled trial.

Trials 2020 Jun 3;21(1):475. Epub 2020 Jun 3.

Department of Global Health,International Clinical Research Center (ICRC), University of Washington, UW Box 359927, 325 Ninth Avenue, Seattle, WA, 98104, USA.

Objectives: Primary Objective • To test the efficacy of Hydroxychloroquine (HCQ) (400 mg orally daily for 3 days then 200 mg orally daily for an additional 11 days, to complete 14 days) to prevent incident SARS-CoV-2 infection, compared to ascorbic acid among contacts of persons with SARS-CoV-2 infection Secondary objectives • To determine the safety and tolerability of HCQ as SARS-CoV-2 Post-exposure Prophylaxis (PEP) in adults • To test the efficacy of HCQ (400 mg orally daily for 3 days then 200 mg orally daily for an additional 11 days, to complete 14 days) to prevent incident SARS-CoV-2 infection 2 weeks after completing therapy, compared to ascorbic acid among contacts of persons with SARS-CoV-2 infection • To test the efficacy of HCQ to shorten the duration of SARS-CoV-2 shedding among those with SARS-CoV-2 infection in the HCQ PEP group • To test the efficacy of HCQ to prevent incident COVID-19 TRIAL DESIGN: This is a randomized, multi-center, placebo-equivalent (ascorbic acid) controlled, blinded study of HCQ PEP for the prevention of SARS-CoV-2 infection in adults exposed to the virus.

Participants: This study will enroll up to 2000 asymptomatic adults 18 to 80 years of age (inclusive) at baseline who are close contacts of persons with polymerase chain reaction (PCR)-confirmed SARS-CoV-2 or clinically suspected COVID-19 and a pending SARS-CoV-2 PCR test. This multisite trial will be conducted at seven sites in Seattle (UW), Los Angeles (UCLA), New Orleans (Tulane), Baltimore (UMB), New York City (NYU), Syracuse (SUNY-Upstate), and Boston (BMC). Read More

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http://dx.doi.org/10.1186/s13063-020-04446-4DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7268961PMC

Inotropic and Antiarrhythmic Transmural Actions of Ranolazine in a Cellular Model of Type 3 Long QT Syndrome.

Arq Bras Cardiol 2020 04 29;114(4):732-735. Epub 2020 May 29.

Departamento de Biofísica, Universidade Federal de São Paulo, São Paulo, SP, Brasil.

Ranolazine (RANO) prevents cardiac arrhythmia by blocking the late sodium current (INaL). A transmural gradient of Nav1.5 is found in the left ventricular wall of the heart. Read More

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http://dx.doi.org/10.36660/abc.20190220DOI Listing

Spatial-Temporal Signals and Clinical Indices in Electrocardiographic Imaging (II): Electrogram Clustering and T-wave Alternans.

Sensors (Basel) 2020 May 29;20(11). Epub 2020 May 29.

Department of Signal Theory and Communications, Telematics and Computing Systems, Rey Juan Carlos University, 28943 Fuenlabrada, Madrid, Spain.

During the last years, attention and controversy have been present for the first commercially available equipment being used in Electrocardiographic Imaging (ECGI), a new cardiac diagnostic tool which opens up a new field of diagnostic possibilities. Previous knowledge and criteria of cardiologists using intracardiac Electrograms (EGM) should be revisited from the newly available spatial-temporal potentials, and digital signal processing should be readapted to this new data structure. Aiming to contribute to the usefulness of ECGI recordings in the current knowledge and methods of cardiac electrophysiology, we previously presented two results: First, spatial consistency can be observed even for very basic cardiac signal processing stages (such as baseline wander and low-pass filtering); second, useful bipolar EGMs can be obtained by a digital processing operator searching for the maximum amplitude and including a time delay. Read More

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http://dx.doi.org/10.3390/s20113070DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7309062PMC

Incidence and determinants of QT interval prolongation in COVID-19 patients treated with hydroxychloroquine and azithromycin.

J Cardiovasc Electrophysiol 2020 Jun 2. Epub 2020 Jun 2.

