Full Text Journal Articles about
Pediatric Long QT Syndrome

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A Review of Long QT Syndrome: Everything a Hospitalist Should Know.

Nandita Sharma, Daniel Cortez, Kristin Disori, Jason R Imundo, Michael Beck,

In this article, we will review various aspects of long QT syndrome (LQTS) necessary for hospitalists who care for children, adolescents, and young adults who have known LQTS and also review presenting features that should make one consider LQTS as a cause of hospitalization. Pediatric hospitalists care for patients who ... Read more >>

Hosp Pediatr (Hospital pediatrics)
[2020, :]

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QTc interval-dependent body posture in pediatrics.

Björn Reynisson, Gustaf Tanghöj, Estelle Naumburg,

BACKGROUND:Syncope is a common and often benign disorder presenting at the pediatric emergency department. Long-QT syndrome may be presented with syncope, ventricular arrhythmias or sudden death and is vital to exclude as an underlying cause in children presented with syncope. Few studies have assessed QTc in relation to body posture ... Read more >>

BMC Pediatr (BMC pediatrics)
[2020, 20(1):107]

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Intentional nontherapy in long QT syndrome.

Ciorsti J MacIntyre, Ram K Rohatgi, Alan M Sugrue, J Martijn Bos, Michael J Ackerman,

BACKGROUND:International guidelines advise universal beta-blocker therapy as either a class I (symptomatic or QTc ≥470 ms) or class II (asymptomatic and QTc <470 ms) recommendation for treatment of long QT syndrome (LQTS). OBJECTIVE:The purpose of this study was to evaluate the outcomes of a highly selected cohort of patients with ... Read more >>

Heart Rhythm (Heart rhythm)
[2020, :]

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Fetal diagnosis of KCNQ1-variant long QT syndrome using fetal echocardiography and magnetocardiography.

Lajja Desai, Ron Wakai, Sabrina Tsao, Janette Strasburger, Nina Gotteiner, Angira Patel,

A pregnant woman with KCNQ1 variant long QT syndrome (LQTS) underwent fetal magnetocardiography (fMCG) after atrioventricular (AV) block was noted during fetal echocardiogram - atypical for LQTS type 1. Concern for fetal LQTS on fMCG prompted monitoring of maternal labs, change of maternal beta blocker therapy, and frequent fetal echocardiograms. ... Read more >>

Pacing Clin Electrophysiol (Pacing and clinical electrophysiology : PACE)
[2020, :]

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Clinical and functional reappraisal of alleged type 5 long QT syndrome: Causative genetic variants in the KCNE1-encoded minK β-subunit.

Ramin Garmany, John R Giudicessi, Dan Ye, Wei Zhou, David J Tester, Michael J Ackerman,

BACKGROUND:KCNE1 loss-of-function variants cause type 5 long QT syndrome (LQT5). However, most alleged LQT5-causative KCNE1 variants were identified before the true rate of background genetic variation was appreciated fully. OBJECTIVE:The purpose of this study was to reassess the clinical and electrophysiological (EP) phenotypes associated with KCNE1 variants detected in a ... Read more >>

Heart Rhythm (Heart rhythm)
[2020, :]

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Variant Frequency and Clinical Phenotype Call Into Question the Nature of Minor, Nonsyndromic Long-QT Syndrome-Susceptibility Gene-Disease Associations.

John R Giudicessi, Ram K Rohatgi, David J Tester, Michael J Ackerman,

Circulation (Circulation)
[2020, 141(6):495-497]

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Novel variants in TECRL cause recessive inherited CPVT type 3 with severe and variable clinical symptoms.

