Standard ECG in Brugada Syndrome as a Marker of Prognosis: From Risk Stratification to Pathophysiological Insights

Background The 12‐lead ECG plays a key role in the diagnosis of Brugada syndrome (BrS). Since the spontaneous type 1 ECG pattern was first described, several other ECG signs have been linked to arrhythmic risk, but results are conflicting. Methods and Results We performed a systematic review to clarify the associations of these specific ECG signs with the risk of syncope, sudden death, or equivalents in patients with BrS. The literature search identified 29 eligible articles comprising overall 5731 patients. The ECG findings associated with an incremental risk of syncope, sudden death, or equivalents (hazard ratio ranging from 1.1–39) were the following: localization of type 1 Brugada pattern (in V2 and peripheral leads), first‐degree atrioventricular block, atrial fibrillation, fragmented QRS, QRS duration >120 ms, R wave in lead aVR, S wave in L1 (≥40 ms, amplitude ≥0.1 mV, area ≥1 mm 2 ), early repolarization pattern in inferolateral leads, ST‐segment depression, T‐wave alternans, dispersion of repolarization, and Tzou criteria. Conclusions At least 12 features of standard ECG are associated with a higher risk of sudden death in BrS. A multiparametric risk assessment approach based on ECG parameters associated with clinical and genetic findings could help improve current risk stratification scores of patients with BrS and warrants further investigation. Registration URL: https://www.crd.york.ac.uk/prospero/ . Unique identifier: CRD42019123794.

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Brugada syndrome: Case report

Srpski arhiv za celokupno lekarstvo ◽

10.2298/sarh1202084b ◽

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.

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Abstract 5669: Brugada Syndrome: The Absence of Symptoms is a Poor Predictor of Identifying Individuals at Risk of Sudden Death

Circulation ◽

10.1161/circ.118.suppl_18.s_981 ◽

2008 ◽

Vol 118 (suppl_18) ◽

Author(s):

Michael Papadakis ◽

Carey Edwards ◽

John C Rawlins ◽

Sanjay Sharma

Keyword(s):

Sudden Cardiac Death ◽

Sudden Death ◽

Risk Stratification ◽

Brugada Syndrome ◽

Cardiac Death ◽

Polymorphic Ventricular Tachycardia ◽

Screening Programs ◽

First Degree Relatives ◽

History Of

Risk stratification for sudden death in Brugada syndrome and hence indications for implantation of an internal cardioverter defibrillator are based on the presence of a spontaneous type 1 electrocardiogram (ECG) pattern, in association with unheralded syncope or documented polymorphic ventricular tachycardia. Increasingly, awareness of sudden death in the young and implementation of pre-participation screening programs in sport will identify young, asymptomatic patients with the Brugada phenotype. We evaluated the predictive accuracy of symptoms, particularly unheralded syncope in victims of sudden death from Brugada syndrome. Over the past 3 years we identified 22 victims of sudden cardiac death secondary to Brugada syndrome. The diagnosis was based on sudden death with normal findings at post mortem and the identification of the type 1 Brugada ECG pattern in first-degree relatives (spontaneous or following ajmaline provocation test). All relatives underwent 12-lead ECG, echocardiography, exercise testing, 24-hour Holter monitor and biochemical tests. Cardiac magnetic resonance imaging and coronary angiography were performed when appropriate. Of the victims dying from Brugada syndrome, 15 (68%) were male. The mean age of sudden death was 30 years (range 8 –56 years) and 95% died suddenly at rest or during sleep. We interviewed first-degree relatives and partners regarding prodromal symptoms or a history of epilepsy. Only 3 out of 22 victims (14%) had significant symptoms. Specifically, 2 individuals experienced unheralded syncope and 1 suffered nocturnal seizures. Only 14% had a family history of premature sudden cardiac death. Indeed we obtained 12-lead ECGs in 3 victims taken less than 6 months prior to sudden death, which failed to reveal the typical Brugada ECG phenotype. Our results indicate that the vast majority of individuals who die from Brugada syndrome in the UK are asymptomatic and raise concerns that the absence of symptoms does not necessarily mean low risk. Better understanding of the disorder and risk stratification protocols is necessary to permit genuine reassurance in asymptomatic individuals exhibiting the Brugada phenotype.

