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Children (Basel, Switzerland) Apr 2024CACNA1C gene encodes the alpha 1 subunit of the CaV1.2 L-type Ca2+ channel. Pathogenic variants in this gene have been associated with cardiac rhythm disorders such as... (Review)
Review
CACNA1C gene encodes the alpha 1 subunit of the CaV1.2 L-type Ca2+ channel. Pathogenic variants in this gene have been associated with cardiac rhythm disorders such as long QT syndrome, Brugada syndrome and Timothy syndrome. Recent evidence has suggested the possible association between CACNA1C mutations and neurologically-isolated (in absence of cardiac involvement) phenotypes in children, giving birth to a wider spectrum of CACNA1C-related clinical presentations. However, to date, little is known about the variety of both neurological and non-neurological signs/symptoms in the neurologically-predominant phenotypes. We conducted a systematic review of neurologically-predominant presentations without cardiac conduction defects, associated with CACNA1C mutations. We also reported a novel de novo missense pathogenic variant in the CACNA1C gene of a children patient presenting with constructional, dressing and oro-buccal apraxia associated with behavioral abnormalities, mild intellectual disability, dental anomalies, gingival hyperplasia and mild musculoskeletal defects, without cardiac conduction defects. The present study highlights the importance of considering the investigation of the CACNA1C gene in children's neurological isolated syndromes, and expands the phenotype of the CACNA1C related conditions. In addition, the present study highlights that, even in absence of cardiac conduction defects, nuanced clinical manifestations of the Timothy syndrome (e.g., dental and gingival defects) could be found. These findings suggest the high variable expressivity of the CACNA1C gene and remark that the absence of cardiac involvement should not mislead the diagnosis of a CACNA1C related disorder.
PubMed: 38790536
DOI: 10.3390/children11050541 -
Clinical Lung Cancer Jun 2024A systematic literature review was conducted to determine the incidence and mortality of QT-interval prolongation (QTp), torsades de pointes (TdP), and heart failure... (Review)
Review
A systematic literature review was conducted to determine the incidence and mortality of QT-interval prolongation (QTp), torsades de pointes (TdP), and heart failure (HF) in patients with non-small cell lung cancer (NSCLC) who received epidermal growth factor receptor (EGFR) TKIs. Of 296 identified publications, 95 met eligibility criteria and were abstracted for QTp/TdP and HF outcomes (QTp/TdP: 83 publications, including 5 case study publications; HF: 79 publications, including 6 case study publications [involving 8 patients]). QTp incidence ranged from 0% to 27.8% in observational studies and from 0% to 11% in clinical trials, with no deaths due to QTp. There were no TdP events or deaths due to TdP. The incidence of HF ranged from 0% to 8%, and HF mortality rates ranged from 0% to 4%. Patients receiving treatment with EGFR TKIs should be monitored for signs of QTp, TdP, and HF per prescribing information. Standardized definitions and methods to improve monitoring of QTp, TdP, and HF-related events are needed in patients with NSCLC.
Topics: Humans; Carcinoma, Non-Small-Cell Lung; Heart Failure; Lung Neoplasms; ErbB Receptors; Torsades de Pointes; Protein Kinase Inhibitors; Long QT Syndrome; Incidence; Tyrosine Kinase Inhibitors
PubMed: 38553324
DOI: 10.1016/j.cllc.2024.02.005 -
The Cochrane Database of Systematic... Mar 2024Cardiovascular diseases (CVDs) are the leading cause of death globally, accounting for almost 18 million deaths annually. People with CVDs have a five times greater... (Review)
Review
BACKGROUND
Cardiovascular diseases (CVDs) are the leading cause of death globally, accounting for almost 18 million deaths annually. People with CVDs have a five times greater chance of suffering a recurrent cardiovascular event than people without known CVDs. Although drug interventions have been shown to be cost-effective in reducing the risk of recurrent cardiovascular events, adherence to medication remains suboptimal. As a scalable and cost-effective approach, mobile phone text messaging presents an opportunity to convey health information, deliver electronic reminders, and encourage behaviour change. However, it is uncertain whether text messaging can improve medication adherence and clinical outcomes. This is an update of a Cochrane review published in 2017.
OBJECTIVES
To evaluate the benefits and harms of mobile phone text messaging for improving medication adherence in people with CVDs compared to usual care.
