Long-Term Prognosis of Patients Diagnosed With Brugada Syndrome: Results From the FINGER Brugada Syndrome Registry
Open accessAbstract
BACKGROUND: Brugada syndrome is characterized by ST-segment elevation in the right precordial leads and an increased risk of sudden cardiac death (SCD). Fundamental questions remain on the best strategy for assessing the real disease-associated arrhythmic risk, especially in asymptomatic patients. The aim of the present study was to evaluate the prognosis and risk factors of SCD in Brugada syndrome patients in the FINGER (France, Italy, Netherlands, Germany) Brugada syndrome registry. METHODS AND RESULTS: Patients were recruited in 11 tertiary centers in 4 European countries. Inclusion criteria consisted of a type 1 ECG present either at baseline or after drug challenge, after exclusion of diseases that mimic Brugada syndrome. The registry included 1029 consecutive individuals (745 men; 72%) with a median age of 45 (35 to 55) years. Diagnosis was based on (1) aborted SCD (6%); (2) syncope, otherwise unexplained (30%); and (3) asymptomatic patients (64%). During a median follow-up of 31.9 (14 to 54.4) months, 51 cardiac events (5%) occurred (44 patients experienced appropriate implantable cardioverter-defibrillator shocks, and 7 died suddenly). The cardiac event rate per year was 7.7% in patients with aborted SCD, 1.9% in patients with syncope, and 0.5% in asymptomatic patients. Symptoms and spontaneous type 1 ECG were predictors of arrhythmic events, whereas gender, familial history of SCD, inducibility of ventricular tachyarrhythmias during electrophysiological study, and the presence of an SCN5A mutation were not predictive of arrhythmic events. CONCLUSIONS: In the largest series of Brugada syndrome patients thus far, event rates in asymptomatic patients were low. Inducibility of ventricular tachyarrhythmia and family history of SCD were not predictors of cardiac events.
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A history of syncope in patients with structural heart disease or inheritable arrhythmia syndromes is associated with a two- to four-fold increased risk of death, but this varies between specific conditions.
“In general, a history of syncope in patients with structural heart disease or inheritable arrhythmia syndromes is associated with a two- to four-fold increased risk of death, 348,350-353 but varies between specific conditions. 354-356”
From 2018 ESC Guidelines for the diagnosis and management of syncope (2018) · cites this paper as [353]
The Multivariable Analysis reports that patients with Brugada syndrome and syncope had a 3.4-fold higher hazard of a first arrhythmic event than asymptomatic patients. The Follow-Up Data show that these events included appropriate ICD shocks as well as sudden cardiac deaths. The paper therefore supports the magnitude for one inherited arrhythmia syndrome, but not a two- to four-fold risk of death across structural heart disease and inherited arrhythmia syndromes generally. (LLM, conf=0.88)
Experimental — accuracy not yet measuredIn the largest registry of 1029 patients, the incidence of arrhythmic events (sustained ventricular tachycardia or ventricular fibrillation, appropriate ICD therapy, or sudden death) in patients with Brugada syndrome was 7.7% per year in those with a history of sudden cardiac arrest, 1.9% per year with syncope, and 0.5% per year in asymptomatic patients.
“In the largest registry (1029 patients), the incidence of arrhythmic events (sustained VT or VF, appropriate ICD therapy, or sudden death) in patients with Brugada syndrome was 7.7% per year in those with a history of sudden cardiac arrest, 1.9% per year with syncope, and 0.5% per year in asymptomatic patients. 353”
From 2018 ESC Guidelines for the diagnosis and management of syncope (2018) · cites this paper as [353]
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Experimental — accuracy not yet measuredA history of syncope may increase the risk of arrhythmic events up to two- to three-fold compared with asymptomatic patients in Brugada syndrome.
“Ahistory of syncope may increase the risk of arrhythmic events up to two- to three-fold compared with that in asymptomatic patients.”
From 2018 ESC Guidelines for the diagnosis and management of syncope (2018) · cites this paper as [353]
The Symptoms and Multivariable Analysis sections report that patients with syncope had more arrhythmic events than asymptomatic patients, supporting the direction of the claim. The reported hazard ratio is 3.4, however, rather than the claimed upper range of two- to three-fold; the Follow-Up Data also give annual event rates of 1.9% versus 0.5%. No page numbers are available in our copy. (LLM, conf=0.86)
Experimental — accuracy not yet measured
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Self-citation
- Authors
- Probst, V., Veltmann, C., Eckardt, L., Meregalli, P. G., Gaita, F., Tan, H. L., Babuty, D., Sacher, F., Giustetto, C., Schulze-Bahr, E., Borggrefe, M., Haissaguerre, M., Mabo, P., Le Marec, H., Wolpert, C., & Wilde, A. A. M.
- Affiliations
- Inserm (FR)
- From INSERM, UMR915, Nantes, France (V.P., H.L.M.); Université de Nantes, l’institut du thorax, Nantes, France (V.P., H.L.M.); CHU Nantes, l’institut du thorax, Service de cardiologie, Nantes, France (V.P., H.L.M.); First Department of Medicine, Cardiology, University Hospital Mannheim, Mannheim, Germany (C.V., M.B., C.W.); University Hospital of Muenster, Department of Cardiology and Angiology, Muenster, Germany (L.E., E.S.-B.); Department of Cardiology, Academic Medical Center, University of...
- ORCID
- Venue
- Circulation
- Published
- 2010-02-09
- Volume
- 121
- Issue
- 5
- Pages
- 635-643
- ISSN
- 0009-7322 (ISSN-L)
- 1524-4539
- OpenAlex
- W2128734390
- Cited by
- 847
- References
- 37
- Citations by year
- 2026: 19 · 2025: 35 · 2024: 42 · 2023: 38 · 2022: 45 · 2021: 46 · 2020: 50 · 2019: 45 · 2018: 55 · 2017: 73 · 2016: 64 · 2015: 60 · 2014: 39 · 2013: 70 · 2012: 75
- FWCI
- 50.93
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Locations
- Circulation (publishedVersion)
- PubMed (publishedVersion)
- Institutional Research Information System University of Turin (University of Turin) (submittedVersion, other-oa)
- Data Archiving and Networked Services (DANS) (submittedVersion)
- HAL (submittedVersion, other-oa)
- Pure Amsterdam UMC (submittedVersion)
- Funders
- Agence Nationale de la Recherche
- Nederlandse Organisatie voor Wetenschappelijk Onderzoek
- Centre Hospitalier Universitaire de Nantes
- Topics
- Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Cardiac electrophysiology and arrhythmias
- Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Cardiovascular Effects of Exercise
- Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Cardiac Arrhythmias and Treatments
- Keywords
- Brugada syndrome, sudden cardiac death, long-term prognosis, asymptomatic patients, syncope, implantable cardioverter-defibrillator shocks, SCN5A mutation, family history of sudden cardiac death
- MeSH terms
- Adult, Death, Electrocardiography, Female, Follow-Up Studies, Humans, Male, Middle Aged, Prognosis, Registries, Ventricular Fibrillation, Brugada Syndrome
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