-
Frontiers in Cardiovascular Medicine 2023To perform a meta-analysis to discover the performance of ML algorithms in identifying Congenital long QT syndrome (LQTS). (Review)
Review
INTRODUCTION
To perform a meta-analysis to discover the performance of ML algorithms in identifying Congenital long QT syndrome (LQTS).
METHODS
The searched databases included Cochrane, EMBASE, Web of Science, and PubMed. Our study considered all English-language studies that reported the detection of LQTS using ML algorithms. Quality was assessed using QUADAS-2 and QUADAS-AI tools. The bivariate mixed effects models were used in our study. Based on genotype data for LQTS, we performed a subgroup analysis.
RESULTS
Out of 536 studies, 8 met all inclusion criteria. The pooled area under the receiving operating curve (SAUROC) for detecting LQTS was 0.95 (95% CI: 0.31-1.00); sensitivity was 0.87 (95% CI: 0.83-0.90), and specificity was 0.91 (95% CI: 0.88-0.93). Additionally, diagnostic odd ratio (DOR) was 65 (95% CI: 39-109). The positive likelihood ratio (PLR) was 9.3 (95% CI: 7.0-12.3) and the negative likelihood ratio (NLR) was 0.14 (95% CI: 0.11-0.20), with very low heterogeneity (= 16%).
DISCUSSION
We found that machine learning can be used to detect features of rare cardiovascular disease like LQTS, thus increasing our understanding of intelligent interpretation of ECG. To improve ML performance in the classification of LQTS subtypes, further research is required.
SYSTEMATIC REVIEW REGISTRATION
identifier PROSPERO CRD42022360122.
PubMed: 37351282
DOI: 10.3389/fcvm.2023.1172451 -
Heart & Lung : the Journal of Critical... 2023The potential benefit of implantable cardioverter-defibrillator (ICD) therapy in individuals with inherited arrhythmia syndromes is well known. However, it is not... (Review)
Review
BACKGROUND
The potential benefit of implantable cardioverter-defibrillator (ICD) therapy in individuals with inherited arrhythmia syndromes is well known. However, it is not deprived of morbidity, in the form of inappropriate therapies and other ICD-related complications.
OBJECTIVE
The aim of this systematic review is to estimate the rate of appropriate and inappropriate therapy, as well as other ICD-related complications, in individuals with inherited arrhythmia syndromes.
METHODS
A systematic review was performed, regarding appropriate and inappropriate therapy, and other ICD-related complications, in individuals with inherited arrhythmia syndromes (Brugada Syndrome, Catecholaminergic Polymorphic Ventricular Tachycardia, Early Repolarization Syndrome, Long QT Syndrome and Short QT syndrome). Studies were identified by searching published papers in PubMed and Embase up to August 23rd, 2022.
RESULTS
From data gathered of 36 studies, with a total of 2750 individuals, during a mean follow-up time of 69 months, appropriate therapies occurred in 21% of the individuals and inappropriate therapies in 20% of the individuals. Concerning the other ICD-related complications, 456 complications were observed, amongst 2084 individuals (22%), with the most frequent being lead malfunction (46%), followed by infectious complications (13%).
CONCLUSIONS
ICD-related complications are not uncommon, especially when one considers the exposure time of young individuals. The incidence of inappropriate therapies was 20%, although lower rates were reported in recent publications. S-ICD is an effective alternative to transvenous ICD for sudden death prevention. The decision to implant an ICD should be individualized, taking into account the risk profile of each patient, as well as the possibility of complications.
Topics: Humans; Defibrillators, Implantable; Electrocardiography; Arrhythmias, Cardiac; Tachycardia, Ventricular; Long QT Syndrome; Death, Sudden, Cardiac; Treatment Outcome
PubMed: 36863123
DOI: 10.1016/j.hrtlng.2023.02.012 -
Archives of Cardiovascular Diseases Feb 2023Conflicting findings have described the association between prolonged heart rate-corrected QT interval (QTc) and cardiovascular disease. (Meta-Analysis)
Meta-Analysis
BACKGROUND
Conflicting findings have described the association between prolonged heart rate-corrected QT interval (QTc) and cardiovascular disease.
AIMS
To identify articles investigating the association between QTc and cardiovascular disease morbidity and mortality, and to summarize the available evidence for the general and type 2 diabetes populations.
METHODS
A systematic search was performed in PubMed and Embase in May 2022 to identify studies that investigated the association between QTc prolongation and cardiovascular disease in both the general and type 2 diabetes populations. Screening, full-text assessment, data extraction and risk of bias assessment were performed independently by two reviewers. Effect estimates were pooled across studies using random-effect models.
