Clinical aspects and physiopathology of Brugada syndrome: review of current concepts

2006 ◽  
Vol 84 (8-9) ◽  
pp. 795-802 ◽  
Author(s):  
E. Herbert ◽  
M. Chahine
Keyword(s):  
Brugada Syndrome ◽  
Structural Heart Disease ◽  
Polymorphic Ventricular Tachycardia ◽  
Clinical Aspects ◽  
Gene Encoding ◽  
Bundle Branch Block ◽  
St Segment Elevation ◽  
Disease Mutations ◽  
St Segment ◽  
Cardiac Sodium Channel

Brugada syndrome (BS) is an inherited cardiac disorder characterized by typical electrocardiographic patterns of ST segment elevation in the precordial leads, right bundle branch block, fast polymorphic ventricular tachycardia in patients without any structural heart disease, and a high risk of sudden cardiac death. The incidence of BS is high in male vs. female (i.e., 8–10/1: male/female). The disorder is caused by mutations in the SCN5A gene encoding Nav1.5, the cardiac sodium channel, which is the only gene in which mutations were found to cause the disease. Mutations in SCN5A associated with the BS phenotype usually result in a loss of channel function by a reduction in Na+ currents. We review the clinical aspects, risk stratification, and therapeutic management of this important syndrome.

scholarly journals Intermittent Brugada Syndrome Presenting with Syncope in an Adult Female

2014 ◽  
Vol 2014 ◽  
pp. 1-3
Author(s):  
Patricia Chavez ◽  
Daniel Bamira ◽  
Abel Casso Dominguez ◽  
Akshai Bhandary ◽  
Eyal Herzog
Keyword(s):  
Brugada Syndrome ◽  
Fatal Outcome ◽  
Structural Heart Disease ◽  
Bundle Branch Block ◽  
St Segment Elevation ◽  
T Wave ◽  
Shockable Rhythm ◽  
St Segment ◽  
History Of

Background.Brugada syndrome accounts for 4–12% of all sudden deaths worldwide and at least 20% of sudden deaths in patients with structurally normal hearts.Case Report.A 48-year-old female presented to the emergency department after two witnessed syncopal episodes. While awaiting discharge had a third collapse followed by cardiac arrest with shockable rhythm. Initial electrocardiogram showed wide QRS complex with left axis deviation, ST-segment elevation of <1 mm in V1 and V2, and flattening of T waves in V1. The angiogram did not demonstrate obstructive coronary disease. The electrocardiogram obtained two days after these events showed a right bundle branch block with ST-segment elevation of >2 mm followed by a negative T wave with no isoelectric separation, suggestive of spontaneous intermittent Brugada type 1 pattern. Cardiac magnetic resonance imaging demonstrated neither structural heart disease nor abnormal myocardium. After placement of an implantable cardioverter defibrillator the patient was discharged. Why should an emergency physician be aware of this? Brugada syndrome is an infrequently encountered clinical entity which may have a fatal outcome. This syndrome primarily presents with syncope. It should be considered as a component of differential diagnosis in patients with family history of syncope and sudden cardiac death.

scholarly journals Experimental Models of Brugada syndrome

2019 ◽  
Vol 20 (9) ◽  
pp. 2123 ◽  
Author(s):  
Sendfeld ◽  
Selga ◽  
Scornik ◽  
Pérez ◽  
Mills ◽  
...  
Keyword(s):  
Brugada Syndrome ◽  
Experimental Models ◽  
Model Systems ◽  
Patient Specific ◽  
Canine Heart ◽  
Stem Cell Technology ◽  
St Segment Elevation ◽  
St Segment ◽  
Cardiac Sodium Channel ◽  
The Right

Brugada syndrome is an inherited, rare cardiac arrhythmogenic disease, associated with sudden cardiac death. It accounts for up to 20% of sudden deaths in patients without structural cardiac abnormalities. The majority of mutations involve the cardiac sodium channel gene SCN5A and give rise to classical abnormal electrocardiogram with ST segment elevation in the right precordial leads V1 to V3 and a predisposition to ventricular fibrillation. The pathophysiological mechanisms of Brugada syndrome have been investigated using model systems including transgenic mice, canine heart preparations, and expression systems to study different SCN5A mutations. These models have a number of limitations. The recent development of pluripotent stem cell technology creates an opportunity to study cardiomyocytes derived from patients and healthy individuals. To date, only a few studies have been done using Brugada syndrome patient-specific iPS-CM, which have provided novel insights into the mechanisms and pathophysiology of Brugada syndrome. This review provides an evaluation of the strengths and limitations of each of these model systems and summarizes the key mechanisms that have been identified to date.

