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

EP Europace ◽  
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

P6582Control and stability theory, modelling ventricular dynamics in superficial 12-lead ECG as a novel contribution to non-invasive risk stratification of Brugada ECG patterns(?)

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
A Sabatini
Keyword(s):  
Risk Stratification ◽  
Brugada Syndrome ◽  
Stability Theory ◽  
Cardiac Conduction ◽  
Polymorphic Ventricular Tachycardia ◽  
Ecg Signal ◽  
Stability Parameters ◽  
Poles And Zeros ◽  
Brugada Pattern

Abstract Background Brugada syndrome (BrS) risk stratification in asymptomatic subjects is still currently the most important yet unresolved clinical problem to determine the subset of patients with BrS requiring ICD implantation. The underlying pathophysiological mechanisms responsible for BrS ECG patterns remain unknown, as well as the mechanisms of the sudden onset of polymorphic ventricular tachycardia which leads to ventricular fibrillation and sudden cardiac death (SCD). Purpose This study aims to analyze from a totally alternative perspective, superficial 12-lead ECG signals. It departs from the numerous and various attempts to characterize and measure single morphology of specific and individual ECG segments, intervals and waves, rather focusing on and studying the dynamics and stability of the superficial 12-lead ECG signal as a whole to determine stability parameters able to contribute to BrS ECG pattern risk stratification and differential diagnosis of BrS. Methods A quantitative stability control closed loop system has been designed to model the electrophysiology dynamics of the cardiac conduction system with a 12-lead superficial ECG signal being the input and output of the system (Fig. 1). A normal ECG signal and a type-1 coved Brugada pattern ECG-V2 portion have been scanned, digitized and quantitatively processed to obtain stability parameters (poles and zeros in the S-plane). Scanning was performed by Digitizeit – Digital River GmbH. Processing in the S-plane was performed by ©2019 Wolfram Demonstrations Project & Contributors, http://demonstrations.wolfram.com/, poles and zeros and Microsoft Excel software was also used. Results Poles and zeros of the system for type-1 coved Brugada pattern ECG-V2 and for the normal ECG-V2 are shown in Fig. 2, together with stability. Conclusions Based on our data, 1. It appears that portions of the ECG patterns, approximated by mathematical continuous time models representing, at the infinitesimal limit, every possible pattern and behaviors of an ECG signal, such as repolarization patterns, may exhibit interesting dynamics characteristics of stability and can be stratified as stable, marginally stable or unstable. 2. Such a classification may then be implemented to risk stratify repolarization patterns. When tending to instability, such patterns seem to be associated to high risk repolarization patterns such as BrS coved type-1 pattern, hence indicating higher risk of developing polymorphic VT or SCD. In conclusion, more work will be needed to further this methodology to improve the understanding of the effects of the various physiological and pathological substrates involved with malignant arrhythmias and to improve risk stratification strategies to determine the subset of patients with Brugada syndrome requiring ICD insertion. Control systems and stability theory may indeed indicate an interesting and effective procedure for future work.

scholarly journals Functional Bowel Disorders in Patients with Brugada Syndrome and Drug-Induced Type 1 Brugada Pattern

2020 ◽  
Author(s):  
Anil Sarica ◽  
Serhat Bor ◽  
Mehmet Orman ◽  
Hector Barajas-Martinez ◽  
Jimmy Juang ◽  
...  
Keyword(s):  
Brugada Syndrome ◽  
Control Group ◽  
Functional Bowel Disorders ◽  
Drug Induced ◽  
Co Morbidity ◽  
History Of ◽  
Bowel Disorders ◽  
Or History ◽  
Brugada Pattern

