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Pediatric electrophysiology

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https://www.readbyqxmd.com/read/28213194/subcortical-mapping-using-an-electrified-cusa-in-pediatric-supratentorial-surgery
#1
Jonathan Roth, Akiva Korn, Yifat Bitan-Talmor, Rivka Kaufman, Margaret Ekstein, Shlomi Constantini
BACKGROUND: Intraoperative electrophysiology is increasingly used for various lesion resections, both in adult and pediatric brain surgery. Subcortical mapping is often used in adult surgery when lesions lie in proximity to the corticospinal tract (CST). We describe a novel technique of continuous subcortical mapping using an electrified Cavitron UltraSonic Aspirator (CUSA) in children with supratentorial lesions. METHODS: We evaluated the method of subcortical mapping using a CUSA as a stimulation probe...
February 14, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/28191656/utility-and-safety-of-combined-interventional-catheterization-and-electrophysiology-procedures-in-a-children-s-hospital
#2
S Yukiko Asaki, Jeffrey W Orcutt, Christina Y Miyake, Henri Justino, Caridad M DE LA Uz, Jeffrey J Kim, Santiago O Valdes, Athar M Qureshi
BACKGROUND: Interventional cardiac catheterization (cath) and electrophysiology (EP) procedures are not routinely performed together. There are several perceived barriers affecting this practice, though there are also advantages for both the patient and practitioner to a combined approach. METHODS: This was a single center retrospective study reviewing combined cath and EP procedures with a pre-procedural intention to intervene at Texas Children's Hospital from 2001 to 2014...
February 13, 2017: Pacing and Clinical Electrophysiology: PACE
https://www.readbyqxmd.com/read/28148335/a-pediatric-cardiology-fellowship-boot-camp-improves-trainee-confidence
#3
Catherine K Allan, Paul Tannous, Elizabeth DeWitt, Michael Farias, Laura Mansfield, Christina Ronai, David Schidlow, Stephen P Sanders, James E Lock, Jane W Newburger, David W Brown
: Introduction New paediatric cardiology trainees are required to rapidly assimilate knowledge and gain clinical skills to which they have limited or no exposure during residency. The Pediatric Cardiology Fellowship Boot Camp (PCBC) at Boston Children's Hospital was designed to provide incoming fellows with an intensive exposure to congenital cardiac pathology and a broad overview of major areas of paediatric cardiology practice. METHODS: The PCBC curriculum was designed by core faculty in cardiac pathology, echocardiography, electrophysiology, interventional cardiology, exercise physiology, and cardiac intensive care...
December 2016: Cardiology in the Young
https://www.readbyqxmd.com/read/28108086/long-term-risk-of-wolff-parkinson-white-pattern-and-syndrome
#4
REVIEW
Susan S Kim, Bradley P Knight
For years, conventional wisdom has held that patients with asymptomatic ventricular pre-excitation (asymptomatic WPW or WPW pattern) were at low risk for adverse outcomes. This assumption has been challenged more recently in a number of observational/natural history studies as well as in prospective trials in which patients were more aggressively studied via invasive electrophysiology study (EPS) and more aggressively treated, in some cases, with pre-emptive catheter ablation, despite the lack of symptoms. In sum, the data do not definitively support one approach (early, up-stream EPS and/or ablation) vs...
December 5, 2016: Trends in Cardiovascular Medicine
https://www.readbyqxmd.com/read/28091832/utility-and-safety-of-the-safesept%C3%A2-transseptal-guidewire-for-electrophysiology-studies-with-catheter-ablation-in-pediatric-and-congenital-heart-disease
#5
Joseph J Knadler, Jeffrey B Anderson, Ahmad S Chaouki, Richard J Czosek, Chad Connor, Timothy K Knilans, David S Spar
PURPOSE: The atrial transseptal procedure is used in catheter ablation of left-sided arrhythmias. Studies in adult patients have shown the SafeSept™ transseptal guidewire (SSTG) to be effective in atrial transseptal procedures. We analyzed our 5-year experience with SSTG use in pediatric and congenital heart disease patients undergoing catheter ablation. METHODS: This is a single-center retrospective analysis of patients undergoing catheter ablation from 2009 to 2014...
