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 Brugada syndrome: Case report

2012 ◽  
Vol 140 (1-2) ◽  
pp. 84-90
Author(s):  
Vesna Bisenic ◽  
Sasa Hinic ◽  
Mirjana Krotin ◽  
Branislav Milovanovic ◽  
Jelena Saric ◽  
...  
Keyword(s):  
Family History ◽  
Sudden Death ◽  
Brugada Syndrome ◽  
Troponin T ◽  
Stable Condition ◽  
St Segment Elevation ◽  
St Segment ◽  
History Of ◽  
The Right

Introduction. Brugada syndrome is an arrhythmogenic disease characterized by coved ST segment elevation and J point elevation of at least 2 mm in at least two of the right precordial ECG leads (V1-3) and ventricular arrhythmias, syncope, and sudden death. Risk stratifications of patients with Brugada electrocardiogram are being strongly debated. Case Outline. A 23-year-old man was admitted to the Coronary Care Unit of the Clinical Centre ?Bezanijska kosa? due to weakness, fatigue and chest discomfort. The patient suffered from fainting and palpitations. There was a family history of paternal sudden death at 36 years. Electrocardiogram showed a coved ST segment elevation of 4 mm in leads V1 and V2, recognised as spontaneous type 1 Brugada pattern. Laboratory investigations revealed normal serum cardiac troponin T and serum potassium, and absence of inflammation signs. Echocardiographic finding was normal, except for a mild enlargement of the right atrium and ventricle. The diagnosis of Brugada syndrome was made by Brugada-type 1 electrocardiogram and the family history of sudden death <45 years. The patient was considered as a high risk, because of pre-syncope and palpitations. He underwent ICD implantation (Medtronic MaximoVR7232Cx) using the standard procedure. After implantation, noninvasive electrophysiology study was done and demonstrated inducible VF that was interrupted with the second 35 J DC shock. The patient was discharged in stable condition with beta-blocker therapy. After a year of pacemaker check-ups, there were no either VT/ VF events or ICD therapy. Conclusion. Clinical presentation is the most important parameter in risk stratification of patients with Brugada electrocardiogram and Brugada syndrome.

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.

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.

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 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.

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.

Fever-induced ST-segment elevation and T-wave alternans in a patient with Brugada syndrome

Resuscitation ◽  
2003 ◽  
Vol 57 (3) ◽  
pp. 315-317 ◽  
Author(s):  
Julián Ortega-Carnicer ◽  
Juan Benezet ◽  
Filomena Ceres
Keyword(s):  
Brugada Syndrome ◽  
St Segment Elevation ◽  
T Wave ◽  
St Segment ◽  
T Wave Alternans

Abstract 2142: Diagnostic and Prognostic Value of Augmented ST-Segment Elevation at Exercise Testing in Patients with Brugada Syndrome

Circulation ◽  
2008 ◽  
Vol 118 (suppl_18) ◽  
Author(s):  
Hisaki Makimoto ◽  
Eiichiro Nakagawa ◽  
Hiroshi Takaki ◽  
Kenichiro Yamagata ◽  
Hiro Kawata ◽  
...  
Keyword(s):  
Exercise Testing ◽  
Brugada Syndrome ◽  
Recovery Phase ◽  
Peak Exercise ◽  
Early Recovery ◽  
St Segment Elevation ◽  
St Segment ◽  
History Of ◽  
Diagnostic And Prognostic Value ◽  
Early Recovery Phase

Background : It has been reported that ST-segment elevation was augmented at early recovery phase or at peak exercise during exercise testing in some patients with Brugada syndrome (BrS), but its diagnostic and prognostic value has not fully been clarified. Methods : Treadmill exercise testing (TMT) was conducted in 93 patients (pts) with BrS (22 documented VF, 31 syncope alone, and 40 asymptomatic; 91 males, 46±14 years) and 22 healthy control subjects (20 males, 48±14 years, 11 with incomplete right bundle branch block (RBBB) and 4 with complete RBBB). Results : The augmentation of ST-segment elevation ≥0.05mV in V1-V3 leads compared with that before exercise was observed at early recovery phase (1– 4 minutes at recovery phase) in 32 BrS pts (34%, Group1), at peak exercise in 8 BrS pts (9%, Group 2), but not in either the remaining 53 BrS pts (57%, Group 3) or 22 control subjects. There were no significant differences among the 3 BrS groups in the baseline clinical (age, gender, family history of sudden death, SCN5A mutation), electrocardiographic (PR, QRS duration, QTc interval and ST amplitude), and electrophysiologic (AH, HV interval, and VF induction) variables except for the positive ratio of signal averaged ECG (87%, 48%, 63%; P= 0.001). No significant difference was observed in the proportion of previous cardiac events (VF/syncope/asymptomatic; 6/12/14, 0/3/5, 16/16/21, respectively for Group 1, 2, and 3). During 76.0, 74.8, and 52.0 months follow-up, VF occurred in 12/32 (38%) pts of Group1, 0/8 (0 %) pts of Group2, and 10/53 (19%) pts of Group3. Augmentation of ST-segment elevation at early recovery phase was a significant and an independent predictor for subsequent VF occurrence (12/32 (38%) in Group1 vs. 10/61 (16%) in Group2 and 3; hazard ratio [HR]= 1.68; P=0.014), especially in 31 pts with a history of syncope alone (6/12 (50%) in Group1 vs. 1/19 (5%) in Group2 and 3; HR=3.44; P=0.004). Conclusions : Our data suggest that augmentation of ST-segment elevation at early recovery phase during exercise testing can be a predictor of poor prognosis in patients with Brugada syndrome, especially in those with a history of syncope alone.

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