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 syndrome: A brand new case

2009 ◽  
Vol 66 (8) ◽  
pp. 667-670
Author(s):  
Ruzica Jurcevic ◽  
Lazar Angelkov ◽  
Dejan Vukajlovic ◽  
Velibor Ristic ◽  
Milosav Tomovic ◽  
...  
Keyword(s):  
Ventricular Tachycardia ◽  
Ventricular Fibrillation ◽  
Brugada Syndrome ◽  
Electrophysiological Study ◽  
Structural Heart Disease ◽  
Left Ventricular ◽  
Left Ventricular Ejection ◽  
Type I ◽  
Ventricular Ejection Fraction ◽  
St Segment Elevation

Background: Brugada syndrome (BS) is a disorder characterized by syncope or sudden death associated with one of several electrocardiographic (ECG) patterns characterized by incomplete right bundle branch block and ST elevation in the anterior precordial leads. Patients with BS are prone to develop ventricular tachyarrhythmias that may lead to syncope, cardiac arrest, or sudden cardiac death. Case report. A 58-year-old woman is the first described case of Brugada syndrome in Serbia with intermittent typical changes in basic electrocardiography (ECG): ST segment elevation in the precordial chest leads like dome or coved - major form or type I. For the last 27 years the patient had suffered of palpitations and dizziness, without syncopal events. Her sister had died suddenly during the night in sleep. During 24-hour Holter monitoring the patient had ventricular premature beats during the night with R/T phenomenon and during the recovery phase of exercise testing had rare premature ventricular beats as the consequence of parasympatethic stimulation. Late potentials were positive. Echocardiography revealed left ventricular ejection fraction of 60%. We performed coronary angiography and epicardial coronary arteries were without significant stenosis and structural heart disease was excluded. In the bigining of the electrophysiological study ECG was normal, and after administration of Propaphenon i.v. Brugada syndrome unmasked with appearance of type I ECG pattern. A programed ventricular stimulation induced non sustained ventricular tachycardia. One-chamber implantable cardioverter defibrillator was implanted and the patient was treated with a combination od amiodarone and metoprolol per os. After one-year follow-up, there were no episodes of ventricular tachycardia and ventricular fibrillation. Conclusion. Brugada syndrome is a myocardial disorder which prognosis and therapy are related to presence of ventricular fibrillation or ventricular tachycardia. Electrophysiologicaly induced malignant ventricular disorders class I are indication for implantation of cardioverter defibrilator, as also occurred in presented patient.

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 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-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.

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 Risk Stratification and Therapeutic Approach in Brugada Syndrome

2012 ◽  
Vol 1 ◽  
pp. 17 ◽  
Author(s):  
Vincent Probst ◽  
Stéphanie Chatel ◽  
Jean-Baptiste Gourraud ◽  
Hervé Le Marec ◽  
◽  
...  
Keyword(s):  
Sudden Death ◽  
Brugada Syndrome ◽  
High Rate ◽  
Icd Implantation ◽  
St Segment Elevation ◽  
Asymptomatic Patients ◽  
St Segment ◽  
History Of ◽  
The Right

Brugada syndrome (BrS) is a clinical entity characterised by an incomplete right bundle branch block associated with an ST segment elevation in the right precordial leads and a risk of ventricular arrhythmia and sudden death in the absence of structural abnormalities. Patients with a personal history of sudden death have an annual arrhythmia risk of recurrence as high as 10 %. Similarly, the presence of syncope is consistently associated with an increased arrhythmic risk. This risk can be estimated at about 1.5 % per year. The risk is lower in asymptomatic patients. Regarding the relatively high rate of complication of Implantable cardioverter defibrillator (ICD) implantation, in most of the cases, asymptomatic patients with a Brugada syndrome revealed during ajmaline challenge do not need to be implanted. The situation is more complex in patients with a spontaneous type 1 aspect since the risk could be estimated to be around 0.8 % per year. For these patients, a careful evaluation of the arrhythmic risk using all the different tools available is mandatory.

scholarly journals Fever Unmasked Brugada Syndrome in Pediatric Patient: A Case Report