Alexander Moscu-Gregor, Christoph Marschall, Carsten Müntjes, Anne Schönecker, Franziska Schuessler-Hahn, Felix Hohendanner, Abdul Shokor Parwani, Leif-Hendrik Boldt, Claus-Eric Ott, Anja Bennewiz, Thomas Paul, Ulrich Krause, Imma Rost,

INTRODUCTION:Catecholaminergic polymorphic ventricular tachycardia (CPVT) is an inherited arrhythmia syndrome characterized by adrenergically stimulated ventricular tachycardia. The most common form of CPVT is due to autosomal dominant variants in the cardiac ryanodine-receptor gene (RYR2). However, trans-2,3-enoyl-CoA reductase-like (TECRL) was recently suggested to be a novel candidate gene for life-threatening inherited ... Read more >>

J. Cardiovasc. Electrophysiol. (Journal of cardiovascular electrophysiology)
[2020, :]

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An International Multicenter Evaluation of Type 5 Long QT Syndrome: A Low Penetrant Primary Arrhythmic Condition.

Jason D Roberts, S Yukiko Asaki, Andrea Mazzanti, J Martijn Bos, Izabela Tuleta, Alison R Muir, Lia Crotti, Andrew D Krahn, Valentina Kutyifa, M Benjamin Shoemaker, Christopher L Johnsrude, Takeshi Aiba, Luciana Marcondes, Anwar Baban, Sharmila Udupa, Brynn Dechert, Peter Fischbach, Linda M Knight, Eric Vittinghoff, Deni Kukavica, Birgit Stallmeyer, John R Giudicessi, Carla Spazzolini, Keiko Shimamoto, Rafik Tadros, Julia Cadrin-Tourigny, Henry J Duff, Christopher S Simpson, Thomas M Roston, Yanushi D Wijeyeratne, Imane El Hajjaji, Maisoon D Yousif, Lorne J Gula, Peter Leong-Sit, Nikhil Chavali, Andrew P Landstrom, Gregory M Marcus, Sven Dittmann, Arthur A M Wilde, Elijah R Behr, Jacob Tfelt-Hansen, Melvin M Scheinman, Marco V Perez, Juan Pablo Kaski, Robert M Gow, Fabrizio Drago, Peter F Aziz, Dominic J Abrams, Michael H Gollob, Jonathan R Skinner, Wataru Shimizu, Elizabeth S Kaufman, Dan M Roden, Wojciech Zareba, Peter J Schwartz, Eric Schulze-Bahr, Susan P Etheridge, Silvia G Priori, Michael J Ackerman,

BACKGROUND:Insight into type 5 long QT syndrome (LQT5) has been limited to case reports and small family series. Improved understanding of the clinical phenotype and genetic features associated with rare KCNE1 variants implicated in LQT5 was sought through an international multicenter collaboration. METHODS:Patients with either presumed autosomal dominant LQT5 (N ... Read more >>

Circulation (Circulation)
[2020, 141(6):429-439]

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SIDS associated RYR2 p.Arg2267His variant may lack pathogenicity.

Utkarsh Kohli, Hemal M Nayak,

Sudden infant death syndrome (SIDS) is the sudden death of an infant under 1 year of age that remains unexplained after death scene and medicolegal investigation, including a complete autopsy and clinical history review. The fatal event typically occurs during sleep and heart rhythm during the event is rarely documented. Large ... Read more >>

J Electrocardiol (Journal of electrocardiology)
[2020, 60:23-26]

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Ventricular Tachycardia in an Infant Without Congenital Anomaly: A Case Report.

Nouhad El Joueid, Marianne Touma Boulos, Simon Abou Jaoude, Linda Daou,

Ventricular tachycardia (VT) is a serious form of arrhythmia that can be life-threatening; that's why diagnosis and treatment are very important in order to avoid serious complications. We are reporting this case of VT which is a rare entity, especially, in healthy infants. This infant, without cardiac pathology known from ... Read more >>

Cardiol Res (Cardiology research)
[2020, 11(1):61-65]

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The effects of ondansetron versus dexamethasone on electrocardiographic markers of ventricular repolarization in children undergoing cochlear implant.

