Clinical Characteristics and Risk Stratification in Symptomatic and Asymptomatic Patients with Brugada Syndrome: Multicenter Study in Japan

Background Neither the clinical characteristics nor risk stratification of symptomatic and asymptomatic patients with Brugada syndrome have been clearly determined. We compared clinical and ECG characteristics of symptomatic and asymptomatic patients with Brugada syndrome to identify new markers for distinguishing high- from low-risk patients. Methods A total of 216 consecutive individuals with Brugada syndrome (mean age 52±14 years, 197 males) were enrolled in the Japan Idiopathic Ventricular Fibrillation Study (J-IVFS). Clinical and ECG characteristics were compared among 3 groups of patients: VF group; patients with aborted sudden death and documented VF (N=34), Syncope (Sy) group; patients with syncope without documented VF (N=70), and Asymptomatic (As) group; subjects without symptoms (N=112). Comparisons were made among the 3 groups as well as between the symptomatic (VF/Sy) and asymptomatic (As) groups. Short-term prognosis was also compared among the 3 groups, and between the VF/Sy and As groups. Results 1) Clinical characteristics: incidence of past history of AF was significantly higher in the VF and Sy groups than in the AS group (26, 26, and 12 %, respectively; [p=0.04]), though no other clinical parameters differed among the groups. 2) On resting 12-lead ECG, r-J interval (interval from QRS onset to J point) in lead V2 and QRS duration in lead V6 were highest in the VF group (104, 98, and 92 msec in V2 [p<0.001]; 106, 103, and 94 msec in V6 [p<0.0001], respectively, VF vs. Sy vs. As). 3) Positive late potential and inducibility of VF by EPS did not differ in incidence among the 3 groups. 4) Clinical follow-up: during a mean follow-up of 36±16 months, incidence of cardiac events (sudden death and/or VF) was higher in the VF/Sy groups than in the As group (29, 8, and 0 %, respectively [p<0.001]). Multivariate analysis showed that the frequencies of r-J interval ≥ 90 msec in lead V2 and QRS duration ≥ 90 msec in lead V6 were significantly higher in patients with cardiac events (p=0.02, 0.02, respectively). Conclusions In symptomatic patients, prolonged ventricular depolarization in precordial leads of the ECG was prominent in the VF group, and this sign can be used to distinguish high- from low-risk patients with Brugada syndrome.

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Risk Stratification in Brugada Syndrome: Clinical Characteristics, Electrocardiographic Parameters and Auxiliary Testing

J Wave Syndromes ◽

10.1007/978-3-319-31578-2_8 ◽

2016 ◽

pp. 173-191

Author(s):

Arnon Adler ◽

Sami Viskin

Keyword(s):

Risk Stratification ◽

Brugada Syndrome ◽

Clinical Characteristics ◽

Electrocardiographic Parameters

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Clinical characteristics and long-term clinical course of patients with Brugada syndrome without previous cardiac arrest: a multiparametric risk stratification approach

EP Europace ◽

10.1093/europace/euz288 ◽

2019 ◽

Cited By ~ 3

Author(s):

Konstantinos P Letsas ◽

George Bazoukis ◽

Michael Efremidis ◽

Stamatis Georgopoulos ◽

Panagiotis Korantzopoulos ◽

...

Keyword(s):

Risk Factors ◽

Risk Stratification ◽

Brugada Syndrome ◽

Clinical Characteristics ◽

Clinical Course ◽

Electrophysiological Study ◽

Rank Test ◽

History Of ◽

Event Rates

Abstract Aims Risk stratification in Brugada syndrome (BrS) still represents an unsettled issue. In this multicentre study, we aimed to evaluate the clinical characteristics and the long-term clinical course of patients with BrS. Methods and results A total of 111 consecutive patients (86 males; aged 45.3 ± 13.3 years) diagnosed with BrS were included and followed-up in a prospective fashion. Thirty-seven patients (33.3%) were symptomatic at enrolment (arrhythmic syncope). An electrophysiological study (EPS) was performed in 59 patients (53.2%), and ventricular arrhythmias were induced in 32 (54.2%). A cardioverter defibrillator was implanted in 34 cases (30.6%). During a mean follow-up period of 4.6 ± 3.5 years, appropriate device therapies occurred in seven patients. Event-free survival analysis (log-rank test) showed that spontaneous type-1 electrocardiogram pattern (P = 0.008), symptoms at presentation (syncope) (P = 0.012), family history of sudden cardiac death (P < 0.001), positive EPS (P = 0.024), fragmented QRS (P = 0.004), and QRS duration in lead V2 > 113 ms (P < 0.001) are predictors of future arrhythmic events. Event rates were 0%, 4%, and 60% among patients with 0–1 risk factor, 2–3 risk factors, and 4–5 risk factors, respectively (P < 0.001). Current multiparametric score models exhibit an excellent negative predictive value and perform well in risk stratification of BrS patients. Conclusions Multiparametric models including common risk factors appear to provide better risk stratification of BrS patients than single factors alone.