scholarly journals Fever Associated with Gastrointestinal Shigellosis Unmasks Probable Brugada Syndrome

2009 ◽  
Vol 2009 ◽  
pp. 1-3 ◽  
Author(s):  
John N. Makaryus ◽  
Jennifer Verbsky ◽  
Scott Schwarz ◽  
David Slotwiner
Keyword(s):  
Brugada Syndrome ◽  
Ventricular Arrhythmias ◽  
Structural Heart Disease ◽  
Underlying Mechanism ◽  
Temperature Dependent ◽  
Bundle Branch Block ◽  
St Segment ◽  
Electrocardiographic Changes ◽  
Clinically Significant ◽  
The Right

Since it was first described approximately 15 years ago, the Brugada Syndrome has spurred a significant quantity of interest in its underlying mechanism and physiology. The Brugada electrocardiographic pattern is characterized by right bundle branch block morphology and ST segment elevations in the right precordial leads with an absence of identifiable underlying structural heart disease. The syndrome is clinically significant since these patients are at a higher risk of developing malignant ventricular arrhythmias. One of the mechanisms behind the disorder involves mutations in specific myocardial sodium channels. Furthermore, these electrocardiographic changes appear to be temperature dependent. We present the case of a 35-year-old male who presented with intestinal Shigellosis and was also found to have Brugada-type electrocardiographic changes on ECG. The electrocardiographic changes that were present when the patient was admitted and febrile resolved following antibiotic therapy and defervescence.

scholarly journals General Anesthetic Management for Patient with Brugada Syndrome: A Case Report

2020 ◽  
Vol 8 (6) ◽  
Author(s):  
Ji Won Bak ◽  
Se Jin Kim ◽  
Yeon Ji Roh ◽  
So Yeon Cho ◽  
Seongsik Kang
Keyword(s):  
Case Report ◽  
Ventricular Arrhythmia ◽  
General Anesthesia ◽  
Brugada Syndrome ◽  
Anesthetic Management ◽  
General Anesthetic ◽  
Bundle Branch Block ◽  
St Segment Elevation ◽  
St Segment ◽  
Block Pattern

Brugada syndrome is an arrhythmogenic cardiopathy characterized by electrocardiography (ECG) pattern of the presence of an atypical right bundle branch block pattern with ST segment elevation in the precordial leads (V1-V3). It is sometimes associated with sudden deaths caused by ventricular arrhythmia. Here, we are reporting a case of a 43-year-old male patient with Brugada syndrome who underwent a tonsillectomy under general anesthesia without any complications.

scholarly journals Mutations in MYBPC3 and MYH7 in Association with Brugada Type 1 ECG Pattern: Overlap between Brugada Syndrome and Hypertrophic Cardiomyopathy?

2021 ◽  
Vol 11 (3) ◽  
pp. 139-147
Author(s):  
Marianna Farnè ◽  
Cristina Balla ◽  
Alice Margutti ◽  
Rita Selvatici ◽  
Martina De Raffele ◽  
...  
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Brugada Syndrome ◽  
Drug Testing ◽  
Positive Family History ◽  
Structural Heart Disease ◽  
Drug Induced ◽  
St Segment Elevation ◽  
St Segment ◽  
Electrocardiogram Ecg

Brugada syndrome (BrS) is an inherited disorder with high allelic and genetic heterogeneity clinically characterized by typical coved-type ST segment elevation at the electrocardiogram (ECG), which may occur either spontaneously or after provocative drug testing. BrS is classically described as an arrhythmic condition occurring in a structurally normal heart and is associated with the risk of ventricular fibrillation and sudden cardiac death (SCD). We studied five patients with spontaneous or drug-induced type 1 ECG pattern, variably associated with symptoms and a positive family history through a Next Generation Sequencing panels approach, which includes genes of both channelopathies and cardiomyopathies. We identified variants in MYBPC3 and in MYH7, hypertrophic cardiomyopathy (HCM) genes (MYBPC3: p.Lys1065Glnfs*12 and c.1458-1G > A, MYH7: p.Arg783His, p.Val1213Met, p.Lys744Thr). Our data propose that Brugada type 1 ECG may be an early electrocardiographic marker of a concealed structural heart disease, possibly enlarging the genotypic overlap between Brugada syndrome and cardiomyopathies.