2020 ◽  
Vol 4 (2) ◽  
pp. 244-246
Author(s):  
Orhay Mirzapolos ◽  
Perry Marshall ◽  
April Brill
Keyword(s):  
Emergency Medicine ◽  
Case Report ◽  
Brugada Syndrome ◽  
Ventricular Arrhythmias ◽  
Cardiac Monitoring ◽  
St Segment Elevation ◽  
Cardioverter Defibrillator ◽  
St Segment ◽  
Viral Illness ◽  
Automatic Implantable Cardioverter Defibrillator

Introduction: Brugada syndrome is an arrhythmogenic disorder that is a known cause of sudden cardiac death. It is characterized by a pattern of ST segment elevation in the precordial leads on an electrocardiogram (EKG) due to a sodium channelopathy. Case Report: This case report highlights the case of a five-year-old female who presented to the emergency department with a febrile viral illness and had an EKG consistent with Brugada syndrome. Discussion: Fever is known to accentuate or unmask EKG changes associated with Brugada due to temperature sensitivity of the sodium channels. Conclusion: Febrile patients with Brugada are at particular risk for fatal ventricular arrhythmias and fevers should be treated aggressively by the emergency medicine provider. Emergency medicine providers should also consider admitting febrile patients with Brugada syndrome who do not have an automatic implantable cardioverter-defibrillator for cardiac monitoring.

scholarly journals B-PO04-024 PREDICTION OF A POSITIVE SODIUM CHANNEL BLOCKER PROVOCATION TEST IN PATIENTS SUSPECTED OF BRUGADA SYNDROME

Heart Rhythm ◽  
2021 ◽  
Vol 18 (8) ◽  
pp. S289
Author(s):  
Martijn Hendrik van der Ree ◽  
Jeroen Vendrik ◽  
Tom E. Verstraelen ◽  
Jan A. Kors ◽  
Ahmad S. Amin ◽  
...  
Keyword(s):  
Sodium Channel ◽  
Brugada Syndrome ◽  
Channel Blocker ◽  
Provocation Test ◽  
Sodium Channel Blocker

scholarly journals Genetic and biophysical basis for bupivacaine-induced ST segment elevation and VT/VF. Anesthesia unmasked Brugada syndrome

Heart Rhythm ◽  
2006 ◽  
Vol 3 (9) ◽  
pp. 1074-1078 ◽  
Author(s):  
Kevin Vernooy ◽  
Serge Sicouri ◽  
Robert Dumaine ◽  
Kui Hong ◽  
Antonio Oliva ◽  
...  
Keyword(s):  
Brugada Syndrome ◽  
St Segment Elevation ◽  
St Segment

scholarly journals Sudden Cause of Cardiac Death—Be Aware of Me: A Case Report and Short Review on Brugada Syndrome

2010 ◽  
Vol 2010 ◽  
pp. 1-3 ◽  
Author(s):  
Jagadeesh K. Kalavakunta ◽  
Vishwaroop Bantu ◽  
Hemasri Tokala ◽  
Mihas Kodenchery
Keyword(s):  
Brugada Syndrome ◽  
Past History ◽  
Short Review ◽  
Case Presentation ◽  
St Segment Elevation ◽  
First Case ◽  
St Segment ◽  
The Right ◽  
Malignant Arrhythmias

Introduction. Brugada syndrome accounts for about 4% of sudden cardiac deaths (SCD). It is characterized by an ST-segment elevation in the right precordial electrocardiogram (EKG) leads.Case Presentation. We describe a 39-year-old healthy Caucasian man who was admitted to the intensive care unit after being cardioverted from ventricular fibrillation (VF) arrest. His past history was significant for an episode of syncope one month prior to this presentation for which he was admitted to an outlying hospital. EKG during that admission showed ST elevations in V1 and V2 leads, a pattern similar to Type 1 Brugada. A diagnosis of Brugada syndrome was missed and the patient had a cardiac arrest a month later. We discuss a short review of Brugada syndrome and emphasize the need to look for it in patients presenting with SCD and malignant arrhythmias.Conclusion. Physicians should always consider Brugada syndrome in the differential diagnosis of ST-segment elevation in anterior precordial leads of EKG and associated VT/VF. Although more than 17 years have passed since the first case was reported, increased awareness of this syndrome is needed to identify patients with EKG changes and treat them accordingly to prevent incidence of (SCD) and its deleterious complications.

Sign in / Sign up

Export Citation Format

Share Document