English Polski
Vol 17, No 2 (2022)
Published online: 2022-04-29

open access

Page views 4573
Article views/downloads 299
Get Citation

Connect on Social Media

Connect on Social Media

Commentary to case report “Brugada syndrome: rare so, beware!” by Paweł Maciński et al.

Michał M. Farkowski1
Folia Cardiologica 2022;17(2):127-128.


Not available


Folia Cardiologica 2022

vol. 17, no. 2, pages 127–128

DOI: 10.5603/FC.2022.0018

Copyright © 2022 Via Medica

ISSN 2353–7752

e-ISSN 2353–7760

Michał M. Farkowski MDPhD
Second Department of Cardiac Arrhythmia, National Institute of Cardiology, Warsaw, Poland

Commentary to case report “Brugada syndrome: rare so, beware!” by Paweł Maciński et al.


Address for correspondence: Michał M. Farkowski MD, PhD, Narodowy Instytut Kardiologii w Warszawie, ul. Alpejska 42, 04–628 Warszawa, Poland, e-mail: mfarkowski@gmail.com

This article is available in open access under Creative Common Attribution-Non-Commercial-No Derivatives 4.0 International (CC BY-NC-ND 4.0) license, allowing to download articles and share them with others as long as they credit the authors and the publisher, but without permission to change them in any way or use them commercially.

Brugada syndrome (BS) is a genetic disorder of ion channels resulting in their dysfunction that may lead to induction of malignant ventricular arrhythmia and sudden cardiac death [1]. The disease is diagnosed based on typical QRS complex morphology in the precordial leads in resting conditions (spontaneous type 1 Brugada pattern) or during provocative testing, although more complex definitions of BS have also been proposed [1, 2].

Multiple controversies have been related to BS, from the underlying pathophysiology through stratification of sudden cardiac death risk (including the role of programmed ventricular stimulation and electrophysiologic study [EPS]) to optimal therapy, currently with an implantable cardioverter-defibrillator (ICD) [1, 3–5]. Patient selection for ICD therapy is based on individual risk stratification but the latter remains challenging, as evidenced by the sheer number of risk factors and available prediction models. In case of a documented cardiac arrest in a patient with diagnosed BS, the situation is straightforward, as ICD is the treatment of choice for secondary prevention of sudden cardiac death [1]. In other cases, the decision may be difficult. Both the European Society of Cardiology (ESC) guidelines and two well-documented risk scores for BS (Sieira score, BRUGADA-RISK) highlight the presence of spontaneous type 1 electrocardiographic (ECG) pattern and syncope of probable arrhythmic aetiology as risk factors for future arrhythmic events [1, 5, 6]. In contrast, asymptomatic patients without spontaneous type 1 ECG pattern are usually in the “grey zone”. The score developed by Sieira et al. [5] includes spontaneous type 1 ECG pattern, family history of early cardiac death (both scoring 1 point), induction of ventricular arrhythmia during EPS, syncope (both scoring 2 points), sinus node dysfunction (3 points), and aborted sudden cardiac death (4 points). Scores ≥2 were associated with a significantly higher risk of an arrhythmic event, and the risk increased with the overall score [5]. The BRUGADA-RISK model is based on somewhat different variables and includes typical spontaneous type 1 ECG pattern, spontaneous type 1 ECG pattern in the limb leads, early repolarization pattern on ECG, and syncope of probable arrhythmic aetiology. Various combinations of these risk factors determine the individual patient risk profile but each of these variables is associated with an increased risk of an arrhythmic event [6]. Clinical scores are not ideal. A validation attempt of the Sieira score by another group showed that as many as 75% of patients with an arrhythmic event in that population would not receive an ICD based on risk stratification using the Sieira score [4, 5].

Due to a reiterating role of syncope as a prognostic factor in BS, efforts should be made to evaluate the precise nature of syncopal events and exclude potential non-arrhythmic causes, such as reflex syncope, hypotension or atrioventricular block. In selected cases, implantation of an event recorder may clarify the situation and protect the patient from unnecessary ICD implantation [7, 8].