scholarly journals Brugada Syndrome: Progress in Diagnosis and Management

2019 ◽  
Vol 8 (1) ◽  
pp. 13-18 ◽  
Author(s):  
Carlo Pappone ◽  
Vincenzo Santinelli
Keyword(s):  
Brugada Syndrome ◽  
Therapeutic Option ◽  
Electrophysiological Study ◽  
Provocation Test ◽  
Structural Heart Disease ◽  
Sodium Channel Blocker ◽  
Icd Implantation ◽  
St Segment Elevation ◽  
St Segment ◽  
Effective Therapeutic Option

Brugada syndrome (BrS) represents an inherited disorder associated with risk of sudden cardiac death due to VF in patients without structural heart disease. Currently, BrS is diagnosed by typical cove-shaped ST-segment elevation >2 mm in >1 RV precordial lead V1, V2 occurring spontaneously or after a sodium-channel blocker provocation test without any further evidence of malignant arrhythmias. An ICD should always be implanted in symptomatic BrS patients to prevent sudden death, despite high rates of complications with these devices. In asymptomatic people, an electrophysiological study should be performed to evaluate the need for an ICD. The recent discovery of a functional substrate has revolutionised our approach to the pathophysiology and management of BrS. Promising new therapeutic options have emerged in the last 3 years. Ajmaline is able to determine the extension of the substrate by prolonging the duration and fragmentation of abnormal epicardial electrograms. Substrate ablation results in the disappearance of both coved-type ECG and ventricular tachycardia/VF inducibility. These findings are clinically relevant, suggesting that epicardial ablation guided by ajmaline infusion may be an effective therapeutic option in BrS, potentially removing the need for ICD implantation.

scholarly journals Brugada-Like Electrocardiographic Patterns Induced by Hyperkalemia

Medicina ◽  
2013 ◽  
Vol 49 (3) ◽  
pp. 24 ◽  
Author(s):  
Dagmara Reingardienė ◽  
Jolita Vilčinskaitė ◽  
Diana Bilskienė
Keyword(s):  
Brugada Syndrome ◽  
Structural Heart Disease ◽  
Electrolyte Disorders ◽  
St Segment Elevation ◽  
Wave Inversion ◽  
St Segment ◽  
Acute Renal Insufficiency ◽  
Channel Mutation ◽  
The Right ◽  
Ecg Abnormalities

Brugada syndrome was described in 1992 as a new clinical and electrocardiographic syndrome involving susceptibility to ventricular arrhythmias and sudden cardiac death in patients with no obvious structural heart disease. Brugada syndrome is characterized by a hereditary anomaly in the sodium ion channel (mutation of the SCN5A gene) identified by a wide QRS associated with the ST-segment elevation and the T‑wave inversion in the right precordial leads. The Brugada-like electrocardiographic pattern can be caused by sodium channel-blocking drugs and electrolyte disorders. Hyperkalemia may produce multiple ECG abnormalities, including the ST-segment elevation and pseudomyocardial infarction with a resolution of these abnormalities after the correction of hyperkalemia. This article describes 8 cases of pseudoanteroseptal myocardial infarction in acute renal insufficiency with hyperkalemia. The ST-segment elevation related to hyperkalemia is resolved by the reduced serum potassium level. Clinicians should recognize that hyperkalemia is one of the etiologies of the Brugada-like electrocardiographic pattern.

44. Publication history of the Brugada Syndrome: Did labeling stimulate research?

2007 ◽  
Vol 30 (4) ◽  
pp. 50
Author(s):  
M. A. Nault ◽  
A. Baranchuk ◽  
C. S. Simpson ◽  
D. P. Redfearn ◽  
H. Abdollah
Keyword(s):  
Sudden Cardiac Death ◽  
Right Bundle Branch Block ◽  
Brugada Syndrome ◽  
Cardiac Death ◽  
Young Men ◽  
Bundle Branch Block ◽  
St Segment Elevation ◽  
Publication History ◽  
St Segment ◽  
History Of

