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Circulation Feb 2020Long 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...
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 LQTS is required.
METHODS
Utilizing an evidence-based framework, 3 gene curation teams blinded to each other's work scored the level of evidence for 17 genes reported to cause LQTS. A Clinical Domain Channelopathy Working Group provided a final classification of these genes for causation of LQTS after assessment of the evidence scored by the independent curation teams.
RESULTS
Of 17 genes reported as being causative for LQTS, 9 () were classified as having limited or disputed evidence as LQTS-causative genes. Only 3 genes () were curated as definitive genes for typical LQTS. Another 4 genes () were found to have strong or definitive evidence for causality in LQTS with atypical features, including neonatal atrioventricular block. The remaining gene () had moderate level evidence for causing LQTS.
CONCLUSIONS
More than half of the genes reported as causing LQTS have limited or disputed evidence to support their disease causation. Genetic variants in these genes should not be used for clinical decision-making, unless accompanied by new and sufficient genetic evidence. The findings of insufficient evidence to support gene-disease associations may extend to other disciplines of medicine and warrants a contemporary evidence-based evaluation for previously reported disease-causing genes to ensure their appropriate use in precision medicine.
Topics: Atrioventricular Block; Evidence-Based Medicine; Female; Genetic Diseases, Inborn; Genetic Predisposition to Disease; Humans; Long QT Syndrome; Male; Multicenter Studies as Topic
PubMed: 31983240
DOI: 10.1161/CIRCULATIONAHA.119.043132 -
Heart Rhythm Aug 2020Failure of drugs and catheter ablation procedures for the treatment of ventricular arrhythmias is still extremely relevant. Recently, stereotactic body radiotherapy has...
Failure of drugs and catheter ablation procedures for the treatment of ventricular arrhythmias is still extremely relevant. Recently, stereotactic body radiotherapy has been introduced to treat therapy refractory patients. In this systematic review (International Prospective Register of Systematic Reviews, CRD42019133212), we aimed to summarize electrophysiological and histopathological effects of radioablation in animals, patients, and extracted and perfused hearts. A systematic search was performed in OVID MEDLINE, OVID Embase, the Cochrane Central Register of Controlled Trials, Web of Science, Google Scholar, ClinicalTrials.gov, and World Health Organization International Clinical Trials Registry Platform (WHO ICTRP) from inception to September 2019. Identified records were independently screened for eligibility by 2 reviewers. Risk of bias and methodological quality were assessed using the SYRCLE, ROBINS-I, or Murad tool and tailored to the different study designs. We included 13 preclinical and 10 clinical publications. Large heterogeneity in study designs prompted a narrative synthesis approach. Baseline, (pre-)procedural details, outcome, target tissue analyses, and safety data were extracted and summarized. In animal studies evaluating electrophysiological parameters, radioablation induced a reduction in voltage/potential amplitude or bidirectional block in target areas in 93.2% of animals. Atrioventricular block (first to third degree) was induced in 78.3% of animals, and in studies evaluating ventricular arrhythmia inducibility, 75% reduction was achieved. In patients, predominantly ventricular tachycardias were targeted with >85% reduction in arrhythmia episodes during follow-up with an encouraging short-term safety profile. Preclinical and clinical evidence on the efficacy and safety of radioablation is limited in both quantity and quality. The results of radioablation for therapy refractory patients with ventricular tachycardia are promising, but further research is needed.
Topics: Catheter Ablation; Heart Conduction System; Humans; Tachycardia, Ventricular
PubMed: 32205299
DOI: 10.1016/j.hrthm.2020.03.013 -
Frontiers in Cardiovascular Medicine 2023Atrioventricular block (AVB) is a serious complication following coronary artery bypass grafting (CABG) surgery, and its high-grade form may necessitate the implantation... (Review)
Review
BACKGROUND AND OBJECTIVES
Atrioventricular block (AVB) is a serious complication following coronary artery bypass grafting (CABG) surgery, and its high-grade form may necessitate the implantation of a permanent pacemaker (PPM). AVB is associated with increased morbidity and mortality rates. This study aims to estimate the incidence of AVB and subsequent PPM implantation after isolated CABG surgery.
MATERIAL AND METHODS
We searched electronic databases of PubMed, Embase, and Scopus from inception to 18 November 2022. Clinical trials and observational studies reporting the incidence of post-CABG AVB or subsequent PPM implantation in adult patients were included. The total incidence for all included outcomes was calculated using the inverse variance method, and the statistic was reported to evaluate the heterogeneity of studies.
