Regional prevalence and clinical benefit of implantable cardioverter defibrillators in Brugada syndrome
TL;DR: Patients with BS benefit from ICD implantation, but less frequently than anticipated, and the problem of inappropriate ICD discharges is still of major concern.
About: This article is published in International Journal of Cardiology.The article was published on 2010-10-08. It has received 25 citations till now. The article focuses on the topics: Brugada syndrome & Implantable cardioverter-defibrillator.
Citations
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TL;DR: ICD implantation carries a significant risk of inappropriate shocks and inhospital and postdischarge complications in relatively young patients with inherited arrhythmia syndromes, and these data can be used to better inform patients and physicians about the expected risk of adverse ICD events and thereby facilitate shared decision making.
199 citations
TL;DR: Radiofrequency ablation of the right ventricular outflow tract epicardium of patients with BrS has recently been shown to reduce arrhythmia vulnerability and the electrocardiographic manifestation of the disease, presumably by destroying the cells with more prominent AP notch.
92 citations
Cites background from "Regional prevalence and clinical be..."
...Prevalence of BrS ECG in the general population varies significantly among continents, countries and ethnic groups.(1-22) The prevalence of the disease is highest in Southeast Asia where the syndrome is endemic, estimated to be greater than 5 per 10,000 inhabitants....
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TL;DR: A significant difference in the worldwide prevalence of BrS and Type-2/3 BrP was revealed and it was revealed that Brugada electrocardiogram patterns are highly prevalent in Southeast Asia.
Abstract: Background Brugada syndrome (BrS) is considered to be an inherited arrhythmic disease associated with fatal complications and premature sudden unexpected death. The prevalence of the Brugada electrocardiogram pattern (BrP) has been reported in several countries. Nonetheless, the specific worldwide prevalence of BrS has not been reported. Objective We estimated the worldwide prevalence of BrS and Type-2/3 BrP in general adult populations using a systematic review and meta-analysis of the existing literature. Methods We thoroughly searched MEDLINE and SCOPUS databases up to March 2017. Included studies were population-based electrocardiogram studies in which prevalence was presented or could be calculated from available data. Pooled prevalence by country/region and/or ethnicity was estimated using a random-effect model. Results Twenty-eight articles with atotal population of 369,068 were included in this study. The worldwide pooled prevalence of BrS was 0.5 per 1,000 [95% confidence interval (CI): 0.3-0.7]. The highest prevalence was reported in Southeast Asia (1.8 per 1,000, 95% CI: 0.5-6.6); the lowest was found in North Africa (0 per 1,000). BrS in Asians was nine times more common than in Caucasians and 36 times more common than in Hispanics. The worldwide pooled prevalence of Type-2/3 BrP was 6.1 per 1,000 (95% CI: 5.0-7.1). The highest prevalence was also reported in Southeast Asia (35.5 per 1,000, 95% CI: 17.1-53.9). Conclusions This study revealed a significant difference in the worldwide prevalence of BrS and Type-2/3 BrP. Brugada electrocardiogram patterns are highly prevalent in Southeast Asia.
75 citations
Journal Article•
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TL;DR: The current knowledge on the epidemiology, pathophysiology, genetics, clinical diagnosis, risk stratification and treatment of patients with BrS are summarized, with special emphasis on COVID-19 comorbidity.
Abstract: Brugada syndrome (BrS) is an inherited cardiac arrhythmia syndrome that causes a heightened risk for ventricular tachyarrhythmias and sudden cardiac death BrS is characterised by a coved ST-segment elevation in right precordial leads The prevalence is estimated to range between 1 in 5,000 to 1 in 2,000 in different populations, with the highest being in Southeast Asia and in males More than 18 genes associated with BrS have been discovered and recent evidence has suggested a complex polygenic mode of inheritance with multiple common and rare genetic variants acting in concert to produce the BrS phenotype Diagnosis of BrS in patients currently relies on presentation with a type-1 Brugada pattern on ECG either spontaneously or following a drug provocation test using a sodium channel blocker Risk assessment in patients diagnosed with BrS is controversial, especially with regard to the predictive value of programmed electrical stimulation and novel ECG parameters, such as QRS fragmentation The first line of BrS therapy remains an implantable cardioverter defibrillator (ICD), although radiofrequency catheter ablation has been shown to be an effective option in patients with contraindications for an ICD True BrS can be unmasked on ECG in susceptible individuals by monitoring factors such as fever, and this has been recently evident in several patients infected with the 2019 novel coronavirus (COVID-19) Aggressive antipyretic therapy and regular ECG monitoring until fever resolves are current recommendations to help reduce the arrhythmic risk in these COVID-19 patients In this review, we summarise the current knowledge on the epidemiology, pathophysiology, genetics, clinical diagnosis, risk stratification and treatment of patients with BrS, with special emphasis on COVID-19 comorbidity
47 citations
TL;DR: The prevalence of BrEP is about 0.4% around the world with different prevalence in region and sex, and this study shows that BrEP may not be taken as a predictor of all-cause death and cardiac death.