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564 Controversial role of intracardiac eletrophysiology study in Brugada syndrome: analysis of a single-centre retrospective cohort study

European Heart Journal Supplements ◽

10.1093/eurheartj/suab127.014 ◽

2021 ◽

Vol 23 (Supplement_G) ◽

Author(s):

Claudio Licciardello ◽

Jacopo Marazzato ◽

Michele Golino ◽

Francesca Seganfreddo ◽

Federica Matteo ◽

...

Keyword(s):

High Risk ◽

Risk Stratification ◽

Right Ventricular ◽

Brugada Syndrome ◽

Type I ◽

Drug Induced ◽

Programmed Ventricular Stimulation ◽

Asymptomatic Patients ◽

Ventricular Stimulation

Abstract Aims According to European guidelines, aborted sudden cardiac death (SCD) in Brugada syndrome (BrS) is regarded as a I class recommendation for secondary prevention implantable cardioverter defibrillator (ICD). However, the risk stratification of BrS patients for primary prevention ICD still represents a clinical conundrum. Although intracardiac electrophysiology (EP) study proved useful for the selection of high-risk patients in this setting. Therefore, aim of this study was to assess all clinical and EP variables associated with the induction of VA at EP study and the rate of appropriate/inappropriate ICD interventions and/or clinical SCD events in these patients occurring at follow-up. Methods and results From 2001 to 2021, all EP studies performed in symptomatic/asymptomatic patients (46 ± 14 years, M 88%) with/without family history of SCD spontaneous/drug-induced type I pattern (TIP) on ECG and no spontaneous ventricular arrhythmias were retrospectively considered at our study centre. Clinical variables, BrS pattern, EP study data (including right ventricular site and type of stimulation protocol), and ICD interventions (DC-shocks or Anti-Tachycardia Pacing events, ATP) and/or SCD events occurring at follow-up were all evaluated. EP study was deemed positive for any polymorphic VA induced during programmed ventricular stimulation; non-sustained episodes included. ICD was routinely implanted in all patients with a positive EP study. Follow-up data were detected by the collection of medical and home-monitoring recordings at study-site level. Follow-up data were available in 50 patients (9 ± 6 years on average). Patients were generally young with few cardiovascular comorbidities. SCD history was known in 21 (42%) with a significant number of asymptomatic patients (48%). Br patterns were equally distributed in the investigated population (spontaneous and drug-induced TIP in 52% and 48%, respectively) and AF history was fairly common (16%). In the study population, EP study tested positive in 30 patients (60%): spontaneous TIP (P = 0.0518), few extrastimuli during programmed ventricular stimulation (P = 0.0015), and right ventricular stimulation at the apical site (P ≤ 0.0001) were the only variables to be clearly associated with a positive EP study in the appraised patients. At follow-up, appropriate ICD shocks were documented in 4 out of 30 implanted patients (13%) at generally 5 ± 7 years from EP study evaluation. Although three ICD interventions (75%) occurred in patients with spontaneous TIP, one patient with drug-induced TIP pattern and positive EP study referred to Emergency Department for unrelenting VT storm after roughly 13 years from ICD implantation. Inappropriate ICD interventions for fast rate AF were detected in 10% of cases. Finally, no SCD events were documented at follow up in patients with a negative EP study. Conclusions In a retrospective analysis, EP study proved useful in the risk stratification of SCD in BrS patients. A few ventricular extrastimuli delivered at the right ventricular apex seem sufficient to prompt the induction of life-threatening VA in high-risk BrS patients during EP study. Moreover, in this setting, a negative EP study seems protective against the development of VA/SCD events at follow-up. However, not only is spontaneous TIP associated with an increased risk of arrhythmic death, but a drug-induced TIP, generally regarded as a low-risk condition, might also be associated with a long-term hazard of SCD in these patients.