RESULTS
Of the 59 studies included, 36 qualified for meta-analysis. Meta-analysis of the general population studies showed a significant association for: overall cardiovascular disease (fatal and non-fatal) (hazard ratio [HR] 1.68, 95% confidence interval [CI] 1.33-2.12; I=69%); coronary heart disease (fatal and non-fatal) in women (HR 1.27, 95% CI 1.08-1.50; I=38%; coronary heart disease (fatal and non-fatal) in men (HR 2.07, 95% CI 1.26-3.39; I=78%); stroke (HR 1.59, 95% CI 1.29-1.96; I=45%); sudden cardiac death (HR 1.60, 95% CI 1.14-2.25; I=68%); and atrial fibrillation (HR 1.55, 95% CI 1.31-1.83; I=0.0%). No significant association was found for cardiovascular disease in the type 2 diabetes population.
CONCLUSION
QTc prolongation was associated with risk of cardiovascular disease in the general population, but not in the type 2 diabetes population.
Topics: Male; Humans; Female; Cardiovascular Diseases; Risk Factors; Heart Rate; Diabetes Mellitus, Type 2; Long QT Syndrome; Atrial Fibrillation; Coronary Disease; Electrocardiography
PubMed: 36690508
DOI: 10.1016/j.acvd.2022.11.007 -
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 -
Frontiers in Cardiovascular Medicine 2022Mutations in the gene-encoding for the major Ca channel of the heart-may exhibit a variety of clinical manifestations. These include typical or atypical Timothy...
Geno- and phenotypic characteristics and clinical outcomes of gene mutation associated Timothy syndrome, "cardiac only" Timothy syndrome and isolated long QT syndrome 8: A systematic review.
BACKGROUND
Mutations in the gene-encoding for the major Ca channel of the heart-may exhibit a variety of clinical manifestations. These include typical or atypical Timothy syndromes (TS) which are associated with multiple organ manifestations, and cardiac involvement in form of malignant arrhythmias, QTc prolongation, or AV block. "Cardiac only" Timothy syndrome (COTS) shows no extracardiac manifestation, whereas some gene mutations are associated with QTc prolongation alone (isolated long QT syndrome 8, LQT8).
METHODS
A systematic search of the literature reporting cases of gene mutation associated syndromes, including TS, COTS and isolated LQT8 major databases published from 2004 through 2019 was performed. Detailed patient-level phenotypic and genotypic characteristics, as well as long-term outcome measures were collected and compared between pre-specified patient groups, defined both on phenotype and genotype.
RESULTS
A total of 59 TS, 6 COTS, and 20 isolated LQT8 index cases were identified. Apart of syndactyly or baldness, there were no major differences regarding clinical manifestations or outcome measures between TS subtypes, either defining TS subtypes on the genotype or based on the phenotype. Both subtypes were characterized by an extreme degree of QTc prolongation (median ≥600 ms) which were reflected in high major adverse cardiac event rate. On the other hand, there were marked differences between TS, COTS, and isolated LQT8. Timothy syndrome was characterized by a much earlier disease onset, much more pronounced QTc prolongation and much higher mortality rate than COTS or isolated LQT8. Similar differences were observed comparing exon 8/8A vs. non-exon 8/8A mutation carriers. TS showed a high degree of genetic homogeneity, as the p.Gly406Arg mutation either in exon 8 or exon 8A alone was responsible for 70% of the cases.
CONCLUSIONS
Clinical phenotypes associated with mutations in the gene show important clinical differences. Timothy syndrome is associated with the most severe clinical phenotype and with the highest risk of morbidity and mortality. However, distinguishing TS subtypes, in any form, are not supported by our data.
SYSTEMATIC REVIEW REGISTRATION
[https://www.crd.york.ac.uk/prospero/], identifier [CRD42020184737].
PubMed: 36523353
DOI: 10.3389/fcvm.2022.1021009 -
Annals of Noninvasive Electrocardiology... Jan 2023QTc prolongation is key in diagnosing long QT syndrome (LQTS), however 25%-50% with congenital LQTS (cLQTS) demonstrate a normal resting QTc. T wave morphology (TWM) can... (Review)
Review
INTRODUCTION
QTc prolongation is key in diagnosing long QT syndrome (LQTS), however 25%-50% with congenital LQTS (cLQTS) demonstrate a normal resting QTc. T wave morphology (TWM) can distinguish cLQTS subtypes but its role in acquired LQTS (aLQTS) is unclear.
METHODS
Electronic databases were searched using the terms "LQTS," "long QT syndrome," "QTc prolongation," "prolonged QT," and "T wave," "T wave morphology," "T wave pattern," "T wave biomarkers." Whole text articles assessing TWM, independent of QTc, were included.