RESULTS
A total of 28 studies met the inclusion criteria. Four studies [3 cohorts, 1 randomized controlled trial (RCT)] reported AVB without specifying its type; one (cohort) reported different degrees of AVB, 20 (12 cohorts, 8 RCTs) reported complete heart block (CHB) (or AVB requiring temporary pacing), and nine (8 cohorts, 1 RCT) reported the number of PPM inserted due to AVB. The pooled incidence of AVB, CHB (or AVB requiring temporary pacing), and PPM due to AVB was 1.16%, 1.73%, and 0.58%, respectively. Meta-regression analysis revealed that age, gender, diabetes, hypertension, hyperlipidemia, or smoking were not significantly associated with AVB, CHB, or PPM implantation.
CONCLUSION
This study highlights the incidence of AVB and the need for PPM implantation following CABG surgery. The findings emphasize the importance of postoperative monitoring and surveillance to improve patient outcomes.
SYSTEMATIC REVIEW REGISTRATION
https://www.crd.york.ac.uk/prospero/display_record.php?ID=CRD42022377181, identifier PROSPERO CRD42022377181.
PubMed: 37593149
DOI: 10.3389/fcvm.2023.1225833 -
Heart Rhythm Apr 2023Fetal long QT syndrome (LQTS) may present with sinus bradycardia, functional 2:1 atrioventricular block (AVB), and ventricular arrhythmias (ventricular tachycardia... (Meta-Analysis)
Meta-Analysis Review
Fetal long QT syndrome (LQTS) may present with sinus bradycardia, functional 2:1 atrioventricular block (AVB), and ventricular arrhythmias (ventricular tachycardia [VT]/torsades de pointes [TdP]) and lead to fetal or postnatal death. We performed a systematic review and individual participant data meta-analysis of 83 studies reporting outcomes of 265 fetuses for which suspected LQTS was confirmed postnatally and determined risk of adverse perinatal and postnatal outcomes using logistic and stepwise logistic regression. A longer fetal QTc was more predictive of death than any other antenatal factor (receiver operating characteristic [ROC] area under the curve [AUC] 0.85; 95% confidence interval [CI] 0.66-1.00). Risk of death was significantly increased with fetal QTc >600 ms. Neither fetal heart rate nor heart rate z-score predicted death (ROC AUC 0.51; 95% CI 0.31-0.71; and ROC AUC 0.59; 95% CI 0.37-0.80, respectively). The combination of antenatal VT/TdP or functional 2:1 AVB and lack of family history of LQTS was also highly predictive of death (ROC AUC 0.82; 95% CI 0.76-0.88). Our data provide clinical screening tools to enable prediction and intervention for fetuses with LQTS at risk of death.
Topics: Humans; Pregnancy; Female; Electrocardiography; Long QT Syndrome; Torsades de Pointes; Heart Rate, Fetal; Atrioventricular Block; Fetus; DNA-Binding Proteins
PubMed: 36566891
DOI: 10.1016/j.hrthm.2022.12.026 -
Pacing and Clinical Electrophysiology :... Apr 2022COVID-19 has recently been associated with the development of bradyarrhythmias, although its mechanism is still unclear. We aim to summarize the existing evidence... (Review)
Review
COVID-19 has recently been associated with the development of bradyarrhythmias, although its mechanism is still unclear. We aim to summarize the existing evidence regarding bradyarrhythmia in COVID-19 and provide future directions for research. Following the PRISMA Extension for Scoping Reviews, we searched MEDLINE and EMBASE for all peer-reviewed articles using keywords including"Bradycardia," "atrioventricular block," and "COVID-19″ from their inception to October 13, 2021. Forty-three articles, including 11 observational studies and 59 cases from case reports and series, were included in the systematic review. Although some observational studies reported increased mortality in those with bradyarrhythmia and COVID-19, the lack of comparative groups and small sample sizes hinder the ability to draw definitive conclusions. Among 59 COVID-19 patients with bradycardia from case reports and series, bradycardia most often occurred in those with severe or critical COVID-19, and complete heart block occurred in the majority of cases despite preserved LVEF (55.9%). Pacemaker insertion was required in 76.3% of the patients, most of which were permanent implants (45.8%). This systematic review summarizes the current evidence and characteristics of bradyarrhythmia in patients with COVID-19. Further studies are critical to assess the reversibility of bradyarrhythmia in COVID-19 patients and to clarify potential therapeutic targets including the need for permanent pacing.