Department of Internal Medicine, Section of Cardiovascular Medicine, Yale School of Medicine, New Haven, Connecticut.

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http://dx.doi.org/10.1111/jce.14594DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7300464PMC

Fatal arrhythmias: Another reason why doctors remain cautious about chloroquine/hydroxychloroquine for treating COVID-19.

Heart Rhythm 2020 May 29. Epub 2020 May 29.

School of Physics, Georgia Institute of Technology, Atlanta, Georgia. Electronic address:

Background: Early during the current coronavirus disease 19 (COVID-19) pandemic, hydroxychloroquine (HCQ) received a significant amount of attention as a potential antiviral treatment, such that it became one of the most commonly prescribed medications for COVID-19 patients. However, not only has the effectiveness of HCQ remained questionable, but mainly based on preclinical and a few small clinical studies, HCQ is known to be potentially arrhythmogenic, especially as a result of QT prolongation.

Objective: The purpose of this study was to investigate the arrhythmic effects of HCQ, as the heightened risk is especially relevant to COVID-19 patients, who are at higher risk for cardiac complications and arrhythmias at baseline. Read More

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http://dx.doi.org/10.1016/j.hrthm.2020.05.030DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7256542PMC

Tpeak-Tend, Tpeak-Tend dispersion and Tpeak-Tend/QT in children and its relationship with clinical variables.

Colomb Med (Cali) 2019 Dec 30;50(4):252-260. Epub 2019 Dec 30.

Universidad Central Marta Abreu de las Villas, Facultad de Ingeniería Eléctrica, Centro de Desarrollo Electrónico. Santa Clara, Villa Clara. Cuba.

Aim: To characterize the Tpeak-Tend, the Tpeak-Tend dispersion and Tpeak-Tend/QT in children and its relationship with clinical variables.

Methods: Cross-sectional study in 126 children between 9 and 12 years of the Camilo Cienfuegos School in Santa Clara, Cuba. Clinical and anthropometric variables were obtained to determine their relationship with electrocardiographic parameters: Tpeak-Tend V5, Tpeak-Tend dispersion and Tpeak-Tend/QT ratio V5. Read More

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http://dx.doi.org/10.25100/cm.v50i4.4199DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7232943PMC
December 2019

A rapid solubility assay of protein domain misfolding for pathogenicity assessment of rare DNA sequence variants.

Genet Med 2020 Jun 1. Epub 2020 Jun 1.

Department of Medicine, University of Wisconsin-Madison, Madison, WI, USA.

Purpose: DNA sequencing technology has unmasked a vast number of uncharacterized single-nucleotide variants in disease-associated genes, and efficient methods are needed to determine pathogenicity and enable clinical care.

Methods: We report an E. coli-based solubility assay for assessing the effects of variants on protein domain stability for three disease-associated proteins. Read More

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http://dx.doi.org/10.1038/s41436-020-0842-1DOI Listing

Silencing of CCR4-NOT complex subunits affect heart structure and function.

Dis Model Mech 2020 May 29. Epub 2020 May 29.

Development Aging and Regeneration Program, Sanford Burnham Prebys Medical Discovery Institute, 10901 N Torrey Pines Rd, La Jolla, CA 92037, USA

Genome wide association studies (GWAS) have identified variants that associate with QT-interval length. Three of the strongest associating variants (SNPs) are located in the putative promotor region of , a gene encoding the central subunit of CCR4-NOT, a multi-functional, conserved complex regulating gene expression and mRNA stability and turnover. We isolated the minimum fragment of the promoter containing all three variants from individuals homozygous for the QT-risk alleles and demonstrated that the haplotype associating with longer QT-interval caused reduced reporter expression in a cardiac cell line, suggesting that reduced expression may contribute to abnormal QT-intervals. Read More

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http://dx.doi.org/10.1242/dmm.044727DOI Listing

Genetic homozygosity in a diverse population: An experience of long QT syndrome.

Int J Cardiol 2020 May 26. Epub 2020 May 26.

