81 research outputs found

    Smart Wearables for Cardiac Monitoring-Real-World Use beyond Atrial Fibrillation

    Get PDF
    The possibilities and implementation of wearable cardiac monitoring beyond atrial fibrillation are increasing continuously. This review focuses on the real-world use and evolution of these devices for other arrhythmias, cardiovascular diseases and some of their risk factors beyond atrial fibrillation. The management of nonatrial fibrillation arrhythmias represents a broad field of wearable technologies in cardiology using Holter, event recorder, electrocardiogram (ECG) patches, wristbands and textiles. Implementation in other patient cohorts, such as ST-elevation myocardial infarction (STEMI), heart failure or sleep apnea, is feasible and expanding. In addition to appropriate accuracy, clinical studies must address the validation of clinical pathways including the appropriate device and clinical decisions resulting from the surrogate assessed

    Catecholaminergic polymorphic ventricular tachycardia patients with multiple genetic variants in the PACES CPVT Registry.

    Get PDF
    BACKGROUND: Catecholaminergic polymorphic ventricular tachycardia (CPVT) is often a life-threatening arrhythmia disorder with variable penetrance and expressivity. Little is known about the incidence or outcomes of CPVT patients with ≥2 variants. METHODS: The phenotypes, genotypes and outcomes of patients in the Pediatric and Congenital Electrophysiology Society CPVT Registry with ≥2 variants in genes linked to CPVT were ascertained. The American College of Medical Genetics & Genomics (ACMG) criteria and structural mapping were used to predict the pathogenicity of variants (3D model of pig RyR2 in open-state). RESULTS: Among 237 CPVT subjects, 193 (81%) had genetic testing. Fifteen patients (8%) with a median age of 9 years (IQR 5-12) had ≥2 variants. Sudden cardiac arrest occurred in 11 children (73%), although none died during a median follow-up of 4.3 years (IQR 2.5-6.1). Thirteen patients (80%) had at least two RYR2 variants, while the remaining two patients had RYR2 variants plus variants in other CPVT-linked genes. Among all variants identified, re-classification of the commercial laboratory interpretation using ACMG criteria led to the upgrade from variant of unknown significance (VUS) to pathogenic/likely pathogenic (P/LP) for 5 variants, and downgrade from P/LP to VUS for 6 variants. For RYR2 variants, 3D mapping using the RyR2 model suggested that 2 VUS by ACMG criteria were P/LP, while 2 variants were downgraded to likely benign. CONCLUSIONS: This severely affected cohort demonstrates that a minority of CPVT cases are related to ≥2 variants, which may have implications on family-based genetic counselling. While multi-variant CPVT patients were at high-risk for sudden cardiac arrest, there are insufficient data to conclude that this genetic phenomenon has prognostic implications at present. Further research is needed to determine the significance and generalizability of this observation. This study also shows that a rigorous approach to variant re-classification using the ACMG criteria and 3D mapping is important in reaching an accurate diagnosis, especially in the multi-variant population

    Flecainide Is Associated With a Lower Incidence of Arrhythmic Events in a Large Cohort of Patients With Catecholaminergic Polymorphic Ventricular Tachycardia

    Get PDF
    BACKGROUND: In severely affected patients with catecholaminergic polymorphic ventricular tachycardia, beta-blockers are often insufficiently protective. The purpose of this study was to evaluate whether flecainide is associated with a lower incidence of arrhythmic events (AEs) when added to beta-blockers in a large cohort of patients with catecholaminergic polymorphic ventricular tachycardia. METHODS: From 2 international registries, this multicenter case cross-over study included patients with a clinical or genetic diagnosis of catecholaminergic polymorphic ventricular tachycardia in whom flecainide was added to beta-blocker therapy. The study period was defined as the period in which background therapy (ie, beta-blocker type [beta1-selective or nonselective]), left cardiac sympathetic denervation, and implantable cardioverter defibrillator treatment status, remained unchanged within individual patients and was divided into pre-flecainide and on-flecainide periods. The primary end point was AEs, defined as sudden cardiac death, sudden cardiac arrest, appropriate implantable cardioverter defibrillator shock, and arrhythmic syncope. The association of flecainide with AE rates was assessed using a generalized linear mixed model assuming negative binomial distribution and random effects for patients. RESULTS: A total of 247 patients (123 [50%] females; median age at start of flecainide, 18 years [interquartile range, 14-29]; median flecainide dose, 2.2 mg/kg per day [interquartile range, 1.7-3.1]) were included. At baseline, all patients used a beta-blocker, 70 (28%) had an implantable cardioverter defibrillator, and 21 (9%) had a left cardiac sympathetic denervation. During a median pre-flecainide follow-up of 2.1 years (interquartile range, 0.4-7.2), 41 patients (17%) experienced 58 AEs (annual event rate, 5.6%). During a median on-flecainide follow-up of 2.9 years (interquartile range, 1.0-6.0), 23 patients (9%) experienced 38 AEs (annual event rate, 4.0%). There were significantly fewer AEs after initiation of flecainide (incidence rate ratio, 0.55 [95% CI, 0.38-0.83]; P=0.007). Among patients who were symptomatic before diagnosis or during the pre-flecainide period (n=167), flecainide was associated with significantly fewer AEs (incidence rate ratio, 0.49 [95% CI, 0.31-0.77]; P=0.002). Among patients with ≥1 AE on beta-blocker therapy (n=41), adding flecainide was also associated with significantly fewer AEs (incidence rate ratio, 0.25 [95% CI, 0.14-0.45]; P&lt;0.001). CONCLUSIONS: For patients with catecholaminergic polymorphic ventricular tachycardia, adding flecainide to beta-blocker therapy was associated with a lower incidence of AEs in the overall cohort, in symptomatic patients, and particularly in patients with breakthrough AEs while on beta-blocker therapy.</p