January 14, 2017: Journal of Interventional Cardiac Electrophysiology: An International Journal of Arrhythmias and Pacing
https://www.readbyqxmd.com/read/28011839/patient-specific-modeling-of-left-ventricular-electromechanics-as-a-driver-for-haemodynamic-analysis
#6
Christoph M Augustin, Andrew Crozier, Aurel Neic, Anton J Prassl, Elias Karabelas, Tiago Ferreira da Silva, Joao F Fernandes, Fernando Campos, Titus Kuehne, Gernot Plank
AIMS: Models of blood flow in the left ventricle (LV) and aorta are an important tool for analysing the interplay between LV deformation and flow patterns. Typically, image-based kinematic models describing endocardial motion are used as an input to blood flow simulations. While such models are suitable for analysing the hemodynamic status quo, they are limited in predicting the response to interventions that alter afterload conditions. Mechano-fluidic models using biophysically detailed electromechanical (EM) models have the potential to overcome this limitation, but are more costly to build and compute...
December 2016: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
https://www.readbyqxmd.com/read/27903347/-clinical-genetic-and-pathological-features-of-neuronal-ceroid-lipofuscinosis-in-5-chinese-patients
#7
S C Ren, B Q Gao, Y J Wang, X J Wu, Z X Tian, Y L Sun
Objective: To report the clinical, genetic and ultrastructural pathological features of neuronal ceroid lipofuscinosis (NCLs) in 5 Chinese patients. Methods: A total of 5 patients with NCLs were collected from 2013 to 2015 diagnosed by the department of pediatrics of Beijing Tian Tan Hospital. Their clinical, electrophysiological and neuroimaging data of the patients were reviewed.A total of 9 underlying genes of NCLs were tested in 4 cases and their parents.Ultrastructural pathology by skin biopsy was performed in 3 cases respectively...
November 22, 2016: Zhonghua Yi Xue za Zhi [Chinese medical journal]
https://www.readbyqxmd.com/read/27882734/novel-mutations-in-sh3tc2-in-a-young-japanese-girl-with-charcot-marie-tooth-disease-type-4c
#8
Kazushi Ichikawa, Keita Numasawa, Saoko Takeshita, Akihiro Hashiguchi, Hiroshi Takashima
Charcot-Marie-Tooth disease type 4C (CMT4C) is an autosomal recessive demyelinating form of CMT characterized clinically by early onset and severe spinal deformities, and is caused by mutations in SH3TC2. We describe the case of a 10-year-old Japanese girl diagnosed with CMT4C. The patient developed progressive foot deformities such as marked pes cavus and ankle contracture, with mild muscle weakness in both legs, and generalized areflexia. On electrophysiological studies, motor nerve conduction velocity ranged from 22...
November 2016: Pediatrics International: Official Journal of the Japan Pediatric Society
https://www.readbyqxmd.com/read/27874252/pediatric-cardiac-readmissions-an-opportunity-for-quality-improvement
#9
Jeffrey H Sacks, Michael Kelleman, Courtney McCracken, Michelle Glanville, Matthew Oster
OBJECTIVE: Hospital readmissions are increasingly becoming a metric for quality in the current landscape of changing and cost effective medicine. However, no 30-d readmission rates have been established for pediatric cardiac medical patients in the United States. Thus, the objective was to determine 30 d readmission rates and risk factors associated with readmission for pediatric cardiac patients, hypothesizing that pediatric cardiac patients would have significantly higher readmission rates than their general pediatric counterparts...
November 22, 2016: Congenital Heart Disease
https://www.readbyqxmd.com/read/27861220/hereditary-neuropathy-with-liability-to-pressure-palsies-diverse-phenotypes-in-childhood
#10
Yohei Harada, Araya Puwanant, David N Herrmann
Hereditary neuropathy with liability to pressure palsies (HNPP) is a rare autosomal-dominant disorder that most commonly produces recurrent painless focal sensory and motor neuropathies often preceded by minor, mechanical stress, or minor trauma. Herein, we report 2 pediatric cases of HNPP with atypical presentations; isolated muscle cramping and toe walking. Electrophysiologic testing disclosed multifocal sensorimotor polyneuropathy with slowing of sensory conduction velocities in both cases, which prompted PMP 22 gene deletion testing...
