163,358 research outputs found
Treatment of Electrical Storm with Amiodarone in Brugada Syndrome- an Unexpected Protective Effect
No full textWe are reporting on a 53 year old man with proven Brugada syndrome and ICD implantation for resuscitation in context of polymorphic VT. After recurrent arrhythmia he was treated with Amiodarone. This showed to have a protective effect despite various reports suggesting avoiding Amiodarone in Brugada syndrome
Proposed diagnostic criteria for the Brugada syndrome - Consensus report
No full textAsyndrome characterized by ST-segment elevation in right precordial leads (V1 to V3) that is unrelated to ischemia, electrolyte disturbances, or obvious structural heart disease was reported as early as 1953,1 but was first described as a distinct clinical entity associated with a high risk of sudden cardiac death in 1992.The Brugada syndrome is a familial disease that displays an autosomal dominant mode of transmission, with incomplete penetrance and an incidence ranging between 5 and 66 per 10 000. In regions of Southeast Asia where it is endemic, the clinical presentation of Brugada syndrome is distinguished by a male predominance (8:1 ratio of male:female) and the appearance of arrhythmic events at an average age of 40 years (range: 1 to 77 years).Although a number of candidate genes are considered plausible, thus far the syndrome has been linked only to mutations in SCN5A, the gene encoding for the α subunit of the sodium channel
Proposed diagnostic criteria for the Brugada syndrome: consensus report
No full textWhat are the proper diagnostic criteria for identifying Brugada syndrome? A definitive answer to this question has been out of reach and is the reason for the establishment of a special task force of Arrhythmia Working Group of the European Society of Cardiology that met from August 31 to September 1, 2000. This report is the consensus statement from that meeting. The diagnostic criteria described herein are based on the currently available clinical data and state-of-the-art understanding of the molecular and cellular mechanisms underlying Brugada syndrome
Brugada syndrome - Report of the second consensus conference
No full textSince 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, alpha-adrenergic agonists, beta-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
Proposed diagnostic criteria for the Brugada syndrome
No full textWlat are the proper diagnostic cliteria to be used in
identifying Brugada syndrome? A definitive answer to
this question has been out ofreach and is the reason for
the establishment of a special Arrhythrn:a Working
Group of the European Society of Cardiology that met
on 31 August-l September 2000. This report is the
consensus document emanating from that meeting. The
diagnostic criteria described hereiu are based on the
currentiy available ciinical data and state-of-the-art
understanding of the molecuiar and cellular mechanisms
underlying Brugada syndrome. The proposed criteria
must be considered a work-in-progress that will be
fine-tuned as confirmatoqr data from future molecular
studies and prospective trials become available
Brugada-type Electrocardiographic Pattern Induced by Fever
No full textST-segment elevation in Brugada syndrome is caused by a shift in the ionic current balance and the creation of a voltage gradient between the epicardium and the endocardium. This ionic mechanism have been shown to be temperature dependent. We describe a 33-year-old man who presented with fever with the dynamic electrocardiographic changes similar to the Brugada syndrome. These electrocardiographic anomalies disappeared when the temperature returned to normal
Brugada syndrome - Report of the second consensus conference - Endorsed by the Heart Rhythm Society and the European Heart Rhythm Association
No full textAbstract
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, alpha-adrenergic agonists, beta-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
Cardiac autonomic control in Brugada syndrome patients during sleep : the effects of sleep disordered breathing
No full textAims Brugada syndrome is characterized by typical ECG features, ventricular arrhythmias and sudden cardiac death (SCD), more frequent during nighttime. Autonomic cardiovascular control has been implicated in triggering the ventricular arrhythmias. Sleep-disordered breathing (SDB) elicits marked autonomic changes during sleep and is also associated with an increased risk of nighttime SCD. Brugada patients may have a higher likelihood of SDB compared to controls. However, no data are available on cardiac autonomic control in Brugada patients, particularly with regard to the comorbidity of SDB. Methods We evaluated autonomic cardiovascular control in Brugada patients with SDB (BRU-SDB, n = 9), without SDB (BRU, n = 9), in controls (CON, n = 8) and in non-Brugada patients with SDB (n = 6), during wakefulness and sleep (N2, N3 and REM). Linear spectral and entropy-derived measures of heart rate variability (HRV) were performed during apnea-free stable breathing epochs. Results Total HRV was attenuated in BRU-SDB compared to CON and BRU. During N2 and REM, in BRU-SDB patients sympathetic modulation decreased compared to BRU and CON, while during REM, they showed an increased parasympathetic modulation, compared to the other two groups. BRU-SDB and SDB were similar in terms of spectral components. Entropy-derived indices showed preserved dynamic changes in Brugada patients compared to controls through the different sleep stages. Conclusion Brugada syndrome per se does not appear associated with an altered autonomic cardiovascular control during wakefulness and sleep. The comorbidity with SDB may contribute to disrupted autonomic cardiovascular regulation during sleep, possibly predisposing to the increased likelihood of sleep-related ventricular tachyarrhythmias and SCD
Integration of “Omics” Strategies for Biomarkers Discovery and for the Elucidation of Molecular Mechanisms Underlying Brugada Syndrome
No full textPurpose: The Brugada syndrome (BrS) is a severe inherited cardiac disorder. Given the high genetic and phenotypic heterogeneity of this disease, three different “omics” approaches are integrated in a synergic way to elucidate the molecular mechanisms underlying the pathophysiology of BrS as well as for identifying reliable diagnostic/prognostic markers. Experimental design: The profiling of plasma Proteome and MiRNome is perfomed in a cohort of Brugada patients that were preliminary subjected to genomic analysis to assess a peculiar gene mutation profile. Results: The integrated analysis of “omics” data unveiled a cooperative activity of mutated genes, deregulated miRNAs and proteins in orchestrating transcriptional and post-translational events that are critical determining factors for the development of the Brugada pattern. Conclusions and clinical relevance: This study provides the basis to shed light on the specific molecular fingerprints underlying BrS development and to gain further insights on the pathogenesis of this life-threatening cardiac disease
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