40 citations
References
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TL;DR: Common clinical and ECG features define a distinct syndrome in this group of patients with recurrent episodes of aborted sudden death unexplainable by currently known diseases, not explainable by electrolyte disturbances, ischemia or structural heart disease.
3,075 citations
"Regional prevalence and clinical be..." refers background in this paper
...Brugada syndrome is characterized by a right bundle-branch block with a “coved-type” ST-segment elevationwith negative T-waves in at least one precordial lead in V1–V3, which is associated with an increased risk of syncope and sudden cardiac death (SCD) caused by ventricular fibrillation or by hemodynamic relevant ventricular tachycardia in the absence of structural heart disease [1]....
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...≥2 mm) followed by a negative T-wave in at least one precordial lead in V1–V3 [1], either at rest or after administration of a class I anti-arrhythmic drug....
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TL;DR: Principles of Ethical Publishing in the International Journal of Cardiology: that the corresponding author has the approval of all other listed authors for the submission and publication of all versions of the manuscript.
2,159 citations
"Regional prevalence and clinical be..." refers methods in this paper
...The authors of this manuscript have certified that they comply with the Principles of Ethical Publishing in the International Journal of Cardiology [38]....
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TL;DR: The present report elaborates further on the diagnostic criteria and examines risk stratification schemes and device and pharmacological approaches to therapy on the basis of the available clinical and basic science data.
Abstract: Since its introduction as a clinical entity in 1992, the Brugada syndrome has progressed from being a rare disease to one that is second only to automobile accidents as a cause of death among young adults in some countries Electrocardiographically characterized by a distinct ST-segment elevation in the right precordial leads, the syndrome is associated with a high risk for sudden cardiac death in young and otherwise healthy adults, and less frequently in infants and children Patients with a spontaneously appearing Brugada ECG have a high risk for sudden arrhythmic death secondary to ventricular tachycardia/fibrillation The ECG manifestations of Brugada syndrome are often dynamic or concealed and may be unmasked or modulated by sodium channel blockers, a febrile state, vagotonic agents, α-adrenergic agonists, β-adrenergic blockers, tricyclic or tetracyclic antidepressants, a combination of glucose and insulin, hypo- and hyperkalemia, hypercalcemia, and alcohol and cocaine toxicity In recent years, an exponential rise in the number of reported cases and a striking proliferation of articles defining the clinical, genetic, cellular, ionic, and molecular aspects of the disease have occurred The report of the first consensus conference, published in 2002, focused on diagnostic criteria The present report, which emanated from the second consensus conference held in September 2003, elaborates further on the diagnostic criteria and examines risk stratification schemes and device and pharmacological approaches to therapy on the basis of the available clinical and basic science data
1,730 citations
TL;DR: The information on the natural history of patients obtained in this study allowed elaboration of a risk-stratification scheme to quantify the risk for sudden cardiac death and to target the use of the implantable cardioverter-defibrillator.
Abstract: Background— Treatment of patients with Brugada syndrome is complicated by the incomplete information on the natural history of the disease related to the small number of cases reported. Furthermore, the value of programmed electrical stimulation (PES) for risk stratification is highly debated. The objective of this study was to search for novel parameters to identify patients at risk of sudden death. Methods and Results— Clinical data were collected for 200 patients (152 men, 48 women; age, 41±18 years) and stored in a dedicated database. Genetic analysis was performed, and mutations on the SCN5A gene were identified in 28 of 130 probands and in 56 of 121 family members. The life-table method of Kaplan-Meier used to define the cardiac arrest-free interval in patients undergoing PES failed to demonstrate an association between PES inducibility and spontaneous occurrence of ventricular fibrillation. Multivariate Cox regression analysis showed that after adjusting for sex, family history of sudden death, and...
1,031 citations
"Regional prevalence and clinical be..." refers methods in this paper
...In one centre, baseline EP studies were performed for risk stratification after ECGbased diagnosis of BS in all patients giving their informed consent [11,12]....
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TL;DR: A similar incidence of potentially lethal arrhythmias in patients displaying transient versus persistent ST-segment elevation and right bundle branch block is demonstrated, as well as the effectiveness of sodium channel blockers to unmask the syndrome and, thus, identify patients at risk.
Abstract: Background—A mutation in the cardiac sodium channel gene (SCN5A) has been described in patients with the syndrome of right bundle branch block, ST-segment elevation in leads V1 to V3, and sudden de...
746 citations