December 2016: Journal of Clinical Neuromuscular Disease
https://www.readbyqxmd.com/read/27810292/special-electrophysiological-characteristics-of-pediatric-idiopathic-ventricular-tachycardia
#11
Shuenn-Nan Chiu, Wei-Lun Wu, Chun-Wei Lu, Kun-Lang Wu, Wei-Chieh Tseng, Ming-Tai Lin, Chien-Chih Chang, Jou-Kou Wang, Mei-Hwan Wu
BACKGROUND: Idiopathic ventricular tachycardia (VT) is an important pediatric arrhythmia but relevant studies in Asian cohort are rare. We determined the electrophysiological characteristics and outcomes of this rhythm disturbance. METHODS: From an institutional database, patients aged <18years without structural heart disease who have sustained monomorphic VT (longer than 30s) during 1995-2016 were enrolled. RESULTS: We enrolled 57 idiopathic VT patients (male:female=31:26)...
January 15, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/27793516/-overnight-polysomnography-versus-respiratory-polygraphy-in-the-diagnosis-of-pediatric-obstructive-sleep-apnea
#12
P Franco, H Bourdin, F Braun, J Briffod, I Pin, M-J Challamel
The French Society of Research and Sleep Medicine (SFRMS) organized a meeting on obstructive sleep apnea syndrome (OSAS) in children. A multidisciplinary group of specialists (pulmonologists, ENT surgeons, pediatricians, neurophysiologists, sleep specialists) drew up a consensus document on the value of electrophysiological recordings in the diagnosis of OSAS in children. Technical considerations and recommended sensors, respiratory event definitions, and scoring criteria are presented according to the 2012 and 2014 recommendations of the American Academy of Sleep Medicine (AASM)...
October 25, 2016: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
https://www.readbyqxmd.com/read/27793469/challenges-in-pediatric-chronic-inflammatory-demyelinating-polyneuropathy
#13
Göknur Haliloğlu, Deniz Yüksel, Cağri Mesut Temoçin, Haluk Topaloğlu
Chronic inflammatory demyelinating neuropathy, a treatable immune-mediated disease of the peripheral nervous system is less common in childhood compared to adults. Despite different sets of diagnostic criteria, lack of a reliable biologic marker leads to challenges in diagnosis, follow-up and treatment. Our first aim was to review clinical presentation, course, response to treatment, and prognosis in our childhood patients. We also aimed to document diagnostic and therapeutic pitfalls and challenges at the bedside...
December 2016: Neuromuscular Disorders: NMD
https://www.readbyqxmd.com/read/27785341/sex-and-gender-medicine-in-physician-clinical-training-results-of-a-large-single-center-survey
#14
Shivani Dhawan, May Bakir, Erika Jones, Sarah Kilpatrick, C Noel Bairey Merz
BACKGROUND: "Sex and Gender Medicine" is a novel medical discipline that takes into account the effects of sex and gender on the health of women and men. The Institute of Medicine in the USA declared in its 2001 and 2010 statements that being a woman or a man significantly impacts the course of diseases, and therefore, this fact must be considered in diagnosis and therapy. We evaluated the representation of Sex and Gender Medicine in clinical training at Cedars-Sinai Medical Center, a large, tertiary, non-profit, academic medical training center in the Western United States...
2016: Biology of Sex Differences
https://www.readbyqxmd.com/read/27784736/characteristics-of-decremental-accessory-pathways-in-children
#15
Allison C Hill, Michael J Silka, Choo Phei Wee, Yaniv Bar-Cohen
BACKGROUND: Although retrograde decremental accessory pathways (DAPs) are thought to typically present as permanent junctional reciprocating tachycardia (permanent junctional reciprocating tachycardia), they may also be diagnosed unexpectedly during electrophysiology study. We aimed to compare the clinical and electrophysiological characteristics of patients with DAPs to an age-matched cohort with nondecremental accessory pathways. METHODS AND RESULTS: We retrospectively studied pediatric patients (<21 years of age) with retrograde DAPs and an age-matched control population with nondecremental accessory pathways who underwent electrophysiology study between 2005 and 2014...
November 2016: Circulation. Arrhythmia and Electrophysiology
https://www.readbyqxmd.com/read/27782541/babymeg-a-whole-head-pediatric-magnetoencephalography-system-for-human-brain-development-research
#16
Yoshio Okada, Matti Hämäläinen, Kevin Pratt, Anthony Mascarenas, Paul Miller, Menglai Han, Jose Robles, Anders Cavallini, Bill Power, Kosal Sieng, Limin Sun, Seok Lew, Chiran Doshi, Banu Ahtam, Christoph Dinh, Lorenz Esch, Ellen Grant, Aapo Nummenmaa, Douglas Paulson
We developed a 375-channel, whole-head magnetoencephalography (MEG) system ("BabyMEG") for studying the electrophysiological development of human brain during the first years of life. The helmet accommodates heads up to 95% of 36-month old boys in the USA. The unique two-layer sensor array consists of: (1) 270 magnetometers (10 mm diameter, ∼15 mm coil-to-coil spacing) in the inner layer, (2) thirty-five three-axis magnetometers (20 mm × 20 mm) in the outer layer 4 cm away from the inner layer. Additionally, there are three three-axis reference magnetometers...