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Risk Stratification for Asymptomatic Patients With Brugada Syndrome

Circulation Journal ◽

10.1253/circj.67.312 ◽

2003 ◽

Vol 67 (4) ◽

pp. 312-316 ◽

Cited By ~ 49

Author(s):

Hiroshi Morita ◽

Shiho Takenaka-Morita ◽

Kengo Fukushima-Kusano ◽

Makoto Kobayashi ◽

Satoshi Nagase ◽

...

Keyword(s):

Risk Stratification ◽

Brugada Syndrome ◽

Asymptomatic Patients

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Risk stratification in asymptomatic patients with Brugada syndrome: Utility of multiple risk factor combination rather than programmed electrical stimulation

Journal of Cardiovascular Electrophysiology ◽

10.1111/jce.14848 ◽

2020 ◽

Author(s):

Tetsuji Shinohara ◽

Masahiko Takagi ◽

Tsukasa Kamakura ◽

Yukio Sekiguchi ◽

Yasuhiro Yokoyama ◽

...

Keyword(s):

Risk Factor ◽

Electrical Stimulation ◽

Risk Stratification ◽

Brugada Syndrome ◽

Multiple Risk Factor ◽

Programmed Electrical Stimulation ◽

Asymptomatic Patients ◽

Factor Combination ◽

Multiple Risk

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Current Controversies and Challenges in Brugada Syndrome

European Cardiology Review ◽

10.15420/ecr.2019.12.2 ◽

2019 ◽

Vol 14 (3) ◽

pp. 169-174 ◽

Cited By ~ 2

Author(s):

Afik D Snir ◽

Hariharan Raju

Keyword(s):

Risk Stratification ◽

Brugada Syndrome ◽

Diagnostic Pathway ◽

Asymptomatic Patients ◽

Initial Description ◽

Shed Light ◽

Risk Modification

More than three decades since its initial description in 1993, Brugada syndrome remains engulfed in controversy. This review aims to shed light on the main challenges surrounding the diagnostic pathway and criteria, risk stratification of asymptomatic patients, pharmacological and interventional risk modification strategies as well as our current pathophysiological understanding of the disease.

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An Open Invitation to Join the International Brugada Electrocardiographic Indices Registry

Cardiovascular Innovations and Applications ◽

10.15212/cvia.2019.0568 ◽

2020 ◽

Vol 4 (3) ◽

pp. 217-221

Author(s):

Gary Tse ◽

Sharen Lee ◽

Xuan Jiang ◽

Dong Chang ◽

Yunfei Gu ◽

...

Keyword(s):

Ventricular Tachycardia ◽

Ventricular Fibrillation ◽

Risk Stratification ◽

Brugada Syndrome ◽

Additional Information ◽

Data Registry ◽

Asymptomatic Patients ◽

Baseline Characteristics ◽

Electrocardiogram Ecg ◽

Repolarization Abnormalities

Background: The Brugada Electrocardiographic Indices Registry is a comprehensive data registry composed of patients with Brugada patterns on the electrocardiogram (ECG). The aim is to test the hypotheses that (i) ECG indices combining both depolarization and repolarization abnormalities can better predict spontaneous ventricular arrhythmias than existing ECG markers in Brugada syndrome and (ii) that serial ECG measurements will provide additional information for risk stratification, especially in asymptomatic patients.Methods: Patients with both Brugada pattern ECGs and Brugada syndrome are eligible for inclusion in this registry. Baseline characteristics and ECG variables reflecting depolarization and repolarization will be determined. The primary outcome is spontaneous ventricular tachycardia/ventricular fibrillation or sudden cardiac death. Secondary outcomes are inducible ventricular tachycardia/ventricular fibrillation and syncope.Results: As of November 15, 2019, 39 investigators from 32 cities in 18 countries had joined this registry. As of December 15, 2019, 1383 cases had been enrolled.Conclusions: The Brugada Electrocardiographic Indices Registry will evaluate the disease life course, risk factors, and prognosis in a large series of Brugada patients. It will therefore provide insights for improving risk stratification.

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