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.

P6586Brugada syndrome risk stratification - Evaluation of clinical and ECG risk markers in a multicenter international primary prevention cohort

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
S Honarbakhsh ◽  
R Providencia ◽  
J Garcia-Hernandez ◽  
C A Martin ◽  
R J Hunter ◽  
...  
Keyword(s):  
Sudden Cardiac Death ◽  
Risk Stratification ◽  
Risk Score ◽  
Cardiac Death ◽  
International Study ◽  
Risk Markers ◽  
History Of ◽  
Score Model

Abstract Background Risk stratification for sudden cardiac death (SCD) in Brugada syndrome (BrS) is a significant challenge. Purpose To evaluate the importance of clinical and ECG factors in the likelihood of developing significant ventricular arrhythmias (VAs)/SCD in BrS patients. Methods VA occurrence during follow-up were assessed and the role of 16 proposed clinical or ECG risk markers evaluated in a multicenter international study of BrS patients and no history of cardiac arrest. Markers with predictive power were identified and incorporated into a risk score model. Results Across 15 international centers, 1084 patients were included. During a follow-up of 5.3 years (IQR 2.7–9.0 years)- 110 patients had VA occurrence (10.1%) with an annual event rate of 1.7% (95% CI 1.4–2.0). Of the 16 proposed risk factors, diagnosis by family screening of sudden cardiac death (HR 4.65; p<0.001), probable arrhythmia related syncope (HR 3.88, p<0.001), type 1 spontaneous ECG (HR 3.56; p<0.001), Early Repolarisation (HR 3.15; p<0.001) and type 1 Brugada pattern in peripheral leads (HR 2.42; p<0.001) were associated with a higher VA occurrence risk during follow-up. These 5 variables were incorporated into a risk score model whereby each variable was allocated a point score based on the variable's predictive strength. The total points obtained from the model for a patient could then be translated into the predicted VA occurrence risk during follow-up (Figure 1). The model showed a sensitivity of 63.5% (95% CI 50.0–76.9) and specificity of 84.2% (95% CI 81.1–87.1) in predicting VA occurrence at 5-years follow-up. The model showed a greater discriminative power compared to an existing model (AUC 0.83 vs. 0.71; p<0.001). Figure 1 Conclusions This multicenter study with longest reported follow-up to date identified 5 risk markers for VA occurrence. Utilizing these markers in a risk score model can aid BrS risk stratification to enable individualized risk prediction and ICD prescription.

scholarly journals Risk Stratification in Brugada Syndrome: Current Status and Emerging Approaches

2018 ◽  
Vol 7 (2) ◽  
pp. 79 ◽  
Author(s):  
Shohreh Honarbakhsh ◽  
Rui Providencia ◽  
Pier D Lambiase ◽  
◽  
◽  
...  
Keyword(s):  
High Risk ◽  
Sudden Cardiac Death ◽  
Risk Stratification ◽  
Brugada Syndrome ◽  
Cardiac Death ◽  
Right Ventricular Outflow Tract ◽  
Current Status ◽  
Drug Induced ◽  
Increased Risk

Brugada syndrome (BrS) is one of the most common inherited channelopathies associated with an increased risk of sudden cardiac death. Appropriate use of an ICD in high-risk patients is life-saving. However, there remains a lack of consensus on risk stratification, and even on the diagnosis of BrS itself. Some argue that people with a type 1 Brugada ECG pattern but no symptoms should not be diagnosed with BrS, and guidelines recommend observation without therapy in these patients. Others argue that the presence of a spontaneous (rather than drug-induced) type 1 ECG pattern alone is enough to label them as high-risk for arrhythmic events, particularly if syncope is also present. Syncope and a spontaneous type 1 ECG pattern are the only factors that have consistently been shown to predict ventricular arrhythmic events and sudden cardiac death. Other markers have yielded conflicting data. However, in combination they may have roles in risk scoring models. Epicardial catheter ablation in the right ventricular outflow tract has shown promise in studies as an alternative management option to an ICD, but longer follow-up is required to ensure that the ablation effect is permanent.

scholarly journals Sudden Cardiac Death in Hereditary Dilated Cardiomyopathy

2020 ◽  
Author(s):  
Marianna Leopoulou ◽  
Jo Ann LeQuang ◽  
Joseph V. Pergolizzi ◽  
Peter Magnusson
Keyword(s):  
Sudden Cardiac Death ◽  
Left Ventricle ◽  
Sudden Death ◽  
Risk Stratification ◽  
Dilated Cardiomyopathy ◽  
Cardiac Death ◽  
Disease Risk ◽  
Systolic Dysfunction ◽  
Preventive Strategies ◽  
Genetic Origin

Dilated cardiomyopathy (DCM) is characterized by the phenotype of a dilated left ventricle with systolic dysfunction. It is classified as hereditary when it is deemed of genetic origin; more than 50 genes are reported to be related to the condition. Symptoms include, among others, dyspnea, fatigue, arrhythmias, and syncope. Unfortunately, sudden cardiac death may be the first manifestation of the disease. Risk stratification regarding sudden death in hereditary DCM as well as preventive management poses a challenge due to the heterogeneity of the disease. The purpose of this chapter is to present the epidemiology, risk stratification, and preventive strategies of sudden cardiac death in hereditary DCM.