September 2016: Review of Scientific Instruments
https://www.readbyqxmd.com/read/27779344/dexmedetomidine-use-in-patients-undergoing-electrophysiological-study-for-supraventricular-tachyarrhythmias
#17
Christopher F Tirotta, Tuan Nguyen, Steven Fishberger, Evelio Velis, Melissa Olen, Lourdes Lam, Danielle R Madril, Jessica Hughes, Richard G Lagueruela
BACKGROUND: Dexmedetomidine is a selective alpha-2 adrenergic agonist with sedative, analgesic, and anxiolytic properties. Dexmedetomidine has not been approved for use in pediatrics. Dexmedetomidine has been reported to depress sinus node and atrioventricular nodal function in pediatric patients; it has been suggested that the use of dexmedetomidine may not be desirable during electrophysiological studies. AIM: We hypothesize that the use of dexmedetomidine does not inhibit the induction of supraventricular tachyarrhythmias (SVT) during electrophysiological studies and does not inhibit the ablation of such arrhythmias...
January 2017: Paediatric Anaesthesia
https://www.readbyqxmd.com/read/27660579/catheter-ablation-to-treat-supraventricular-arrhythmia-in-children-and-adults-with-congenital-heart-disease-what-we-know-and-where-we-are-going
#18
REVIEW
Patricia E Thomas, Scott L Macicek
BACKGROUND: Catheter ablation has been used to manage supraventricular arrhythmia in children since 1990. This article reviews the history of catheter ablation used to treat arrhythmia in children and discusses new frontiers in the field. We also address ablation in adult patients with a history of congenital heart disease (CHD) that was diagnosed and initially treated in childhood. METHODS: We conducted an evidence-based literature review to gather available data on ablation for supraventricular tachycardia in children and adult patients with CHD...
2016: Ochsner Journal
https://www.readbyqxmd.com/read/27638585/clinical-electrophysiological-and-biochemical-markers-of-peripheral-and-central-nervous-system-disease-in-canine-globoid-cell-leukodystrophy-krabbe-s%C3%A2-disease
#19
Allison M Bradbury, Jessica H Bagel, Xuntian Jiang, Gary P Swain, Maria L Prociuk, Caitlin A Fitzgerald, Patricia A O'Donnell, Kyle G Braund, Daniel S Ory, Charles H Vite
Globoid cell leukodystrophy (GLD), or Krabbe's disease, is a debilitating and always fatal pediatric neurodegenerative disease caused by a mutation in the gene encoding the hydrolytic enzyme galactosylceramidase (GALC). In the absence of GALC, progressive loss of myelin and accumulation of a neurotoxic substrate lead to incapacitating loss of motor and cognitive function and death, typically by 2 years of age. Currently, there is no cure. Recent convincing evidence of the therapeutic potential of combining gene and cell therapies in the murine model of GLD has accelerated the requirement for validated markers of disease to evaluate therapeutic efficacy...
November 2016: Journal of Neuroscience Research
https://www.readbyqxmd.com/read/27615598/electrophysiologic-features-of-fibular-neuropathy-in-childhood-and-adolescence
#20
Ioannis Karakis, Mohammad Khoshnoodi, Wendy Liew, Elizabeth S Nguyen, H Royden Jones, Basil T Darras, Peter B Kang
OBJECTIVE: To analyze patterns of nerve injury in pediatric common fibular neuropathy (CFN). METHODS: A retrospective analysis was performed on data from 53 children with CFN at a pediatric electromyography laboratory. RESULTS: Conduction block at the fibular head was present in 35% of patients. Deep fibular axonal loss was identified in 77%, while superficial fibular axonal loss was identified in 45%. The pathophysiology was predominantly axonal in 72%, mostly demyelinating in 6%, and mixed in 22%...
September 12, 2016: Muscle & Nerve
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