SEARCH METHODS
We searched CENTRAL, MEDLINE, Embase, four other databases, and two trial registers. We also checked the reference lists of all primary included studies and relevant systematic reviews and meta-analyses. The date of the latest search was 30 August 2023.
SELECTION CRITERIA
We included randomised controlled trials (RCTs) with participants with established arterial occlusive events. We included trials investigating interventions using short message service (SMS) or multimedia messaging service (MMS) with the aim of improving adherence to medication for the secondary prevention of cardiovascular events. The comparator was usual care. We excluded cluster-RCTs and quasi-RCTs.
DATA COLLECTION AND ANALYSIS
We used standard Cochrane methods. Our primary outcomes were medication adherence, fatal cardiovascular events, non-fatal cardiovascular events, and combined CVD event. Secondary outcomes were low-density lipoprotein cholesterol for the effect of statins, blood pressure for antihypertensive drugs, heart rate for the effect of beta-blockers, urinary 11-dehydrothromboxane B2 for the antiplatelet effects of aspirin, adverse effects, and patient-reported experience. We used GRADE to assess the certainty of the evidence for each outcome.
MAIN RESULTS
We included 18 RCTs involving a total of 8136 participants with CVDs. We identified 11 new studies in the review update and seven studies in the previous version of the review. Participants had various CVDs including acute coronary syndrome, coronary heart disease, stroke, myocardial infarction, and angina. All studies were conducted in middle- and high-income countries, with no studies conducted in low-income countries. The mean age of participants was 53 to 64 years. Participants were recruited from hospitals or cardiac rehabilitation facilities. Follow-up ranged from one to 12 months. There was variation in the characteristics of text messages amongst studies (e.g. delivery method, frequency, theoretical grounding, content used, personalisation, and directionality). The content of text messages varied across studies, but generally included medication reminders and healthy lifestyle information such as diet, physical activity, and weight loss. Text messages offered advice, motivation, social support, and health education to promote behaviour changes and regular medication-taking. We assessed risk of bias for all studies as high, as all studies had at least one domain at unclear or high risk of bias. Medication adherence Due to different evaluation score systems and inconsistent definitions applied for the measurement of medication adherence, we did not conduct meta-analysis for medication adherence. Ten out of 18 studies showed a beneficial effect of mobile phone text messaging for medication adherence compared to usual care, whereas the other eight studies showed either a reduction or no difference in medication adherence with text messaging compared to usual care. Overall, the evidence is very uncertain about the effects of mobile phone text messaging for medication adherence when compared to usual care. Fatal cardiovascular events Text messaging may have little to no effect on fatal cardiovascular events compared to usual care (odds ratio 0.83, 95% confidence interval (CI) 0.47 to 1.45; 4 studies, 1654 participants; low-certainty evidence). Non-fatal cardiovascular events We found very low-certainty evidence that text messaging may have little to no effect on non-fatal cardiovascular events. Two studies reported non-fatal cardiovascular events, neither of which found evidence of a difference between groups. Combined CVD events We found very low-certainty evidence that text messaging may have little to no effect on combined CVD events. Only one study reported combined CVD events, and did not find evidence of a difference between groups. Low-density lipoprotein cholesterol Text messaging may have little to no effect on low-density lipoprotein cholesterol compared to usual care (mean difference (MD) -1.79 mg/dL, 95% CI -4.71 to 1.12; 8 studies, 4983 participants; very low-certainty evidence). Blood pressure Text messaging may have little to no effect on systolic blood pressure (MD -0.93 mmHg, 95% CI -3.55 to 1.69; 8 studies, 5173 participants; very low-certainty evidence) and diastolic blood pressure (MD -1.00 mmHg, 95% CI -2.49 to 0.50; 5 studies, 3137 participants; very low-certainty evidence) when compared to usual care. Heart rate Text messaging may have little to no effect on heart rate compared to usual care (MD -0.46 beats per minute, 95% CI -1.74 to 0.82; 4 studies, 2946 participants; very low-certainty evidence).
AUTHORS' CONCLUSIONS
Due to limited evidence, we are uncertain if text messaging reduces medication adherence, fatal and non-fatal cardiovascular events, and combined cardiovascular events in people with cardiovascular diseases when compared to usual care. Furthermore, text messaging may result in little or no effect on low-density lipoprotein cholesterol, blood pressure, and heart rate compared to usual care. The included studies were of low methodological quality, and no studies assessed the effects of text messaging in low-income countries or beyond the 12-month follow-up. Long-term and high-quality randomised trials are needed, particularly in low-income countries.