RESULTS
Seventeen studies met criteria. TWM measurements included T-wave amplitude, duration, magnitude, Tpeak-Tend, QTpeak, left and right slope, center of gravity (COG), sigmoidal and polynomial classifiers, repolarizing integral, morphology combination score (MCS) and principal component analysis (PCA); and vectorcardiographic biomarkers. cLQTS were distinguished from controls by sigmoidal and polynomial classifiers, MCS, QTpeak, Tpeak-Tend, left slope; and COG x axis. MCS detected aLQTS more significantly than QTc. Flatness, asymmetry and notching, J-Tpeak; and Tpeak-Tend correlated with QTc in aLQTS. Multichannel block in aLQTS was identified by early repolarization (ERD ) and late repolarization (LRD ), with ERD reflecting hERG-specific blockade. Cardiac events were predicted in cLQTS by T wave flatness, notching, and inversion in leads II and V , left slope in lead V ; and COG last 25% in lead I. T wave right slope in lead I and T-roundness achieved this in aLQTS.
CONCLUSION
Numerous TWM biomarkers which supplement QTc assessment were identified. Their diagnostic capabilities include differentiation of genotypes, identification of concealed LQTS, differentiating aLQTS from cLQTS; and determining multichannel versus hERG channel blockade.
Topics: Humans; Electrocardiography; Long QT Syndrome; Genotype; Biomarkers
PubMed: 36345173
DOI: 10.1111/anec.13015 -
Cureus Aug 2022Torsades de Pointes (TdP) is a rare form of tachyarrhythmia which can potentially be fatal due to its tendency to degenerate into ventricular fibrillation. It is... (Review)
Review
Torsades de Pointes (TdP) is a rare form of tachyarrhythmia which can potentially be fatal due to its tendency to degenerate into ventricular fibrillation. It is described as a polymorphic ventricular tachycardia characterized by twisting of the QRS complexes around the electrocardiogram (ECG) baseline in patients with a prolonged QT interval. Prolonged QT interval is known as long QT syndrome. Torsades de Poccurs most commonly in patients with an extended QT interval duration, and even though monitoring an ECG can assist in its prevention, there is no defined duration of a QT interval that can lead to an increased risk of Torsades de Pointes. So, it is hard to determine what QT interval constitutes enough risk for Torsades de Pointes to require intervention. The QT interval duration also depends on other factors, namely heart rate (HR) and other factors such as drugs, congenital diseases, and a combination of both. In this study, we considered various causes of QT prolongation but mainly focused on congenital diseases, drugs, or perioperative risk of QT prolongation and the correlation with the risk of impending TdP. By following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines and researching studies on various databases, namely PubMed, Science Direct, Medline, and CiNii we were able to find various systematic reviews and articles showing the association between prolonged QT interval and its degeneration into TdP. This review encourages further research into this topic to understand the implications of QT prolongation and how it can help save the lives of patients with known long QT syndrome, or those on QT prolonging drugs with simple ECG monitoring and treatment for the respective cause.
PubMed: 36110477
DOI: 10.7759/cureus.27833 -
Journal of Pharmacy Practice Feb 2024Drug-drug interactions (DDIs) are considered an emerging threat to the patients if undetected. DDIs can prolong QT interval, leading to fatal ventricular arrhythmia.... (Meta-Analysis)
Meta-Analysis Review
Drug-drug interactions (DDIs) are considered an emerging threat to the patients if undetected. DDIs can prolong QT interval, leading to fatal ventricular arrhythmia. Antipsychotics and antidepressants prescribed commonly to psychiatric patients have the propensity to prolong QT interval and can precipitate Torsades de pointes (TdP). This review aimed to summarize the prevalence of QT interval prolonging DDIs in psychiatric patients. This meta-analysis was carried out following the MOOSE (Meta-analysis of Observational Studies in Epidemiology) statement. Databases like Pubmed/MEDLINE, Google Scholar and Research gate were scanned for English language papers. Indexed terms from Medical Subject (MeSH) and other search terms for "QT prolongation", "Drug interactions", and "Psychiatry" were used to identify the articles. All published articles available until the day of the collection were considered. Outcome measures were analyzed with meta package in R language. A total of 5 studies were eligible for inclusion. From the included studies, QT-prolonging DDIs were found in 14806 patients out of 30122 patients. The prevalence of QT-prolonging DDIs in psychiatric patients was found to be 42% (95% confidence interval: 21%, 66%). The factors associated with potential drug-drug interactions were related to patient characteristics such as polypharmacy, age and comorbid disease. This review concluded that psychiatric patients were prescribed the drugs/drug combinations which can prolong QT interval and can cause adverse effects on the cardiovascular system. Hence, it is important to implement precautionary safety interventions, be vigilant and prevent QT prolongation and adverse cardiac effects in clinical practice.