Reza Safaeian, Valiollah Hassani, Alimohamad Asghari, Masood Mohseni, Haleh Ashraf, Zahra Sadat Koleini,

INTRODUCTION:Congenital hearing loss is associated with cardiac rhythm disturbances namely long Q-T syndrome. This study was designed to investigate the effect of anti-emetic doses of ondansetron and dexamethasone on ECG recordings in children undergoing cochlear implant surgery. METHODS:Sixty-three pediatric patients scheduled for elective cochlear implantation were enrolled in the study. ... Read more >>

Int. J. Pediatr. Otorhinolaryngol. (International journal of pediatric otorhinolaryngology)
[2020, 132:109896]

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Increased QT Interval Dispersion is Associated with Coronary Artery Involvement in Children with 
Kawasaki Disease.

Ehsan Aghaei Moghadam, Leila Hamzehlou, Bobak Moazzami, Mina Mehri, Vahid Ziaee,

Objectives:Coronary artery (CA) involvement is the most well known complication of Kawasaki disease (KD). Previous studies have suggested that QT dispersion has a predictive value in diagnosing cardiac ischemia, ventricular arrhythmia, and sudden cardiac death. However, limited data exits regarding the application of QT dispersion in KD. Therefore, we sought ... Read more >>

Oman Med J (Oman medical journal)
[2020, 35(1):e88]

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Initially unexplained cardiac arrest in children and adolescents: A national experience from the Canadian Pediatric Heart Rhythm Network.

Taylor Cunningham, Thomas M Roston, Sonia Franciosi, Michelle Chang Liu, Joseph Atallah, Carolina A Escudero, Sharmila Udupa, Jason D Roberts, Santokh Dhillon, Frédéric Dallaire, Anne Fournier, Meena Fatah, Robert Hamilton, Shubhayan Sanatani,

BACKGROUND:Unexplained cardiac arrest (UCA) is rare in children. Despite investigations, the etiology in up to one-half of patients remains unknown. OBJECTIVE:The purpose of this study was to assess the management and outcomes of pediatric UCA survivors through the Canadian Pediatric Heart Rhythm Network. METHODS:A retrospective case series of children (age ... Read more >>

Heart Rhythm (Heart rhythm)
[2020, :]

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An International, Multicentered, Evidence-Based Reappraisal of Genes Reported to Cause Congenital Long QT Syndrome.

Arnon Adler, Valeria Novelli, Ahmad S Amin, Emanuela Abiusi, Melanie Care, Eline A Nannenberg, Harriet Feilotter, Simona Amenta, Daniela Mazza, Hennie Bikker, Amy C Sturm, John Garcia, Michael J Ackerman, Raymond E Hershberger, Marco V Perez, Wojciech Zareba, James S Ware, Arthur A M Wilde, Michael H Gollob,

BACKGROUND:Long QT syndrome (LQTS) is the first described and most common inherited arrhythmia. Over the last 25 years, multiple genes have been reported to cause this condition and are routinely tested in patients. Because of dramatic changes in our understanding of human genetic variation, reappraisal of reported genetic causes for ... Read more >>

Circulation (Circulation)
[2020, 141(6):418-428]

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Cardiac phenotype in propionic acidemia - Results of an observational monocentric study.

A Kovacevic, S F Garbade, G F Hoffmann, M Gorenflo, S Kölker, C Staufner,

BACKGROUND:Propionic acidemia (PA) is an organic aciduria caused by inherited deficiency of propionyl-CoA carboxylase. Left ventricular dysfunction and QT prolongation may lead to life-threatening complications. Systematic analyses of cardiac phenotypes, in particular effects of specific cardiac therapies, are scarce. METHODS:In this longitudinal observational monocentric study (data from 1989 to 2017) ... Read more >>

Mol. Genet. Metab. (Molecular genetics and metabolism)
[2020, :]

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Improving long QT syndrome diagnosis by a polynomial-based T-wave morphology characterization.