Topics: Humans; Middle Aged; Text Messaging; Cardiovascular Diseases; Secondary Prevention; Cell Phone; Cholesterol, LDL; Medication Adherence
PubMed: 38533994
DOI: 10.1002/14651858.CD011851.pub3 -
Pediatric Blood & Cancer May 2024Previous studies have documented that electrocardiography (ECG) can reveal a range of abnormalities, offering valuable insights into the cardiac evaluation of patients... (Meta-Analysis)
Meta-Analysis Review
BACKGROUND
Previous studies have documented that electrocardiography (ECG) can reveal a range of abnormalities, offering valuable insights into the cardiac evaluation of patients with sickle cell disease (SCD). The objective of this study is to assess the patterns of ECG abnormalities observed in these patients with SCD, and to determine their prevalence.
METHOD
We systematically reviewed the literature using online databases of PubMed, Scopus, Web of Science, Embase, and Google Scholar to identify original studies that reported findings of standard ECG assessments in patients with SCD. Statistical analyses were performed using the random effects model. Additional analyses including sensitivity analysis and subgroup analysis were also conducted.
RESULTS
Analysis of data from 59 studies involving 897,920 individuals with SCD revealed that 75% of these patients had abnormal ECG findings (67%-81%), which were predominantly nonspecific ST-T changes, left ventricular hypertrophy, T-wave changes, prolonged corrected QT (QTc) interval, and ischemic changes. Besides, it was shown that these patients had significantly higher odds of having any ECG abnormalities (OR of 17.50, 4.68-65.49), right atrial enlargement (6.09, 1.48-25.09), left ventricular hypertrophy (3.45, 1.73-6.89), right ventricular hypertrophy (7.18, 2.28-22.57), biventricular hypertrophy (10.11, 1.99-51.38), prolonged QTc interval (5.54, 2.44-12.59), ST depression (3.34, 1.87-5.97), and T-wave changes (5.41, 1.43-20.56). Moreover, the mean of QTc interval was significantly higher among those with SCD (23.51 milliseconds, 16.08-30.94).
CONCLUSION
Our meta-analysis showed a higher prevalence of abnormal ECG findings among individuals with SCD. A significant proportion of these patients had various ECG abnormalities, suggesting a potential need for regular ECG assessments for patients with SCD.
Topics: Humans; Hypertrophy, Left Ventricular; Anemia, Sickle Cell; Electrocardiography
PubMed: 38348531
DOI: 10.1002/pbc.30916 -
Drugs Feb 2024Pain associated with cancer is a common feature among children and adolescents. Among opioids, methadone is a unique drug for its multiple mechanisms of action.... (Review)
Review
Pain associated with cancer is a common feature among children and adolescents. Among opioids, methadone is a unique drug for its multiple mechanisms of action. Methadone is currently underutilized in children. The use of methadone for cancer pain management in children was assessed in a systematic review. Altogether, 141 children receiving methadone were examined, and another 126 children were assessed for QT prolongation. In the clinical studies, modalities of use, dosing, and duration of assessment were highly variable. In general, methadone was effective and well tolerated with a limited tendency for dose increases. QT prolongation was reported in a percentage of patients independently of the dosages or other variables. The majority of studies considered the use of methadone to be safe and effective in children. Despite methadone possessing interesting properties that make this drug unique in a pediatric context, data is limited, and the literature available is based on retrospective studies. Methadone could be an effective, inexpensive, and versatile medication in children with cancer who have pain. This drug deserves more interest and should prompt studies of better quality with a larger number of patients.
Topics: Adolescent; Humans; Child; Methadone; Pain Management; Retrospective Studies; Analgesics, Opioid; Pain; Neoplasms; Long QT Syndrome
PubMed: 38324240
DOI: 10.1007/s40265-024-02001-y -
British Paramedic Journal Dec 2023The current Joint Royal Colleges Ambulance Liaison Committee guidelines in the United Kingdom provide clear national guidance for low-voltage electrical injury patients....