Topics: Atrioventricular Block; Bradycardia; COVID-19; Humans
PubMed: 35182433
DOI: 10.1111/pace.14466 -
Current Problems in Cardiology Jun 2023The pathophysiology of Bradycardia-Renal Failure-Atrioventricular Nodal Blockade-Shock-Hyperkalemia (BRASH) syndrome involves acute renal injury leading to ineffective... (Review)
Review
The pathophysiology of Bradycardia-Renal Failure-Atrioventricular Nodal Blockade-Shock-Hyperkalemia (BRASH) syndrome involves acute renal injury leading to ineffective clearance of AV nodal agents and potassium. Theoretically, the synergy between AV nodal blockade and hyperkalemic cardiac dysconduction results in circulatory collapse at less-than-expected doses of both. Our study aims to characterize the presentation of BRASH and provide clinical evidence of its risk factors. This systematic review comprises all reported cases of BRASH until February 2022. The average age and Charleston Comorbidity Index at presentation was 69 years and 3.8 respectively - hypertension (71%) was most prevalent followed by diabetes mellitus (48%) and chronic kidney disease (44%). The most frequent presenting complaint was fatigue or syncope (49%). More than half of all patients presented with nonsevere hyperkalemia (less than 6.5 mmol/L) and the mean serum creatinine was 3.6 mg/dL. Beta-blockers (75%) were the most commonly implicated nodal agents. Presenting mean arterial pressure was 62 mm Hg and heart rate averaged 36 bpm; junctional escape rhythm (50%), sinus bradycardia (17.1%), and complete heart block (12.9%) were generally observed on EKG. While most patients responded to medical management, 20% of patients required renal replacement therapy and 33% required transvenous or transcutaneous pacing. No patients underwent permanent pacemaker placement and the in-hospital mortality of BRASH was 5.7%. The diagnosis of BRASH requires a high index of suspicion; its synergistic pathology results in a dramatic clinical presentation that can be easily overlooked. As hypothesized, the degree of renal failure and hyperkalemia are not congruent with the presenting circulatory shock. The significant mortality of this syndrome presents an opportunity for intervention with timely recognition.
Topics: Humans; Hyperkalemia; Bradycardia; Shock; Atrioventricular Block; Arrhythmias, Cardiac; Renal Insufficiency
PubMed: 36842470
DOI: 10.1016/j.cpcardiol.2023.101663 -
Clinical Infectious Diseases : An... Oct 2014Lyme carditis is an uncommon manifestation of Lyme disease that most commonly involves some degree of atrioventricular conduction blockade. Third-degree conduction block... (Review)
Review
Lyme carditis is an uncommon manifestation of Lyme disease that most commonly involves some degree of atrioventricular conduction blockade. Third-degree conduction block is the most severe form and can be fatal if untreated. Systematic review of the medical literature identified 45 published cases of third-degree conduction block associated with Lyme carditis in the United States. Median patient age was 32 years, 84% of patients were male, and 39% required temporary pacing. Recognizing patient groups more likely to develop third-degree heart block associated with Lyme carditis is essential to providing prompt and appropriate therapy.
Topics: Adolescent; Adult; Aged; Aged, 80 and over; Child; Demography; Female; Heart Block; Humans; Lyme Disease; Male; Middle Aged; Myocarditis; United States; Young Adult
PubMed: 24879781
DOI: 10.1093/cid/ciu411 -
Anatolian Journal of Cardiology Mar 2023Over the past few years, smartwatches have become increasingly popular in the monitoring of arrhythmias. Although the detection of atrial fibrillation with smartwatches... (Review)
Review
BACKGROUND
Over the past few years, smartwatches have become increasingly popular in the monitoring of arrhythmias. Although the detection of atrial fibrillation with smartwatches has been the subject of various articles, there is no comprehensive research on the detection of arrhythmias other than atrial fibrillation. In this study, we included individual cases from the literature to identify the characteristics of patients with smartwatch-detected arrhythmias other than atrial fibrillation.
METHODS
PubMed, Embase, and SCOPUS were searched for case reports, case series, or cohort studies that reported individual participant-level data, until January 6, 2022. The following search string was used for each databases: ('Smart Watch' OR 'Apple Watch' OR 'Samsung Gear') AND ('Supraventricular Tachycardia' OR 'Cardiac Arrhythmia' OR 'Ventricular Tachycardia' OR 'Atrioventricular Nodal Reentry Tachycardia' OR 'Atrioventricular Reentrant Tachycardia' OR 'Heart Block' OR 'Atrial Flutter' OR 'Ectopic Atrial Tachycardia' OR 'Bradyarrhythmia').