Topics: Humans; Prevalence; Long QT Syndrome; Risk Factors; Drug Interactions; Drug-Related Side Effects and Adverse Reactions; Psychiatry; Observational Studies as Topic
PubMed: 35968552
DOI: 10.1177/08971900221121371 -
Drug Safety Oct 2022Electrocardiogram (ECG) monitoring is an important tool to detect and mitigate the risk of potentially fatal drug-induced QT prolongation and remains fundamental in... (Meta-Analysis)
Meta-Analysis
INTRODUCTION
Electrocardiogram (ECG) monitoring is an important tool to detect and mitigate the risk of potentially fatal drug-induced QT prolongation and remains fundamental in supporting the quality use of high-risk QT interval prolonging medicines.
OBJECTIVE
The aim of this systematic review was to determine the prevalence of baseline and/or follow-up ECG use in adult patients taking high-risk QT interval prolonging medicines in clinical practice.
METHODS
CINAHL, Cochrane Library, Embase, PubMed, EThOS, OpenGrey and Proquest were searched for studies in adults that reported ECG use at baseline and/or at follow-up in relation to the initiation of a high-risk QT interval prolonging medicine in any clinical setting; either hospital or non-hospital. Two reviewers independently assessed the methodological quality of included studies. Proportional meta-analysis was conducted with all studies reporting baseline ECG use, before medicine initiation, and follow-up ECG use, within 30 days of medicine initiation.
RESULTS
There was variability in baseline ECG use according to the practice setting. The prevalence of baseline ECG use for high-risk QT interval prolonging medicines was moderate to high in the hospital setting at 75.1% (95% CI 64.3-84.5); however, the prevalence of baseline ECG use was low in the non-hospital setting at 33.7% (95% CI 25.8-42.2). The prevalence of follow-up ECG use was low to moderate in the hospital setting at 39.2% (95% CI 28.2-50.8) and could not be determined for the non-hospital setting.
CONCLUSIONS
The use of ECG monitoring for high-risk QT interval prolonging medicines is strongly influenced by the clinical practice setting. Baseline ECG use occurs more in the hospital setting in comparison to the non-hospital setting. There is lower use of follow-up ECG in comparison to baseline ECG.
Topics: Adult; Electrocardiography; Humans; Long QT Syndrome; Prevalence; Risk Factors
PubMed: 35947343
DOI: 10.1007/s40264-022-01215-x -
Journal of the American Heart... Jul 2022Background Diagnosis is particularly challenging in concealed or asymptomatic long QT syndrome (LQTS). Provocative testing, unmasking the characterization of LQTS, is a... (Meta-Analysis)
Meta-Analysis Review
Background Diagnosis is particularly challenging in concealed or asymptomatic long QT syndrome (LQTS). Provocative testing, unmasking the characterization of LQTS, is a promising alternative method for the diagnosis of LQTS, but without uniform standards. Methods and Results A comprehensive search was conducted in PubMed, Embase, and the Cochrane Library through October 14, 2021. The fixed effects model was used to assess the effect of the provocative testing on QTc interval. A total of 22 studies with 1137 patients with LQTS were included. At baseline, QTc interval was 40 ms longer in patients with LQTS than in controls (mean difference [MD], 40.54 [95% CI, 37.43-43.65]; <0.001). Compared with the control group, patients with LQTS had 28 ms longer ΔQTc upon standing (MD, 28.82 [95% CI, 23.05-34.58]; <0.001), nearly 30 ms longer both at peak exercise (MD, 27.31 [95% CI, 21.51-33.11]; <0.001) and recovery 4 to 5 minutes (MD, 29.85 [95% CI, 24.36-35.35]; <0.001). With epinephrine infusion, QTc interval was prolonged both in controls and patients with QTS, most obviously in LQT1 (MD, 68.26 [95% CI, 58.91-77.60]; <0.001) and LQT2 (MD, 60.17 [95% CI, 50.18-70.16]; <0.001). Subgroup analysis showed QTc interval response to abrupt stand testing and exercise testing varied between LQT1, LQT2, and LQT3, named Type Ⅰ, Type Ⅱ, and Type Ⅲ. Conclusions QTc trend Type Ⅰ and Type Ⅲ during abrupt stand testing and exercise testing can be used to propose a prospective evaluation of LQT1 and LQT3, respectively. Type Ⅱ QTc trend combined epinephrine infusion testing could distinguish LQT2 from control. A preliminary diagnostic workflow was proposed but deserves further evaluation.
Topics: Electrocardiography; Epinephrine; Exercise Test; Genotype; Humans; Long QT Syndrome
PubMed: 35861842
DOI: 10.1161/JAHA.122.025246