INTRODUCTION
The current Joint Royal Colleges Ambulance Liaison Committee guidelines in the United Kingdom provide clear national guidance for low-voltage electrical injury patients. While patients can be considered safe to discharge with an apparently 'normal' initial electrocardiogram (ECG), some evidence questions the safety profile of these patients with a risk of a 'delayed arrhythmia'. This review aims to examine this as well as identifying the frequency and common arrhythmias that require patients to be conveyed to hospital for further monitoring post electrical injury. It will also aim to improve the understanding of potentially clinically significant arrhythmias that may require clinical intervention or even admission within an in-hospital environment.
METHODS
A systematic review using three electronic databases (CINAHL, MEDLINE, AMED) was conducted in January 2022. A preferred reporting items for systematic reviews and meta-analyses (PRISMA) approach was used to identify relevant studies with a suitable quality to support a critical review of the topic. A modified Critical Appraisal Skills Programme quality assessment checklist was used across suitable studies and a descriptive statistics approach was adopted to present the findings.
RESULTS
Seven studies, largely retrospective reviews, met the inclusion criteria. The findings showed 26% of patients had an arrhythmia on initial presentation (n = 364/1234) with incidences of sinus tachycardia, sinus bradycardia and premature ventricular contractions. However, making definitive statements is challenging due to the lack of access to individual patients' past ECGs. Within these arrhythmias' ST segment changes, atrial fibrillation and long QT syndrome could be considered potentially significant, however associated prognosis with these and electrical injuries is unknown. Only six (0.5%) patients required treatment by drug therapy, and a further three died from associated complications. Most patients with a normal ECG were discharged immediately with only a limited follow-up. No presentation of a 'delayed arrhythmia' was identified throughout the studies.
CONCLUSION
The data for low-voltage electrical injuries are limited, but the potential arrhythmias for this patient group seldom require intervention. The entity of the 'delayed arrhythmia' may not be a reason to admit or monitor patients for prolonged periods. Further studies should consider the safety profile of discharging a patient with a normal ECG.
PubMed: 38046790
DOI: 10.29045/14784726.2023.12.8.3.27 -
Journal of Arrhythmia Dec 2023Inherited Primary Arrhythmias Syndromes (IPAS), especially Brugada syndrome (BrS), have been associated with arrhythmogenic substrates that can be targeted through...
BACKGROUND
Inherited Primary Arrhythmias Syndromes (IPAS), especially Brugada syndrome (BrS), have been associated with arrhythmogenic substrates that can be targeted through ablation. This meta-analysis evaluated the outcomes of catheter ablation (CA) in different types of IPAS based on procedural guidance and location.
METHODS
A systematic search was conducted across multiple databases to identify studies reporting on ventricular arrhythmia (VA) events before and after CA in IPAS, including BrS, Long-QT syndrome (LQTS), Early repolarization syndrome (ERS), and Idiopathic ventricular fibrillation (IVF). The primary outcomes were VA recurrence and VA burden, evaluated through conditional subgroup analysis. Procedural data were collected as secondary outcomes.
RESULTS
A total of 21 studies involving 584 IPAS patients who underwent CA were included. Following a mean follow-up duration of 33.5 months, substrate-based ablation demonstrated efficacy in reducing VA recurrence across all types of IPAS [RR 0.23; 95% CI (0.13-0.39); < .001; = 74%]. However, activation guidance ablation was found to be effective only in IVF cases. Although recurrences still occurred, CA was successful in reducing VA burden [MD -4.70; 95% CI (-6.11-(-3.29); < .001; = 74%]. The mean size of arrhythmogenic substrate was 15.70 cm [95% CI (12.34-19.99 cm)], predominantly distributed in the epicardial right ventricular outflow tract (RVOT) in BrS cases and LQTS [Proportion 0.99; 95% CI (0.96-1.00) and Proportion 0.82; 95% CI ( 0.59-1.00), respectively].
CONCLUSION
Substrate-based CA has demonstrated effective prevention of VA and reduction in VA burden in IPAS cases.
PubMed: 38045449
DOI: 10.1002/joa3.12947 -
Heart Rhythm Nov 2022Electronic gaming has recently been reported as a precipitant of life-threatening cardiac arrhythmia in susceptible individuals.
BACKGROUND
Electronic gaming has recently been reported as a precipitant of life-threatening cardiac arrhythmia in susceptible individuals.
OBJECTIVE
The purpose of this study was to describe the population at risk, the nature of cardiac events, and the type of game linked to cardiac arrhythmia associated with electronic gaming.
METHODS
A multisite international case series of suspected or proven cardiac arrhythmia during electronic gaming in children and a systematic review of the literature were performed.