RESULTS
A total of 52 studies from PubMed, 20 studies from Embase, and 200 studies from SCOPUS were identified. After screening, 18 articles were included. A total of 22 patients were obtained from 14 case reports or case series. Four cohort studies evaluating various arrhythmias were included. Arrhythmias, including ventricular tachycardia, atrial fibrillation, atrial flutter, atrioventricular nodal reentry tachycardia, atrioventricular reentrant tachycardia, second- or third-degree atrioventricular block, and sinus bradycardia, were detected with smartwatches.
CONCLUSIONS
Cardiac arrhythmias other than atrial fibrillation are also commonly detected with smartwatches. Smartwatches have an important potential besides traditional methods in the detection of arrhythmias and clinical practice.
Topics: Humans; Atrial Fibrillation; Bradycardia; Atrial Flutter; Tachycardia, Ventricular; Atrioventricular Block; Tachycardia, Atrioventricular Nodal Reentry
PubMed: 36856589
DOI: 10.14744/AnatolJCardiol.2023.2799 -
Heart Rhythm Aug 2021Syncope may be caused by intermittent complete heart block in patients with bundle branch block. Electrophysiology studies (EPS) testing for infra-Hisian heart block are... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Syncope may be caused by intermittent complete heart block in patients with bundle branch block. Electrophysiology studies (EPS) testing for infra-Hisian heart block are recommended by the European Society of Cardiology syncope guidelines on the basis of decades-old estimates of their negative predictive values (NPVs) for complete heart block.
OBJECTIVE
The aim of this study was to determine the NPV of EPS for complete heart block in patients with syncope and bundle branch block.
METHODS
We searched MEDLINE, EMBASE, Cochrane Central Register of Controlled Trials, and CINAHL without language restriction from database inception to October 2019 for Medical Subject Headings terms and keywords related to "syncope," "heart block," and "programmed electrical stimulation." A random effects meta-analysis was conducted with a primary outcome of the proportion of patients with a negative EPS who later presented with complete heart block, diagnosed with surface electrocardiographic (ECG) recordings vs continuous implantable cardiac monitor (ICM).
RESULTS
Ten reports contained 12 cohorts with 639 patients who met the inclusion criteria. The mean age was 69 ± 7 years; 35% ± 10% were women; and 85% of patients had bifascicular block. Seven cohorts recorded clinical outcomes with external ECG recordings, and 5 cohorts featured ICMs. The mean prespecified His-to-ventricle interval criterion was ≥70 ms. In studies featuring surface ECG recordings, there were 7% (95% confidence interval 7%-17%) patients who developed complete heart block compared with 29% (95% confidence interval 24%-35%) in the studies featuring ICM (P = .0001).
CONCLUSION
The NPV of EPS in patients with syncope and bundle branch block is 0.71, sufficiently low to question its use.
Topics: Atrioventricular Block; Cardiac Electrophysiology; Electrocardiography; Heart Conduction System; Humans; Syncope
PubMed: 33887450
DOI: 10.1016/j.hrthm.2021.04.010 -
Food and Chemical Toxicology : An... Dec 2015Mad honey, produced by honeybees from the nectars of Rhododendron genus (R. ponticum and R. luteum) flowers, is widely used in indigenous medicine, especially in the... (Review)
Review
Mad honey, produced by honeybees from the nectars of Rhododendron genus (R. ponticum and R. luteum) flowers, is widely used in indigenous medicine, especially in the treatment of hypertension and sexual dysfunction. However, the consumption of this honey can result in intoxication soon after. The diagnosis of honey poisoning and a full understanding of its treatment is important for both effective and immediate treatment, and also for the prevention of unnecessary costs. Upon the evaluation of approximately 34 years of case reports between 1981 and 2014, it was found that the cases of poisoning were more frequently reported in males (75.17%) and between the ages 41 to 65. The most common complaints related to honey poisoning were dizziness, nausea, presyncope and the ECG findings were: sinus bradycardia (79.58%), complete atrioventricular block (45.83%), atrioventricular block (30.91%), ST-segment elevation (22.63%), and nodal rhythm (11.27%), As a result of the evaluation of 1199 cases, it was found that no deaths were reported. The patients were most frequently treated with 0.5 mg atropine (37.79%), 1 mg atropine (49.73%), salin (iv fluid) (65.35%), and generally the patients were discharged within 24 h after recovery.
Topics: Atropine; Dizziness; Food Contamination; Heart Diseases; Honey; Humans; Rhododendron
PubMed: 26547022
DOI: 10.1016/j.fct.2015.10.018