    Clinical presentation of calmodulin mutations: the International Calmodulinopathy Registry

    Get PDF
    AIMS: Calmodulinopathy due to mutations in any of the three CALM genes (CALM1-3) causes life-threatening arrhythmia syndromes, especially in young individuals. The International Calmodulinopathy Registry (ICalmR) aims to define and link the increasing complexity of the clinical presentation to the underlying molecular mechanisms. METHODS AND RESULTS: The ICalmR is an international, collaborative, observational study, assembling and analysing clinical and genetic data on CALM-positive patients. The ICalmR has enrolled 140 subjects (median age 10.8 years [interquartile range 5-19]), 97 index cases and 43 family members. CALM-LQTS and CALM-CPVT are the prevalent phenotypes. Primary neurological manifestations, unrelated to post-anoxic sequelae, manifested in 20 patients. Calmodulinopathy remains associated with a high arrhythmic event rate (symptomatic patients, n = 103, 74%). However, compared with the original 2019 cohort, there was a reduced frequency and severity of all cardiac events (61% vs. 85%; P = .001) and sudden death (9% vs. 27%; P = .008). Data on therapy do not allow definitive recommendations. Cardiac structural abnormalities, either cardiomyopathy or congenital heart defects, are present in 30% of patients, mainly CALM-LQTS, and lethal cases of heart failure have occurred. The number of familial cases and of families with strikingly different phenotypes is increasing. CONCLUSION: Calmodulinopathy has pleiotropic presentations, from channelopathy to syndromic forms. Clinical severity ranges from the early onset of life-threatening arrhythmias to the absence of symptoms, and the percentage of milder and familial forms is increasing. There are no hard data to guide therapy, and current management includes pharmacological and surgical antiadrenergic interventions with sodium channel blockers often accompanied by an implantable cardioverter-defibrillator

    Genomic Characterization of Methanomicrobiales Reveals Three Classes of Methanogens

    Get PDF
    BACKGROUND:Methanomicrobiales is the least studied order of methanogens. While these organisms appear to be more closely related to the Methanosarcinales in ribosomal-based phylogenetic analyses, they are metabolically more similar to Class I methanogens. METHODOLOGY/PRINCIPAL FINDINGS:In order to improve our understanding of this lineage, we have completely sequenced the genomes of two members of this order, Methanocorpusculum labreanum Z and Methanoculleus marisnigri JR1, and compared them with the genome of a third, Methanospirillum hungatei JF-1. Similar to Class I methanogens, Methanomicrobiales use a partial reductive citric acid cycle for 2-oxoglutarate biosynthesis, and they have the Eha energy-converting hydrogenase. In common with Methanosarcinales, Methanomicrobiales possess the Ech hydrogenase and at least some of them may couple formylmethanofuran formation and heterodisulfide reduction to transmembrane ion gradients. Uniquely, M. labreanum and M. hungatei contain hydrogenases similar to the Pyrococcus furiosus Mbh hydrogenase, and all three Methanomicrobiales have anti-sigma factor and anti-anti-sigma factor regulatory proteins not found in other methanogens. Phylogenetic analysis based on seven core proteins of methanogenesis and cofactor biosynthesis places the Methanomicrobiales equidistant from Class I methanogens and Methanosarcinales. CONCLUSIONS/SIGNIFICANCE:Our results indicate that Methanomicrobiales, rather than being similar to Class I methanogens or Methanomicrobiales, share some features of both and have some unique properties. We find that there are three distinct classes of methanogens: the Class I methanogens, the Methanomicrobiales (Class II), and the Methanosarcinales (Class III)

    The state of the art in monitoring and verification—Ten years on

    Full text link

    Factors Contributing to Clinical Judgment Development in Nursing Students During Simulation Using the Creighton Competency Evaluation Instrument