Rajaie Cardiovascular Medical and Research Center, Iran University of Medical Sciences, Tehran, Iran; Growth and development research center, Tehran University of Medical Sciences, Tehran, Iran. Electronic address:

Background: Genomic variations have shown an ethnic-specific pattern within various cohorts. Genetic variants of KCNQ1, KCNH2, SCN5A and KCNE1 causing LQT syndrome have been described in many populations. In this article the spectrum of variants of these genes is presented in Iranian patients. Read More

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http://dx.doi.org/10.1016/j.ijcard.2020.05.056DOI Listing
May 2020
4.036 Impact Factor

The QTc-Polygenic Risk Score (QTc-PRS) and its Contribution to Type 1, Type 2, and Type 3 Long QT Syndrome in Probands and Genotype-Positive Family Members.

Circ Genom Precis Med 2020 May 29. Epub 2020 May 29.

Mayo Clinic Graduate School of Biomedical Sciences, Department of Molecular Pharmacology & Experimental Therapeutics, Windland Smith Rice Sudden Death Genomics Laboratory, Department of Cardiovascular Medicine/Division of Heart Rhythm Services, Mayo Clinic Alix School of Medicine, Department of Pediatrics/Division of Pediatric Cardiology, Mayo Clinic, Rochester, MN.

- Long QT syndrome (LQTS) is characterized by a prolonged heart rate corrected QT interval (QTc). Genome-wide association studies identified common genetic variants that collectively explain ~8-10% of QTc variation in the general population. - Overall, 423 patients with type 1-3 LQTS were genotyped for 61 QTc-associated genetic variants used in a prototype QTc-polygenic risk score (QTc-PRS). Read More

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http://dx.doi.org/10.1161/CIRCGEN.120.002922DOI Listing

Cardiac Dysrhythmia Associated With Opioid Toxicity.

Cureus 2020 May 22;12(5):e8243. Epub 2020 May 22.

Cardiology, Rapides Regional Medical Center, Alexandria, USA.

Irritable bowel syndrome (IBS) is a functional gastrointestinal disorder involving abnormal motility. The patients are commonly started on symptom control management for IBS - diarrhea subtype by prescribing antimotility agents, usually having opioid agonist activity, and newer medications have been emerging for this as well. Patients sometimes self-medicate themselves to exceedingly high doses of these medications to achieve symptoms control. Read More

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http://dx.doi.org/10.7759/cureus.8243DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7249760PMC

What Is the Risk of Sudden Cardiac Arrest in Inherited Cardiac Conditions?

J Am Coll Cardiol 2020 Jun;75(21):2708-2710

National Heart and Lung Institute and MRC London Institute of Medical Sciences, Imperial College London, London, United Kingdom; Cardiovascular Research Centre, Royal Brompton and Harefield NHS Foundation Trust, London, United Kingdom.

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http://dx.doi.org/10.1016/j.jacc.2020.04.010DOI Listing

A Population-Based Registry of Patients With Inherited Cardiac Conditions and Resuscitated Cardiac Arrest.

J Am Coll Cardiol 2020 Jun;75(21):2698-2707

Greenlane Paediatric and Congenital Cardiac Services, Starship Children's Hospital, Auckland, New Zealand; Cardiac Inherited Disease Group, Auckland, New Zealand; Department of Paediatrics Child and Youth Health, University of Auckland, Auckland, New Zealand. Electronic address:

Background: The relative proportion of each cardiac inherited disease (CID) causing resuscitated sudden cardiac arrest (RSCA) on a population basis is unknown.

Objectives: This study describes the profile of patients with CIDs presenting with RSCA; their data were collected by the national Cardiac Inherited Diseases Registry New Zealand (CIDRNZ).

Methods: Data were collated from CIDRNZ probands presenting with RSCA (2002 to 2018). Read More

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http://dx.doi.org/10.1016/j.jacc.2020.04.004DOI Listing

Chloroquine or Hydroxychloroquine for COVID-19: Is Cardiotoxicity a Concern?

J Am Heart Assoc 2020 06 28;9(12):e016887. Epub 2020 May 28.