Introduction: Irritable bowel syndrome (IBS) is one of the most widely recognized functional bowel disorders (FBDs) with a genetic component. SCN5A gene and SCN1B loci have been identified in population-based IBS cohorts and proposed to have a mechanistic role in the pathophysiology of IBS. These same genes have been associated with Brugada syndrome (BrS). The present study examines the hypothesis that these two inherited syndromes are linked. Methods and Results: Prevalence of FBDs over a 12 months period were compared between probands with BrS/drug-induced type 1 Brugada pattern (DI-Type1 BrP) (n=148) and a control group (n=124) matched for age, female sex, presence of arrhythmia and co-morbid conditions. SCN5A/SCN1B genes were screened in 88 patients. Prevalence of IBS was 25% in patients with BrS/DI-Type1 BrP and 8.1% in the control group (p=2.34×10−4). On stepwise logistic regression analysis, presence of current and/or history of migraine (OR of 2.75; 95% CI: 1.08 to 6.98; p=0.033) was a predictor of underlying BrS/DI-Type1 BrP among patients with FBDs. We identified 8 putative SCN5A/SCN1B variants in 7 (12.3%) patients with BrS/DI-Type1 BrP and 1 (3.2%) patient in control group. Five out of 8 (62.5%) patients with SCN5A/SCN1B variants had FBDs. Conclusion: IBS is a common co-morbidity in patients with BrS/DI-Type1 BrP. Presence of current and/or history of migraine is a predictor of underlying BrS/DI-Type1 BrP among patients with FBDs. Frequent co-existence of IBS and BrS/DI-Type1 BrP necessitates cautious use of certain drugs among the therapeutic options for IBS that are known to exacerbate the Brugada phenotype.

scholarly journals P5529Difference of late potentials detected by signal-averaged ECG in patients with spontaneous or drug-induced type 1 electrocardiogram pattern of Brugada syndrome

2017 ◽  
Vol 38 (suppl_1) ◽  
Author(s):  
J.A. Ribeiro Agostinho ◽  
P. Antonio ◽  
N. Cortez Dias ◽  
G. Lima Da Silva ◽  
T. Guimaraes ◽  
...  
Keyword(s):  
Brugada Syndrome ◽  
Late Potentials ◽  
Drug Induced

scholarly journals Two zebras and a cardiac arrest: a case report of concomitant Brugada syndrome and an anomalous coronary artery

2020 ◽  
Author(s):  
Alexander Bates ◽  
Waqas Ullah ◽  
James Wilkinson ◽  
James Shambrook
Keyword(s):  
Cardiac Arrest ◽  
Coronary Artery ◽  
Coronary Angiography ◽  
Risk Stratification ◽  
Right Coronary Artery ◽  
Brugada Syndrome ◽  
Exercise Stress ◽  
Vagal Activity ◽  
World Cup

Abstract Background Discovering concomitant diagnoses results in a challenge to determine the true cause of a patient’s presentation. Evaluating this fully is vital to plan appropriate and avoid inappropriate therapy. Case summary A 55-year-old gentleman presents in cardiac arrest whilst watching an unusual occurrence of England dominating a Football World Cup game vs. Panama in 2018. Diagnostic coronary angiography discovered an anomalous right coronary artery from the opposite sinus (R-ACAOS), but clinical suspicion this was incidental lead to a further diagnosis of Type 1 Brugada Syndrome (BrS) following a positive Ajmaline provocation challenge. Risk stratification of these two zebras using computed tomography coronary angiography (CTCA), Cardiac magnetic resonance imaging (CMRI), Exercise Stress Echocardiography was performed and following a multi-disciplinary meeting, BrS was felt to be the primary diagnosis. The patient received a secondary prevention implantation of a cardiac defibrillator and avoided cardiac surgery. Discussion Diagnosing a rare condition does not necessarily mean it is the cause of a patient’s presentation and should not end the investigative process. Right coronary artery from the opposite sinus rarely causes cardiac arrest in middle age and is typically associated with peak exercise. Type 1 BrS is associated with cardiac arrest with vagal activity, perhaps such as England winning a World Cup game! Clinical correlation and risk stratification is required for suspected incidental findings.

Non-invasive assessment of the arrhythmogenic substrate in Brugada syndrome using signal-averaged electrocardiogram: clinical implications from a prospective clinical trial

EP Europace ◽  
2019 ◽  
Author(s):  
Giuseppe Ciconte ◽  
Vincenzo Santinelli ◽  
Gabriele Vicedomini ◽  
Valeria Borrelli ◽  
Michelle M Monasky ◽  
...  
Keyword(s):  
Risk Stratification ◽  
Brugada Syndrome ◽  
Predictive Value ◽  
Area Under The Curve ◽  
Late Potentials ◽  
Non Invasive ◽  
Arrhythmogenic Substrate ◽  
Risk Stratification Tool ◽  
Group 2 ◽  
Group 1