    No full text
    It is imperative that nurse educators find inventive ways to advance nursing practice by developing a dynamic and divergent nursing workforce. Health care environments can be unpredictable and unsettling due to high acuity levels of patients. The development of excellent clinical judgment is necessary for nurses to meet the workforce demands that this type of environment poses. Nurse leaders report dissatisfaction with new graduates' ability to adequately perform competencies such as critical thinking. Nurse educators must establish approaches that teach, enforce, and evaluate the development of higher level thinking in nursing students. This non-experimental descriptive correlational study explored factors that affect the development of clinical judgment in Bachelor of Science nursing students during a synthesis simulation. The Creighton Competency Evaluation Instrument (CCEI) was used to measure clinical judgment. Tanner's Clinical Judgment Model provided the theoretical foundation for this study. The clinical judgment of 108 BSN students in their fourth semester of a traditional nursing program was assessed by the researcher using the CCEI in the lab during a synthesis simulation. Students also completed a demographic questionnaire. Statistical methods used to analyze the data included descriptive statistics, independent samples t-tests, one way analysis of variance, and Pearson product correlation. Males were found to have significantly higher overall clinical judgment. Males also scored statistically higher on the communication subscale. There were no other statistical differences in demographics or the subscale scores of the CCEI. Work experience had no significant impact on total clinical judgment scores. Participants that reported working in healthcare scored significantly higher on the patient safety subscale of the CCEI that those reporting working but not in healthcare. The unemployed group did not differ significantly from the employed in healthcare or the employed not in healthcare groups on the patient safety subscale. Small correlations were found between HESITM (E2) raw scores and CCEI total as well as communication and clinical judgment subscale scores. The findings of this study contributed to the overall knowledge about clinical judgment and the CCEI as a means to evaluate clinical judgment. Application of findings could be used to increase the use of simulation and foster the development of clinical judgment in nursing students. Clinical judgment terminology should be a standard part of every nursing curriculum. Simulation, with an emphasis on clinical judgment, could be used to increase HESI[trademark] (E2) scores of students thus better preparing them for NCLEX. It is recommended that future studies use a longitudinal approach to measuring clinical judgment. This would provide more discernible data to evaluate the development of clinical judgment over time. It is also recommended that more research be done to establish the CCEI as a valid and reliable tool to measure clinical judgment as well as faculty perceptions of the CCEI

    Factors Contributing to Clinical Judgment Development in Nursing Students During Simulation Using the Creighton Competency Evaluation Instrument

    No full text
    It is imperative that nurse educators find inventive ways to advance nursing practice by developing a dynamic and divergent nursing workforce. Health care environments can be unpredictable and unsettling due to high acuity levels of patients. The development of excellent clinical judgment is necessary for nurses to meet the workforce demands that this type of environment poses. Nurse leaders report dissatisfaction with new graduates' ability to adequately perform competencies such as critical thinking. Nurse educators must establish approaches that teach , enforce , and evaluate the development of higher level thinking in nursing students. This non-experimental descriptive correlational study explored factors that affect the development of clinical judgment in Bachelor of Science nursing students during a synthesis simulation. The Creighton Competency Evaluation Instrument (CCEI) was used to measure clinical judgment. Tanner's Clinical Judgment Model provided the theoretical foundation for this study. The clinical judgment of 108 BSN students in their fourth semester of a traditional nursing program was assessed by the researcher using the CCEI in the lab during a synthesis simulation. Students also completed a demographic questionnaire. Statistical methods used to analyze the data included descriptive statistics , independent samples t-tests , one way analysis of variance , and Pearson product correlation. Males were found to have significantly higher overall clinical judgment. Males also scored statistically higher on the communication subscale. There were no other statistical differences in demographics or the subscale scores of the CCEI. Work experience had no significant impact on total clinical judgment scores. Participants that reported working in healthcare scored significantly higher on the patient safety subscale of the CCEI that those reporting working but not in healthcare. The unemployed group did not differ significantly from the employed in healthcare or the employed not in healthcare groups on the patient safety subscale. Small correlations were found between HESITM (E2) raw scores and CCEI total as well as communication and clinical judgment subscale scores. The findings of this study contributed to the overall knowledge about clinical judgment and the CCEI as a means to evaluate clinical judgment. Application of findings could be used to increase the use of simulation and foster the development of clinical judgment in nursing students. Clinical judgment terminology should be a standard part of every nursing curriculum. Simulation , with an emphasis on clinical judgment , could be used to increase HESI[trademark] (E2) scores of students thus better preparing them for NCLEX. It is recommended that future studies use a longitudinal approach to measuring clinical judgment. This would provide more discernible data to evaluate the development of clinical judgment over time. It is also recommended that more research be done to establish the CCEI as a valid and reliable tool to measure clinical judgment as well as faculty perceptions of the CCEI

    Modern Approach to the Sudden Unexpected Death Investigation

    No full text
    • …
    corecore