Inherited Ventricular Arrhythmias, the Channelopathies and SCD: Current Knowledge and Future Speculations - Risk Stratification, Management Plans and Future Speculations

Channelopathy constitutes significant proportion of SCD worldwide (around 10% or 370,000 deaths annually). It was creating a mysterious group of diseases until the second half of the last century when Anton Jervell and Fred Lange-Nielsen described Jervell Lange-Nielsen syndrome in 1957. It was late until 1995 where genetic characterization commenced. Later on, the massive genetic information with the discovery of genetic heterogeneity and allelic het¬erogeneity was a major victory in the field. The basic sciences in cellular electrophysiology and genetics complemented by meticulous clinical detection and the different clinical trials in the field opened a new era of wide therapeutic choices for clinicians. The knowledge obtained from the different experimental platforms especially the induced pluripotent stem cells is promising. The revolutionary move in SCD and channelopathies is described where correlation between the arrhythmogenesis and fluctuation in SGMA is established and must be investigated. The observation of the arrhythmogenicity of SGMA fluctuation and its effect on HRV together with the differential effect of certain sympathovagal tones (more sympathetic innervation is favoring VT/VF in LQTS1, LQTS2 and SQTS but not BrS or ERS) are all future directions to optimize our preventive, diagnostic as well as therapeutic options of SCD and channelopathy in humans

HRS/EHRA/APHRS Expert Consensus Statement on the Diagnosis and Management of Patients with Inherited Primary Arrhythmia Syndromes

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Executive Summary: HRS/EHRA/APHRS Expert Consensus Statement on the Diagnosis and Management of Patients with Inherited Primary Arrhythmia Syndromes

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Long-term Use of Beta-blocker Medication in Pediatric Long QT Syndrome Patients: Neuropsychological Profiles

Long QT syndrome (LQTS) is a heart rhythm disorder characterized by a disruption of the heart’s electrical activity that may cause accelerated and uncontrolled heartbeats referred to as ventricular fibrillation. LQTS is primarily treated with beta-blocker medications, which reduce the risk of experiencing an arrhythmia through regulating the heart rate. However, the potential neuropsychological side-effects associated with the use of beta-blocker medication may impact the executive functioning skills, mental health, and behavior of the affected pediatric population at home. As a result, a child’s academic performance and emotional regulation etiology may be misunderstood by his or her parents, caregivers, and teachers. The present study used an exploratory research design to further understand and conceptualize neurocognitive profiles of children diagnosed with LQTS and concurrently taking beta-blocker medications. Three participants were administered tailored test batteries with commonly used measures that assess general intellectual functioning, academic achievement, memory and new learning, fine motor and dexterity, and executive functioning skills. Additionally, the participants were administered self-report measures that assess symptoms associated with depression and anxiety, as well as self and parental perceptions of disease impact on quality of life. Overall, the participants demonstrated several inefficiencies within their executive functioning skills and basic sustained attention that had been were documented in their past educational histories, as well as in their performances on measures within the current study

Do Complexity Measures of Frontal EEG Distinguish Loss of Consciousness in Geriatric Patients Under Anesthesia?

While geriatric patients have a high likelihood of requiring anesthesia, they carry an increased risk for adverse cognitive outcomes from its use. Previous work suggests this could be mitigated by better intraoperative monitoring using indexes defined by several processed electroencephalogram (EEG) measures. Unfortunately, inconsistencies between patients and anesthetic agents in current analysis techniques have limited the adoption of EEG as standard of care. In attempts to identify new analyses that discriminate clinically-relevant anesthesia timepoints, we tested 1/f frequency scaling as well as measures of complexity from nonlinear dynamics. Specifically, we tested whether analyses that characterize time-delayed embeddings, correlation dimension (CD), phase-space geometric analysis, and multiscale entropy (MSE) capture loss-of-consciousness changes in EEG activity. We performed these analyses on EEG activity collected from a traditionally hard-to-monitor patient population: geriatric patients on beta-adrenergic blockade who were anesthetized using a combination of fentanyl and propofol. We compared these analyses to traditional frequency-derived measures to test how well they discriminated EEG states before and after loss of response to verbal stimuli. We found spectral changes similar to those reported previously during loss of response. We also found significant changes in 1/f frequency scaling. Additionally, we found that our phase-space geometric characterization of time-delayed embeddings showed significant differences before and after loss of response, as did measures of MSE. Our results suggest that our new spectral and complexity measures are capable of capturing subtle differences in EEG activity with anesthesia administration-differences which future work may reveal to improve geriatric patient monitoring

HRS/EHRA/APHRS Expert Consensus Statement on the Diagnosis and Management of Patients with Inherited Primary Arrhythmia Syndromes

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2015 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death the Task Force for the Management of Patients with Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death of the European Society of Cardiology (ESC) Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC)

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The Trigeminocardiac Reflex — An Example of Reflexive Heart Rhythm Change

The trigeminocardiac reflex (TCR) is defined as the sudden onset of parasympathetic dysrhythmia, sympathetic hypotension, apnoea or gastric hyper-motility during mechanical/thermal stimulation of any of the sensory branches of the trigeminal nerve. The risk factors that are already known for increasing the prevalence of the TCR include anatomical location, hypercapnia, hypoxemia, light general anaesthesia, age (more pronounced in children), the nature of the provoking stimulus (stimulus strength and duration) and different drugs. Already different potential confounders are also identified. This discussion about risk factors has its importance because of the substantial consequences for functional outcome after intraoperative TCR occurrence. But there remains still a substantial lack of thorough understanding of the TCR, the current treatment options for patients with TCR include a mostly empirical approach: (i) risk factor identification and modification; (ii) prophylactic measures of vital signs and (iii) administration of vagolytic agents or sympathomimetics. In this context, we have now created different thinking models so that we can preoperatively plan a skull base surgery procedure safely in relation to a potential occurrence of the TCR episodes. This chapter provides an overview of this unique reflex that presents a unique interaction between heart and brain. In addition, this also illustrates the mechanism of various cardiac rhythm changes related to the TCR