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Predictors of Appropriate Shocks and Ventricular Arrhythmia in Indonesian with Brugada Syndrome

Indonesian Journal of Cardiology ◽

10.30701/ijc.v40i2.767 ◽

2019 ◽

Vol 40 (2) ◽

Author(s):

Ardian Rizal ◽

Sunu Budhi Raharjo ◽

Dicky Armein Hanafy ◽

Yoga Yuniadi

Keyword(s):

Ventricular Arrhythmia ◽

Risk Stratification ◽

Brugada Syndrome ◽

S Wave ◽

Early Repolarization ◽

Predictive Values ◽

Wave Duration ◽

Fragmented Qrs ◽

History Of

Background : Brugada syndrome is an inherited disease characterized by an increased risk of sudden cardiac death owing to ventricular arrhythmias in the absence of structural heart disease. It has been reported that this syndrome is more prevalent in South-East Asia than in Western countries. Furthermore, genetic studies showed important contributions of several gene mutations to the phenotype of BrS. These suggest that ethnic difference play significant roles in the pathogenesis of BrS. In addition, ICD implantation remains the cornerstone management with a low rate of appropriate shocked. Therefore, it is important to investigate patients’ characteristics for risk stratification. Our objective to investigate the clinical, electrocardiography (ECG) and electrophysiological characteristics that can be used as predictor of appropriate shock due to ventricular arrhythmia (VA) in Indonesian patients with BrS. Methods : We analyse data from Brugada syndrome registry at National Cardiovascular Centre Harapan Kita since January 2013. Total 22 patients were included. Characteristics of BrS that we analysed were baseline characteristics (age and sex), Clinical finding (syncope, cardiac arrest), ECG finding (spontaneous type 1 or drug induced) and Electrophysiology study result (inducible VA and RV ERP). We also added some new ECG characteristic (S wave in lead 1, S wave duration in V1, Fragmented QRS, Junction ST elevation and early repolarization pattern in infero-lateral) to be analysed. Our end point are appropriate shock during ICD interrogation for those who have been implanted an ICD, and documented VA for those who didn’t receive ICD. Result : We found high incidence of appropriate ICD’s shock in our population (50% in our study vs 5-11.5% in real world). Predictors of appropriate shock and documented VA are history of syncope (p = 0.045; OR 2.57 [1.44-4.59]), spontaneous type-1 ECG (p = 0.005) and right ventricular effective refractory period (RV ERP) of <200 ms (p=0.018). Other parameters that have been reported to correlate with the occurrence of VA (S Wave in lead 1 (p = 0.530), early repolarization pattern (p = 0.578), fragmented QRS (p = 0.601), S Wave duration (p = 0.365) and J Point STE (p = 0.800) were found to be not correlated to appropriate shock in our populations. Conclusion : History of syncope, spontaneous type-1 Brugada ECG and RV ERP of <200 ms have predictive values for risk stratification of Indonesian patients with Brugada syndrome. Keywords : Brugada Syndrome, Ventricular arrhythmia, ICD shock

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Rhabdomyoma of the interventricular septum presenting as a Brugada phenocopy

Cardiology in the Young ◽

10.1017/s1047951111000424 ◽

2011 ◽

Vol 21 (5) ◽

pp. 591-594 ◽

Cited By ~ 7

Author(s):

Timothy Nguyen ◽

John Smythe ◽

Adrian Baranchuk

Keyword(s):

Sudden Death ◽

Brugada Syndrome ◽

Ventricular Arrhythmias ◽

Interventricular Septum ◽

St Segment Elevation ◽

St Segment ◽

The Right

AbstractBrugada syndrome is a channelopathy characterised electrocardiographically by distinctive coved ST-segment elevation in the right precordial leads and is associated with a predisposition for sudden death secondary to ventricular arrhythmias in otherwise healthy patients. Previously known as Brugada-like patterns, Brugada phenocopies include agents and conditions that mimic true Brugada syndrome, presenting with an acquired Brugada Type-1 ECG pattern. We describe the first reported case of a 17-month-old female with an asymptomatic rhabdomyoma of the interventricular septum that presented as a Brugada phenocopy.

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Risk Stratification and Therapeutic Approach in Brugada Syndrome

Arrhythmia & Electrophysiology Review ◽

10.15420/aer.2012.1.17 ◽

2012 ◽

Vol 1 ◽

pp. 17 ◽

Cited By ~ 3

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.

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P945What is the role of late-potentials determined by signal-averaged ECG in predicting flecainide provocative test in brugada pattern?

EP Europace ◽

10.1093/europace/euaa162.284 ◽

2020 ◽

Vol 22 (Supplement_1) ◽

Author(s):

J Brito ◽

N Cortez-Dias ◽

N Nunes-Ferreira ◽

I Aguiar-Ricardo ◽

G Silva ◽

...