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.

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 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.

scholarly journals Outcome of Insertable Cardiac Monitors in Symptomatic Patients with Brugada Syndrome at Low Risk of Sudden Cardiac Death

Cardiology ◽  
2020 ◽  
Vol 145 (7) ◽  
pp. 413-420
Author(s):  
Rafi Sakhi ◽  
Amira Assaf ◽  
Dominic A.M.J. Theuns ◽  
Judith M.A. Verhagen ◽  
Tamas Szili-Torok ◽  
...  
Keyword(s):  
Sudden Cardiac Death ◽  
Ventricular Arrhythmia ◽  
Brugada Syndrome ◽  
Cardiac Death ◽  
Ventricular Arrhythmias ◽  
Electrophysiological Study ◽  
Pacemaker Implantation ◽  
Low Risk ◽  
History Of

Introduction: There is limited data on the experience with insertable cardiac monitors (ICMs) in patients with Brugada syndrome. Objective: To evaluate the outcome of ICM in symptomatic patients with Brugada syndrome who are at suspected low risk of sudden cardiac death (SCD). Methods: We conducted a prospective single-center cohort study including all symptomatic patients with Brugada syndrome who received an ICM (Reveal LINQ) between July 2014 and October 2019. The main indication for monitoring was to exclude ventricular arrhythmias as the cause of symptoms and to establish a symptom-rhythm relationship. Results: A total of 20 patients (mean age, 39 ± 12 years; 55% male) received an ICM during the study period. Nine patients (45%) had a history of syncope (presumed nonarrhythmogenic), and 5 patients had a recent syncope (<6 months). During a median follow-up of 32 months (interquartile range, 11–36 months), 3 patients (15%) experienced an episode of nonsustained ventricular arrhythmia. No patient died suddenly or experienced a sustained ventricular arrhythmia, and no patient had a recurrence of syncope. Overall, 17 patients (85%) experienced symptoms during follow-up, of whom 10 patients had an ICM-detected arrhythmia. In 4 patients (20%), the ICM-detected arrhythmia was an actionable event. ICM-guided management included antiarrhythmic drug therapy for symptomatic ectopic beats (n = 3), pulmonary vein isolation, and oral anticoagulation for atrial fibrillation (n = 1), electrophysiological study for risk stratification (n = 1), and pacemaker implantation for atrioventricular block (n = 1). Conclusions: An ICM can be used to exclude ventricular arrhythmias in symptomatic patients with Brugada syndrome at low risk of SCD. Furthermore, an ICM-detected arrhythmia changed clinical management in 20% of patients.

scholarly journals Transient Type 1 Brugada Pattern without Ongoing Fever during COVID-19 Pneumonia

2020 ◽  
Author(s):  
Maria Cristina Pasqualetto ◽  
Andrea Corrado ◽  
Eleonora Secco ◽  
Fabio Graceffa ◽  
Fausto Rigo
Keyword(s):  
Family History ◽  
Sudden Cardiac Death ◽  
Adverse Effects ◽  
Cardiac Death ◽  
Antiviral Drugs ◽  
Electrolyte Disturbance ◽  
History Of ◽  
Brugada Pattern

Patients with COVID-19 pneumonia can develop heart problems and may also may be susceptible to proarrhythmia, virus-related issues such as fever, stress, electrolyte disturbance and adverse effects from the use of antiviral drugs. We report a transient Brugada-like ECG pattern without ongoing fever in a 57-year-old man, admitted with a diagnosis of COVID-19 pneumonia, who did not have syncope or a family history of sudden cardiac death.

scholarly journals GENETIC REASONS OF SUDDEN CARDIAC DEATH

2017 ◽  
Vol 19 (2) ◽  
pp. 15-22
Author(s):  
S N Kolyubaeva
Keyword(s):  
Ventricular Tachycardia ◽  
Sudden Cardiac Death ◽  
Long Qt Syndrome ◽  
Brugada Syndrome ◽  
Cardiac Death ◽  
Polymorphic Ventricular Tachycardia ◽  
Normal Heart ◽  
Short Qt Syndrome ◽  
Long Qt ◽  
Qt Syndrome

The review presents the recent data on genetic reasons of sudden cardiac death. Mutations discuss in gens associated with sudden cardiac death. Channalopathies, such as Brugada syndrome, long QT syndrome, short QT syndrome and catecholaminergic polymorphic ventricular tachycardia are characterized by arrhythmias in normal heart resulting from genetic anomalies in ion channels

Sign in / Sign up

Export Citation Format

Share Document