Division of Cardiovascular Medicine Department of Medicine University of Wisconsin-Madison Madison WI.

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http://dx.doi.org/10.1161/JAHA.120.016887DOI Listing

Role of cardiac sympathetic denervation in ventricular tachycardia: A meta-analysis.

Pacing Clin Electrophysiol 2020 May 27. Epub 2020 May 27.

Kansas City Heart Rhythm Institute & Research Foundation, Overland Park, Kansas.

Background: Cardiac sympathetic denervation (CSD) is being used in the management of refractory ventricular tachycardia (VT) and electrical storm. However, data on the role of CSD in the management of ventricular arrhythmia is limited.

Methods: We performed a meta-analysis of retrospective studies to calculate the pooled rate of freedom from VT and the standard mean difference of ICD shocks before and after CSD. Read More

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http://dx.doi.org/10.1111/pace.13968DOI Listing
May 2020
1.250 Impact Factor

A Unique Interplay of Multiple Predisposing Factors Culminating in a Catastrophic QT Prolongation.

Cureus 2020 Apr 21;12(4):e7757. Epub 2020 Apr 21.

Cardiology, Electrophysiology, State University of New York (SUNY) Upstate Medical University, Syracuse, USA.

Implantable cardioverter-defibrillators (ICDs) are used in patients without a reversible cause for long QT syndrome (LQTS) and secondary prevention in patients with LQTS-associated sudden cardiac arrest. We present a female patient with multiple reversible factors for QT prolongation, including the use of antidepressants, antidiarrheals, antiemetics, and antihistamines; chronic malabsorption from bariatric surgery; probable Gitelman syndrome and urinary losses of electrolytes, causing QT prolongation which leads to polymorphic ventricular tachycardia and a successfully resuscitated cardiac arrest. Our patient also had history suggestive of probable congenital LQTS with multiple childhood syncopal episodes and a history of seizures in first-degree relatives, further justifying the placement of an ICD. Read More

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http://dx.doi.org/10.7759/cureus.7757DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7243073PMC

Purkinje system hyperexcitability and ventricular arrhythmia risk in type 3 long QT syndrome.

Heart Rhythm 2020 May 23. Epub 2020 May 23.

Division of Heart Rhythm Services, Department of Cardiovascular Medicine, Mayo Clinic, Rochester, Minnesota; Windland Smith Rice Sudden Death Genomics Laboratory, Department of Molecular Pharmacology and Experimental Therapeutics, Mayo Clinic, Rochester, Minnesota; Division of Pediatric Cardiology, Department of Pediatric and Adolescent Medicine, Mayo Clinic, Rochester, Minnesota. Electronic address:

Background: Gain-of-function variants in the SCN5A-encoded Na1.5 sodium channel cause type 3 long QT syndrome (LQT3) and multifocal ectopic Purkinje-related premature contractions. Although the Purkinje system is uniquely sensitive to the action potential-prolonging effects of LQT3-causative variants, the existence of additional Purkinje phenotype(s) in LQT3 is unknown. Read More

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http://dx.doi.org/10.1016/j.hrthm.2020.05.019DOI Listing

Dynamic QT Response to Cold-Water Face Immersion in Long-QT Syndrome Type 3.

Pediatr Int 2020 May 25. Epub 2020 May 25.

Department of Pediatric Cardiology, Okinawa Nanbu and Children's Medical Center, Okinawa, Japan.

Background: Abnormal dynamics of QT intervals in response to sympathetic nervous system stimulation is used to diagnose long-QT syndrome (LQTS). We hypothesized that parasympathetic stimulation with cold-water face immersion following exercise influences QT dynamics in patients with LQTS type 3 (LQT3).

Method: Study participants (n=42; mean age=11. Read More

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http://dx.doi.org/10.1111/ped.14319DOI Listing

Head-up tilt test induces T-wave alternans in long QT syndrome with KCNQ1 gene mutation: Case report CARE-compliant article.