Ben J M Hermans, Frank C Bennis, Arja S Vink, Tijmen Koopsen, Aurore Lyon, Arthur A M Wilde, Dieter Nuyens, Tomas Robyns, Laurent Pison, Pieter G Postema, Tammo Delhaas,

BACKGROUND:Diagnosing long QT syndrome (LQTS) remains challenging because of a considerable overlap in QT interval between patients with LQTS and healthy subjects. Characterizing T-wave morphology might improve LQTS diagnosis. OBJECTIVE:The purpose of this study was to improve LQTS diagnosis by combining new polynomial-based T-wave morphology parameters with the corrected QT ... Read more >>

Heart Rhythm (Heart rhythm)
[2020, :]

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Abnormal myocardial expression of SAP97 is associated with arrhythmogenic risk.

Hassan Musa, Cherisse A Marcou, Todd J Herron, Michael Makara, David J Tester, Ryan O'Connell, Brad Rosinski, Guadalupe Guerrero-Serna, Michelle L Milstein, Andre Monteiro Da Rocha, Dan Ye, Lia Crotti, Vladislav V Nesterenko, Silvia Castelletti, Margherita Torchio, Maria-Christina Kotta, Federica Dagradi, Charles Antzelevitch, Peter J Mohler, Peter J Schwartz, Michael J Ackerman, Justus M B Anumonwo,

Background Synapse-associated protein 97 (SAP97) is a scaffolding protein crucial for the functional expression of several cardiac ion channels and therefore proper cardiac excitability. Alterations in the functional expression of SAP97 can modify the ionic currents underlying the cardiac action potential and consequently confer susceptibility for arrhythmogenesis. In this study, ... Read more >>

Am. J. Physiol. Heart Circ. Physiol. (American journal of physiology. Heart and circulatory physiology)
[2020, :]

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Mothers with long QT syndrome are at increased risk for fetal death: findings from a multicenter international study.

Bettina F Cuneo, Alexander M Kaizer, Sally Ann Clur, Heikki Swan, Ulrike Herberg, Annika Winbo, Annika Rydberg, Kristina Haugaa, Susan Etheridge, Michael J Ackerman, Federica Dagradi, Stacy A S Killen, Annette Wacker-Gussmann, D Woodrow Benson, A A M Wilde, Zhaoxing Pan, Aimee Lam, Carla Spazzolini, Hitoshi Horigome, Peter J Schwartz, ,

BACKGROUND:Most fetal deaths are unexplained. Long QT syndrome is a genetic disorder of cardiac ion channels. Affected individuals, including fetuses, are predisposed to sudden death. We sought to determine the risk of fetal death in familial long QT syndrome, in which the mother or father carries the long QT syndrome ... Read more >>

Am. J. Obstet. Gynecol. (American journal of obstetrics and gynecology)
[2020, 222(3):263.e1-263.e11]

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Thoracoscopic Left Cardiac Sympathetic Denervation in a Child with Refractory Long QT Syndrome.

Sachit Anand, Vishesh Jain, Sandeep Agarwala, Sakshi Sachdeva, Shyam Sunder Kothari,

Long QT syndrome is a cardiac disorder which presents with recurrent syncopal attacks and has risk of sudden cardiac death. A 5-year-old boy presented to us with this syndrome. The child was symptomatic despite medical management and was successfully managed with cardiac denervation. The current report highlights the efficacy and ... Read more >>

J Indian Assoc Pediatr Surg (Journal of Indian Association of Pediatric Surgeons)
[2019, 24(4):297-299]

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Stellate Ganglion Catheter Effective for Treatment of Ventricular Tachycardia Storm in a Pediatric Patient on Extracorporeal Membrane Oxygenation: A Case Report.