The value of EPS in the stratification of arrhythmic risk in BS is unclear [1, 5, 6, 9]. According to the ESC guidelines, induction of ventricular arrhythmia during EPS may be one factor to be considered when selecting patients for ICD therapy. In the Sieira score, 2 points are assigned for a positive EPS result, thus placing the patient at an increased risk. The BRUGADA-RISK score does not include EPS, as induction of ventricular arrhythmia was not shown to be a significant prognostic factor even in univariate analysis. It seems, however, that this diagnostic tool should not be discarded completely. In an analysis of data on 1312 patients from 8 studies, an association was found between arrhythmia induction during EPS and a history of an arrhythmic event, but the predictive value of EPS was strongly related to typical risk factors in BS: spontaneous type 1 ECG pattern and a history of syncope. It seems that the risk of arrhythmic events in patients without these risk factors and a negative EPS result is minimal [9].

Appropriate patient selection for ICD therapy protects high-risk individuals from an arrhythmic death, while those who do not require an ICD may be spared major complications of device therapy such as death, infective endocarditis, infection of the device pocket, inappropriate interventions, depression etc. [1, 10].


  1. Priori SG, Blomström-Lundqvist C, Mazzanti A, et al. Task Force for the Management of Patients with Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death of the European Society of Cardiology (ESC). 2015 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death: The Task Force for the Management of Patients with Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death of the European Society of Cardiology (ESC) endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC). Europace. 2015; 17(11): 1601–1687, doi: 10.1093/europace/euv319, indexed in Pubmed: 26318695.
  2. Antzelevitch C, Yan GX, Ackerman MJ, et al. J-Wave syndromes expert consensus conference report: emerging concepts and gaps in knowledge. Heart Rhythm. 2016; 13(10): e295–e324, doi: 10.1016/j.hrthm.2016.05.024, indexed in Pubmed: 27423412.
  3. Behr ER, Ben-Haim Y, Ackerman MJ, et al. Brugada syndrome and reduced right ventricular outflow tract conduction reserve: a final common pathway? Eur Heart J. 2021; 42(11): 1073–1081, doi: 10.1093/eurheartj/ehaa1051, indexed in Pubmed: 33421051.
  4. Chow JJ, Leong KMW, Yazdani M, et al. A multicenter external validation of a score model to predict risk of events in patients with Brugada syndrome. Am J Cardiol. 2021; 160: 53–59, doi: 10.1016/j.amjcard.2021.08.035, indexed in Pubmed: 34610873.
  5. Sieira J, Conte G, Ciconte G, et al. A score model to predict risk of events in patients with Brugada Syndrome. Eur Heart J. 2017; 38(22): 1756–1763, doi: 10.1093/eurheartj/ehx119, indexed in Pubmed: 28379344.
  6. Honarbakhsh S, Providencia R, Garcia-Hernandez J, et al. Brugada Syndrome Risk Investigators. A primary prevention clinical risk score model for patients with Brugada Syndrome (BRUGADA-RISK). JACC Clin Electrophysiol. 2021; 7(2): 210–222, doi: 10.1016/j.jacep.2020.08.032, indexed in Pubmed: 33602402.
  7. Mascia G, Bona RD, Ameri P, et al. Brugada syndrome and syncope: a practical approach for diagnosis and treatment. Europace. 2021; 23(7): 996–1002, doi: 10.1093/europace/euaa370, indexed in Pubmed: 33367713.
  8. Scrocco C, Ben-Haim Y, Devine B, et al. Role of subcutaneous implantable loop recorder for the diagnosis of arrhythmias in Brugada syndrome: a United Kingdom single-center experience. Heart Rhythm. 2022; 19(1): 70–78, doi: 10.1016/j.hrthm.2021.08.034, indexed in Pubmed: 34487893.
  9. Sroubek J, Probst V, Mazzanti A, et al. Programmed ventricular stimulation for risk stratification in the Brugada syndrome: a pooled analysis. Circulation. 2016; 133(7): 622–630, doi: 10.1161/CIRCULATIONAHA.115.017885, indexed in Pubmed: 26797467.
  10. Blomstrom-Lundqvist C, Traykov V, Erba PA, et al. European Heart Rhythm Association (EHRA) international consensus document on how to prevent, diagnose, and treat cardiac implantable electronic device infections-endorsed by the Heart Rhythm Society (HRS), the Asia Pacific Heart Rhythm Society (APHRS), the Latin American Heart Rhythm Society (LAHRS), International Society for Cardiovascular Infectious Diseases (ISCVID) and the European Society of Clinical Microbiology and Infectious Diseases (ESCMID) in collaboration with the European Association for Cardio-Thoracic Surgery (EACTS). Europace. 2020; 22(4): 515–549, doi: 10.1093/europace/euz246, indexed in Pubmed: 31702000.