Abstract Aims Brugada syndrome (BrS) represents a major cause of sudden cardiac death in young individuals. The risk stratification to forecast future life-threatening events is still controversial. Non-invasive assessment of late potentials (LPs) has been proposed as a risk stratification tool. However, their nature in BrS is still undetermined. The purpose of this study is to assess the electrophysiological determinants of non-invasive LPs. Methods and Results Two hundred and fifty consecutive patients with (Group 1, n = 96) and without (Group 2, n = 154) BrS-related symptoms were prospectively enrolled in the registry. Signal-averaged electrocardiogram (SAECG) was performed in all subjects before undergoing epicardial mapping. Group 1 patients exhibited larger arrhythmogenic substrates (AS; 5.8 ± 2.8 vs. 2.6 ± 2.1 cm2, P < 0.001) with more delayed potentials (220.4 ± 46.0 vs. 186.7 ± 42.3 ms, P < 0.001). Late potentials were present in 82/96 (85.4%) Group 1 and in 31/154 (20.1%) Group 2 individuals (P < 0.001). Patients exhibiting LPs had more frequently a spontaneous Type 1 pattern (30.1% vs. 10.9%, P < 0.001), SCN5A mutation (34.5% vs. 21.2%, P = 0.02), and exhibited a larger AS with longer potentials (5.8 ± 2.7 vs. 2.2 ± 1.7 cm2; 231.2 ± 37.3 vs. 213.8 ± 39.0 ms; P < 0.001, respectively). Arrhythmogenic substrate dimension was the strongest predictor of the presence of LPs (odds ratio 1.9; P < 0.001). An AS area of at least 3.5 cm2 identified patients with LPs (area under the curve 0.88, 95% confidence interval 0.843–0.931; P < 0.001) with a sensitivity of 86%, specificity 88%, positive predictive value 85%, and negative predictive value 89%. Conclusion The results of this study support the role of the epicardial AS as an electrophysiological determinant of non-invasive LPs, which may serve as a tool in the non-invasive assessment of the BrS substrate, as SAECG-LPs could be considered an expression of the abnormal epicardial electrical activity. ClinicalTrials.gov number (NCT02641431; NCT03106701).

scholarly journals A case series of Brugada syndrome with a novel mutation in the ankyrin-B gene: an unusual unmasking in acute myocarditis

2021 ◽  
Vol 5 (6) ◽  
Author(s):  
Maria E Marketou ◽  
Ilias Zareas ◽  
Emmanuel Kanoupakis ◽  
Alexandros Patrianakos ◽  
Fragiskos Parthenakis
Keyword(s):  
Brugada Syndrome ◽  
Novel Mutation ◽  
Beta Blockers ◽  
Acute Myocarditis ◽  
Case Series ◽  
Provocation Test ◽  
Left Ventricular ◽  
Provocative Test ◽  
Ankyrin B

Abstract Background  Brugada syndrome (BrS) is a genetically heterogeneous channelopathy that may lead to sudden death. We report a novel mutation of the ankyrin-B gene that is probably related to the occurrence of BrS in two brothers. Case summary  First, we present the case of a 27-year-old male who was admitted to the hospital with acute myocarditis. The patient showed left ventricular dysfunction and was given carvedilol. Six days later, while asymptomatic and afebrile, the patient exhibited an electrocardiogram (ECG) with repolarization ‘saddleback’ ST changes in V2. A procainamide provocative test was performed with a response for Type 1 Brugada ECG pattern. Genetic testing revealed a novel mutation, c.5418T&gt;A (+/−) (p.His1806Gln), in the ankyrin-B gene encoding. His 34 years old brother had an ECG J point elevation in leads V1 and V2 of 1 mm not fulfilling diagnostic criteria for Brugada ECG pattern. He also experienced arrhythmia-related syncope. Flecainide provocation test changed ECG towards a Type 1 Brugada pattern. A subcutaneous implantable defibrillator (ICD) was implanted. Patient 1 remains asymptomatic while Patient 2 experienced an appropriate ICD shock during follow-up. Discussion  In this case series, two brothers with BrS exhibited the same mutation of the ankyrin-B gene. Ankyrin-B is associated with the stability of plasma membrane proteins in the voltage-gated ion channels. Our finding provides a foundation for further investigation of this mutation in relation to BrS. Moreover, the timing of its presentation raises concerns as to whether myocarditis or beta-blockers are associated with the presentation of BrS ECG.