Keyword(s):

Risk Stratification ◽

Brugada Syndrome ◽

Fold Increase ◽

Diagnostic Study ◽

Late Potentials ◽

Qrs Complex ◽

Provocative Test ◽

Sudden Cardiac Death Risk ◽

Brugada Pattern

Abstract Introduction The sudden cardiac death risk in Brugada Syndrome (BrS) is higher in patients with spontaneous type 1 pattern. Brugada diagnosis is also established in patients with induced type 1 morphology after provocative test with intravenous administration with a sodium blocker channel. Nevertheless, this group of patients is known to be at a lower risk of SCD, and their risk stratification is still a matter of discussion. Late potentials (LP) detected on signal-averaged ECG (SAECG) on the RVOT have been previously proposed as a predictor factor for BrS, even though data is lacking on its value. Purpose To evaluate the association between positive LP (LMS40> 38ms) on SAECG with modified Brugada leads and a positive flecainide test in patients with non-type 1 BrS. Methods Retrospective single-center study of non-type 1 BrS patients referred for the performance of a flecainide provocative test. Patients presenting with spontaneous type 1 morphology were excluded from the study. Study of LP on SAECG with modified leads for Brugada were evaluated before administration of flecainide [2mg/kg (maximum150mg), for 10minutes] with determination of filtered QRS duration (fQRS), root mean square voltage of the last 40ms of the QRS complex (RMS40) and duration of low amplitude signals <40μV of the terminal QRS complex (LMS40). Results 126 patients (47.3 ± 14.1 years, 61.9% males) underwent study with LP SAECG and flecainide test. Among these patients, 7.9% were symptomatic and 16.7% had familiar history of BrS. Flecainide test was positive in 46.8% of patients. In patients with a positive flecainide test, 64.4% presented LMS40 > 38ms whereas LMS40 > 38ms was present in only 46% of those with a negative flecainide test (p = 0.031). The presence of positive LMS40 was a positive predictor for a positive flecainide test, associated with a two-fold increase likelihood in the induction of a Brugada pattern (OR: 2,12; IC95% 1,025-4,392; P = 0,043). There was no association between fQRS or RMS40 and a positive flecainide test (p = NS). fQRS > 114ms and RMS40 <20uV was present in 22% and 61% of patients with a positive flecainide test, respectively. Conclusion In patient with non-type 1 Brugada syndrome, LMS40 > 38ms in SAECG was a predictor for a positive flecainide test, suggesting that this finding could be helpful on the risk stratification of patients undergoing diagnostic study for Brugada syndrome. Abstract Figure. Effect of LMS 40 in flecainide test

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

Case Reports in Medicine ◽

10.1155/2010/823490 ◽

2010 ◽

Vol 2010 ◽

pp. 1-3 ◽

Cited By ~ 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.

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6123A novel ECG parameter predicts lack of eligibility for Subcutaneous Implantable Cardioverter Defibrillator (S-ICD) in patients with Brugada Syndrome

European Heart Journal ◽

10.1093/eurheartj/ehz746.0149 ◽

2019 ◽

Vol 40 (Supplement_1) ◽

Author(s):

A Mazzanti ◽

S Marelli ◽

T Chargeishvili ◽

A Trancuccio ◽

M Marino ◽

...

Keyword(s):

Brugada Syndrome ◽

Multivariable Analysis ◽

Previous History ◽

Standing Position ◽

Implantable Cardioverter Defibrillators ◽

S Wave ◽

History Of ◽

Automated Screening ◽

Screening Sensitivity

Abstract Background A conclusive estimate of the eligibility rate for the use of subcutaneous implantable cardioverter defibrillators (S-ICD) in patients with Brugada Syndrome (BrS) is lacking. Objective We aimed to: 1) evaluate the eligibility for S-ICD in patients with BrS using a novel automated tool; 2) investigate predictors of ineligibility for S-ICD, based on baseline 12-lead electrocardiogram. Methods Automated screening for S-ICD was performed in 118 consecutive BrS patients using the programmer provided by the S-ICD producer. The system automatically assessed the acceptability of each of the three sense vectors used by the S-ICD for the detection of cardiac rhythm. Eligibility was defined when at least one vector was acceptable both in supine and standing position. Results The clinical characteristics of 118 BrS patients were as follow: age 43±11 years; 89% males; 2% with aborted cardiac arrest; 14% with a history of syncope; 81% with spontaneous type 1 ECG pattern; 21% with a familial history of sudden cardiac death; 24% with an SCN5A mutation. No patients had an indication for pacing. Only 8/118 (7%) patients were ineligible for S-ICD. Of note, 5/8 (63%) patients who failed the screening exhibited a slurred S wave of ≥80 ms duration in the peripheral lead aVF on the 12-lead baseline electrocardiogram, vs. 4/110 (4%) of those who passed the screening (sensitivity of S wave in aVF for screening failure 63%, specificity 97%; p<0.001). Remarkably, the presence of a slurred S wave of ≥80 ms duration in lead aVF remained significantly associated to the failure of eligibility for S-ICD (odds ratio 50, p<0.001) in a multivariable analysis that included the previous history of symptoms, the presence of a spontaneous type 1 ECG pattern, the gender and the presence of SCN5A mutations. ECG predictor of S-ICD screening Conclusion Up to 93% of BrS patients are eligible for S-ICD when the automated screening tool is used. The presence of a slurred S wave in lead aVF on the 12-lead electrocardiogram is a powerful predictor of screening failure.

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