Medicine (Baltimore) 2020 May;99(20):e19818

Department of Cardiology, Shanghai Children's Medical Center, Shanghai Jiaotong University School of Medicine, Shanghai, China.

Introduction: Long QT syndrome (LQTS) is a congenital disorder characterized by a prolongation of the QT interval on electrocardiograms (ECGs) and a propensity to ventricular tachyarrhythmias, which may lead to syncope, cardiac arrest, or sudden death. T-wave alternans (TWA) refers to the periodic beat-to-beat alternation of T-wave shape, polarity and amplitude on surface ECG during regular heart rhythm. In this report, a case of long QT syndrome with KCNQ1 gene mutation induced TWA in the head-up tilt test (HUTT), which has not been reported yet. Read More

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http://dx.doi.org/10.1097/MD.0000000000019818DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7253722PMC

[Hydroxychloroquine. Cardiology's viewpoint in times of coronavirus pandemic].

Medicina (B Aires) 2020 ;80(3):271-274

Grupo de Trabajo COVID-19 de la Sociedad Interamericana de Cardiología.

Due to the coronavirus disease 2019 (COVID-19) pandemic, a wide number of compounds are under scrutiny regarding their antiviral activity, one of them being hydroxychloroquine. Cardiac aspects of the use of chloroquine and hydroxychloroquine are reviewed in this manuscript. A non-systematic review of the medical literature was performed. Read More

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The Use of "Novel Pharmacology" in the Treatment of COVID-19 and Potential Psychiatric Risks.

Prim Care Companion CNS Disord 2020 05 21;22(3). Epub 2020 May 21.

Waltham Forest Mental Health Team, North East London Foundation Trust, Red Oak Lodge, London, England E11 4HU.

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http://dx.doi.org/10.4088/PCC.20com02638DOI Listing

Effects of amiodarone on rodent ventricular cardiomyocytes: Novel perspectives from a cellular model of Long QT Syndrome Type 3.

Life Sci 2020 Aug 19;255:117814. Epub 2020 May 19.

Laboratory of CardioBiology, Department of Biophysics, Paulista School of Medicina, Federal University of Sao Paulo, Brazil. Electronic address:

Aims: Amiodarone (AMIO) is currently used in medical practice to reverse ventricular tachycardia. Here we determine the effects of AMIO in the electromechanical properties of isolated left ventricle myocyte (LVM) from mice and guinea pig and in a cellular model of Long QT Syndrome Type 3 (LQTS-3) using anemone neurotoxin 2 (ATX II), which induces increase of late sodium current in LVM.

Main Methods And Key Findings: Using patch-clamp technique, fluorescence imaging to detect cellular Ca transient and sarcomere detection systems we evaluate the effect of AMIO in healthy LVM. Read More

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http://dx.doi.org/10.1016/j.lfs.2020.117814DOI Listing

A cardiac sodium channel mutation associated with epinephrine-induced marked QT-prolongation.

J Cardiovasc Electrophysiol 2020 May 21. Epub 2020 May 21.

Division of Cardiology, Department of Internal Medicine, American University of Beirut Medical Center, Beirut, Lebanon.

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http://dx.doi.org/10.1111/jce.14572DOI Listing

Regulation of cardiovascular calcium channel activity by post-translational modifications or interacting proteins.

Pflugers Arch 2020 Jun 20;472(6):653-667. Epub 2020 May 20.

Department of Physiology, Yong Loo Lin School of Medicine, National University of Singapore, Singapore, 117593, Singapore.

Voltage-gated calcium channels are the major pathway for Ca influx to initiate the contraction of smooth and cardiac muscles. Alterations of calcium channel function have been implicated in multiple cardiovascular diseases, such as hypertension, atrial fibrillation, and long QT syndrome. Post-translational modifications do expand cardiovascular calcium channel structure and function to affect processes such as channel trafficking or polyubiquitination by two E3 ubiquitin ligases, Ret finger protein 2 (Rfp2) or murine double minute 2 protein (Mdm2). Read More

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http://dx.doi.org/10.1007/s00424-020-02398-xDOI Listing