Sudden cardiac death (SCD) in healthy young men was first recognized in 1917. Combined with an electrocardiographic (ECG) abnormality reported in 1953, the resulting syndrome would by 1996 eventually be recognized worldwide as Brugada Syndrome (BrS). There is evidence that “labeling” (i.e. the process of naming a disease state or ascribing a diagnosis) alters perceptions, awareness and behaviours in medicine. Our objective was to determine whether naming a cluster of signs and symptoms as a specific syndrome raised attention given to a previously recognized though poorly defined condition as evidenced through an increase in publications. We hypothesized that naming BrS resulted in such a “labeling” stimulus. A systematic review of the Pubmed database of indexed journals was performed to identify references to BrS between 1950 and 2006. Search terms were: “Brugada Syndrome”; “Sudden Cardiac Death AND Right Bundle Branch Block”; “Bangungot” (Filipino); “Bangungut” (Filipino); “Pok kuri” (Japanese); “Lai tai” (Thai); “Sudden Unexplained Death Syndrome”; and “SUNDS”. Publications identified after 1996 by search strategies other than “Brugada Syndrome” were omitted to avoid double counting. The search resulted in 1,042 citations. Of these, 208 occurred after 1996 and were omitted, leaving a total of 834 citations to be analyzed. Between 1950 and 1993 a total of 32 publications met the above search criteria. Thereafter, publication rate on this topic increased exponentially from 16 articles in 1994-1995 to 290 in 2005-2006. Though numerous articles recognized either RBBB-like ECG pattern, ST segment elevation or SCD in otherwise healthy young men, it was not until publication of the 1992 Brugada and Brugada paper that the coexistence of these two conditions was recognized as a syndrome. Rising interest, as identified by publication frequency, preceded the naming of this syndrome by 4 years. This finding suggests that factors other than labeling have also contributed to the publication history of BrS. Osher H, Wolff L. Electrocardiographic pattern simulating acute myocardial injury. Am J Med Sci 1953; 226:541-5. Brugada P, Brugada J. Right bundle branch block, persisting ST segment elevation and sudden cardiac death: A distinct clinical and electrocardiographic syndrome. J Am Coll Cardiol 1992; 20:1391-6. Yan G-X, Antzelevitch C. Cellular basis for the electrocardiographic J wave. Circulation 1996; 93:372-9.

Intraventricular conduction defects in patients with st-segment elevation myocardial infarction – the paradox of right bundle branch block

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
S Fernandes ◽  
F Montenegro ◽  
M Cabral ◽  
R Carvalho ◽  
L Santos ◽  
...  
Keyword(s):  
Hospital Mortality ◽  
Right Bundle Branch Block ◽  
Independent Predictor ◽  
Left Ventricular Ejection ◽  
Bundle Branch Block ◽  
Hospital Outcomes ◽  
St Segment Elevation ◽  
Intraventricular Conduction ◽  
St Segment ◽  
Worse Prognosis

Abstract   Intraventricular conduction defects (IVCD) in patients with acute myocardial infarct (AMI) are predictors of a worse prognosis. When acquired they can be the result of an extensive myocardial damage. Purpose To assess the impact of IVCD, regardless of being previously known or presumed new, on in-hospital outcomes of patients with AMI with ST segment elevation (STEMI) or undetermined location. Methods From a series of patients included in the National Registry of Acute Coronary Syndrome between 10/1/2010 and 9/1/2019, were selected patients with STEMI or undetermined AMI, undergoing coronary angiography. Results 7805 patients were included: 461 (5.9%) presenting left bundle branch block (LBBB), 374 (4.8%) with right bundle branch block (RBBB) and 6970 (89.3%) with no IVCD. Clinical characteristics as well as in-hospital outcomes are described in the table 1. An unexpected worse prognosis in patients with RBBB has motivated a multivariate analysis. RBBB remained an independent predictor of in-hospital mortality (OR 1.91, 95% CI 1.04–3.50, p=0.038), along with female gender (OR 1.73, 95% CI 1.11–2.68, p=0.015), Killip Class&gt;1 (OR 2.26, 95% CI 1.45–3.53, p&lt;0.001), left ventricular ejection fraction &lt;50% (OR 3.93, 95% CI 2.19–7.05, p&lt;0.001) and left anterior descending artery as the culprit lesion (OR 1.85, 95% CI 1.16–2.91, p=0.009). Conclusion In spite of an apparent better clinical profile, in the current large series of unselected STEMI patients, the presence of RBBB is associated with the worst in-hospital outcome. RBBB doubles the risk of death, being an independent predictor of in-hospital mortality. Funding Acknowledgement Type of funding source: None

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