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

2021 ◽  
Author(s):  
Francesco Vitali ◽  
Alessandro Brieda ◽  
Cristina Balla ◽  
Rita Pavasini ◽  
Elisabetta Tonet ◽  
...  
Keyword(s):  
Sudden Death ◽  
Risk Stratification ◽  
Brugada Syndrome ◽  
S Wave ◽  
Unique Identifier ◽  
Dispersion Of Repolarization ◽  
St Segment ◽  
Assessment Approach ◽  
Lead Avr

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.

Association of atrial septal defect and Brugada syndrome in a young woman

2021 ◽  
pp. 1-3
Author(s):  
Tomoya Tsuchihashi ◽  
Masahiro Kamada ◽  
Yukiko Nakano
Keyword(s):  
Atrial Septal Defect ◽  
Young Woman ◽  
Atrioventricular Block ◽  
Brugada Syndrome ◽  
Septal Defect ◽  
Bundle Branch Block ◽  
Slight Fever ◽  
Asd Closure ◽  
Brugada Pattern

Abstract We report a 25-year-old woman who was diagnosed with atrial septal defect (ASD). An ECG showed only first-degree atrioventricular block and incomplete right bundle branch block. One day after the percutaneous ASD closure, she had a slight fever and an ECG showed a type 1 Brugada pattern. ECG characteristics of ASD are similar to those of a Brugada ECG. This case is rare combination of Brugada syndrome with ASD.

Abstract 1825: Abnormal ECG Markers suggest that Accentuated Intracardiac Conduction Delay is The Common and Distinguishing Feature of Patients with Spontaneous Type 1 ECG or SCN5A Mutation in Brugadas Syndrome

Circulation ◽  
2007 ◽  
Vol 116 (suppl_16) ◽  
Author(s):  
Juliane Theilade ◽  
Raffaella Bloise ◽  
Janny Nastoli ◽  
Elena Ronchetti ◽  
S. Facchinetti ◽  
...  
Keyword(s):  
Brugada Syndrome ◽  
Relative Size ◽  
Conduction Delay ◽  
List Type ◽  
Late Potentials ◽  
Loss Of Function ◽  
Intraventricular Conduction ◽  
Qrs Duration ◽  
Scn5a Mutation

The conclusive diagnosis of Brugada Syndrome (BrS) is established in patients (pts) with spontaneous type 1 ECG or in carriers of a loss of function SCN5A mutation. We carried out a systematic analysis of ECG recordings in BrS pts to test the hypothesis that the presence of intraventricular (Intra-Ven) and atrioventricular (A-Ven) conduction delay is the distinguishing common feature of pts with SCN5A mutations (Mut+) and in pts with a spontaneous type 1 ECG pattern (spont-ECG). We assessed the following 5 ECG parameters: late potentials (quantified as RMS at 40 Hz), aVR sign (relative size of the R and q waves in aVR), QRS complex fragmentation (defined as an additional R′ or notching of the of S wave), PQ and QRS duration. We studied 200 consecutive BrS pts to define whether the prevalence of ECG markers correlate with 1) the presence of a SCN5A mutation (Mut+) or 2) the presence of a spontaneous type 1 pattern ECG (spont-ECG). Results are summarized in the table . Interestingly, Mut+ not only presented prolonged PQ (p<0.003 vs Mut÷) as previously reported but more significantly they showed the presence of abnormal markers of intraventricular conduction (QRS duration, aVR sign and late potentials). Similarly, also the presence of a spont-ECG was associated with prolonged PQ and QRS intervals and with late potentials. Based on our ECG analysis we propose that Brugada syndrome is a disease characterized by impairment of A-Ven and Intra-Ven conduction, the presence of a mutation in the SCN5A gene and, the presence of a spontaneous type 1 ECG identify patients with accentuated conduction defects. These findings suggest that the presence of interventricular conduction delay is a landmark of BrS and should be considered as diagnostic markers in BrS.

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

Circulation ◽  
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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