RESULTS
Twenty-two patients (18 in the case series and 4 via systematic review; aged 7-16 years; 19 males [86%]) were identified as having experienced suspected or proven ventricular arrhythmia during electronic gaming; 6 (27%) had experienced cardiac arrest, and 4 (18%) died suddenly. A proarrhythmic cardiac diagnosis was known in 7 (31%) patients before their gaming event and was established afterward in 12 (54%). Ten patients (45%) had catecholaminergic polymorphic ventricular tachycardia, 4 (18%) had long QT syndrome, 2 (9%) were post-congenital cardiac surgery, 2 (9%) had "idiopathic" ventricular fibrillation, and 1 (after Kawasaki disease) had coronary ischemia. In 3 patients (14%), including 2 who died, the diagnosis remains unknown. In 13 (59%) patients for whom the electronic game details were known, 8 (62%) were war games.
CONCLUSION
Electronic gaming can precipitate lethal cardiac arrhythmias in susceptible children. The incidence appears to be low, but syncope in this setting should be investigated thoroughly. In children with proarrhythmic cardiac conditions, electronic war games in particular are a potent arrhythmic trigger.
Topics: Male; Child; Humans; Arrhythmias, Cardiac; Heart; Tachycardia, Ventricular; Death, Sudden; Video Games; Death, Sudden, Cardiac
PubMed: 37850595
DOI: 10.1016/j.hrthm.2022.08.003 -
Biochemical and Biophysical Research... Nov 2023QT prolongation is one of the main unwanted cardiac effects caused by drugs, such as anti-psychotics and anti-depressants, inducing mainly via hERG channel dysfunction....
PURPOSE
QT prolongation is one of the main unwanted cardiac effects caused by drugs, such as anti-psychotics and anti-depressants, inducing mainly via hERG channel dysfunction. The precise and underlying mechanism of adverse effects on hERG channel are still indecisive, but these effects limit their use in patients with cardiac risk factors. The aim of this review was studying mechanism of Long-term QT syndrome induction via hERG channel dysfunction by these Drugs.
METHOD
Search was performed in PubMed, and Scopus. All human, animals, and cell lines studies, English and full text publications were included. Among 1280 papers, 23 studies were eligible for more assessments. Quality of studies cheeked by two researchers independently.
KEY FINDING
most of studies were done on anti-psychotic drugs, especially typical class. Most used investigated method to long-term QT induction was patch clamp.
SIGNIFICANCE
results suggests in susceptible cases with heart risk factors, these drugs should be taken with caution and monitored.
PubMed: 37774574
DOI: 10.1016/j.bbrc.2023.09.043 -
Clinical Cardiology Sep 2023Screening elite athletes for conditions associated with sudden cardiac death is recommended by numerous international guidelines. Current athlete electrocardiogram... (Review)
Review
Screening elite athletes for conditions associated with sudden cardiac death is recommended by numerous international guidelines. Current athlete electrocardiogram interpretation criteria recommend the Bazett formula (QTcB) for correcting QT interval. However, other formulae may perform better at lower and higher heart rates (HR). This review aimed to examine the literature on various QT correction methods in athletes and young people aged 14-35 years and determine the most accurate method of calculating QTc in this population. A systematic review of MEDLINE, EMBASE, Scopus, and SportDiscus was performed. Papers comparing at least two different methods of QT interval correction in athletes or young people were included. Quality and risk of bias were assessed using a standardized tool. The search strategy identified 545 papers, of which 10 met the criteria and were included. Nine of these studies concluded that QTcB was least reliable for removing the effect of HR and was inaccurate at both high (>90 beats per min [BPM]) and low (<60 BPM) HRs. No studies supported the use of QTcB in athletes and young people. Alternative QT correction algorithms such as Fridericia (QTcF) produce more accurate correction of QT interval at HRs seen in athletes and young people. QTcB is less accurate at lower and higher HRs. QTcF has been shown to be more accurate in these HR ranges and may be preferred to QTcB for QTc calculation in athletes and young people. However, accurate QTc reference values for discrete HRs using alternative algorithms are not well established and require further research.
Topics: Humans; Adolescent; Long QT Syndrome; Heart Rate; Death, Sudden, Cardiac; Athletes; Algorithms; Electrocardiography
PubMed: 37470093
DOI: 10.1002/clc.24093