Andrew D Franklin, J Rodrigo Llobet, Christopher M Sobey, Jessica M Daniels, Prince J Kannankeril,

Ventricular tachycardia (VT) storm, defined as recurrent VT requiring electrical cardioversion ≥3 times within 24 hours, is a rare presentation of long-QT syndrome. Pharmacologic autonomic modulation and/or left cardiac sympathetic denervation are established therapies in long-QT syndrome in adults but may not be effective or practical in the emergent treatment ... Read more >>

A A Pract (A&A practice)
[2019, 13(7):245-249]

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Editorial to "Association of QT dispersion with mortality and arrhythmic events-A meta-analysis of observational studies".

Cem Karadeniz,

J Arrhythm (Journal of arrhythmia)
[2020, 36(1):116-117]

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The ketogenic diet and the QT interval.

Sivakumar Sudhakaran, Laila Yazdani, Kevin R Wheelan, Praveen K Rao,

Long QT syndrome is a disorder of ventricular myocardial repolarization associated with an increased risk of life-threatening cardiac arrhythmias and sudden cardiac death. This report highlights a case of QT prolongation with torsades de pointes in a patient with baseline congenital long QT syndrome, believed to be precipitated by metabolic ... Read more >>

Proc (Bayl Univ Med Cent) (Proceedings (Baylor University. Medical Center))
[2020, 33(1):77-79]

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Type 8 long QT syndrome: pathogenic variants in CACNA1C-encoded Cav1.2 cluster in STAC protein binding site.

Greg J Mellor, Pankaj Panwar, Andrea K Lee, Christian Steinberg, Julie A Hathaway, Kirsten Bartels, Susan Christian, Seshadri Balaji, Jason D Roberts, Chris S Simpson, Nicole J Boczek, David J Tester, Andrew E Radbill, Ngai-Shing Mok, Robert M Hamilton, Elizabeth S Kaufman, Paul L Eugenio, Raul Weiss, Craig January, George M McDaniel, Richard A Leather, Christopher Erickson, Shelley Falik, Elijah R Behr, Arthur A M Wilde, Shubhayan Sanatani, Michael J Ackerman, Filip Van Petegem, Andrew D Krahn, Zachary Laksman,

AIMS:Pathogenic gain-of-function variants in CACAN1C cause type-8 long QT syndrome (LQT8). We sought to describe the electrocardiographic features in LQT8 and utilize molecular modelling to gain mechanistic insights into its genetic culprits. METHODS AND RESULTS:Rare variants in CACNA1C were identified from genetic testing laboratories. Treating physicians provided clinical information. Variant ... Read more >>

Europace (Europace : European pacing, arrhythmias, and cardiac electrophysiology : journal of the working groups on cardiac pacing, arrhythmias, and cardiac cellular electrophysiology of the European Society of Cardiology)
[2019, 21(11):1725-1732]

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The Effect of Left Cardiac Sympathetic Denervation on Exercise in Patients With Long QT Syndrome.

Heather N Anderson, J Martijn Bos, Ram K Rohatgi, Michael J Ackerman,

OBJECTIVES:This study evaluated the effect of left cardiac sympathetic denervation (LCSD) on heart rate, cardiac contractility, and cardiopulmonary fitness in human subjects. BACKGROUND:The primary treatment for long QT syndrome (LQTS) is beta-blocker (BB) therapy, but some patients experience breakthrough cardiac events or intolerable side effects. LCSD provides a significant antifibrillatory, ... Read more >>

JACC Clin Electrophysiol (JACC. Clinical electrophysiology)
[2019, 5(9):1084-1090]

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QT Prolongation After Minor Head Trauma in a Pediatric Patient.

Lamya Mubayed, Angela Romme, Hoang H Nguyen,

We report a case of QTc prolongation associated with mild concussion in a pediatric patient. An 11-year-old male presented to the emergency department after sustaining a head injury during football practice. He complained of headache and blurry vision. Physical examination was within normal apart from an irregular heart rhythm. Electrocardiogram ... Read more >>

Pediatr Cardiol (Pediatric cardiology)
[2020, 41(2):414-417]

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