Review

. 2024 Jan;17(1):e012072.

doi: 10.1161/CIRCEP.123.012072. Epub 2023 Dec 15.

Clinical Management of Brugada Syndrome: Commentary From the Experts

Michael J Cutler^#¹, Lee L Eckhardt^#², Elizabeth S Kaufman^#³, Elena Arbelo^{4

5

6}, Elijah R Behr^{7

8}, Pedro Brugada^{9

10}, Marina Cerrone¹¹, Lia Crotti^{12

13}, Carlo deAsmundis¹⁴, Michael H Gollob¹⁵, Minoru Horie¹⁶, David T Huang¹⁷, Andrew D Krahn¹⁸, Barry London¹⁹, Steven A Lubitz²⁰, Judith A Mackall²¹, Koonlawee Nademanee^{22

23}, Marco V Perez²⁴, Vincent Probst²⁵, Dan M Roden²⁶, Frederic Sacher²⁷, Georgia Sarquella-Brugada^{28

29}, Melvin M Scheinman³⁰, Wataru Shimizu³¹, Benjamin Shoemaker³², Raymond W Sy^{33

34}, Atsuyuki Watanabe³⁵, Arthur A M Wilde^{36

37}

Affiliations

¹ Intermountain Heart Institute, Intermountain Medical Center, Salt Lake City, UT (M.J.C.).
² Cellular and Molecular Arrhythmia Research Program, Division of CVM, Department of Medicine, University of Wisconsin-Madison (L.L.E.).
³ Heart and Vascular Center, MetroHealth Campus, Case Western Reserve University, Cleveland, OH (E.S.K.).
⁴ Arrhythmia Section, Cardiology Department, Hospital Clínic, Universitat de Barcelona (E.A.).
⁵ Centro de Investigacion Biomedica en Red de Enfermedades Cardiovasculares (CIBERCV), Madrid (E.A.).
⁶ IDIBAPS, Institut d'Investigacio August Pi I Sunyer, Barcelona, Spain (E.A.).
⁷ Cardiovascular Clinical Academic Group, Cardiology Section, St. George's, University of London and St. George's University Hospitals NHS Foundation Trust (E.R.B.).
⁸ Mayo Clinic Healthcare, London, United Kingdom (E.R.B.).
⁹ Cardiovascular Division, UZ Brussel-VUB, Belgium (P.B.).
¹⁰ Arrhythmia Unit, Helicopteros Sanitarios Hospital (HSH), Puerto Banús, Marbella, Malaga, Spain (P.B.).
¹¹ New York Univ Grossman School of Medicine, Leon H. Charney Division of Cardiology (M.C.).
¹² Department of Medicine and Surgery, University of Milano-Bicocca (L.C.).
¹³ Istituto Auxologico Italiano IRCCS, Center for Cardiac Arrhythmias of Genetic Origin and Laboratory of Cardiovascular Genetics, Milan, Italy (L.C.).
¹⁴ Heart Rhythm Management Center, Postgraduate Program in Cardiac Electrophysiology and Pacing, Universitair Ziekenhuis Brussel-Vrije Universiteit Brussel, European Reference Networks Guard-Heart, Belgium (C.D.).
¹⁵ Peter Munk Cardiac Center, Division of Cardiology, Toronto General Hospital, University Health Network, Canada (M.H.G.).
¹⁶ Department of Cardiovascular Medicine, Shiga University of Medical Science, Ohtsu, Japan (M.H.).
¹⁷ University of Rochester Medical Center, NY (D.T.H.).
¹⁸ Center for Cardiovascular Innovation, Division of Cardiology, University of British Columbia, Vancouver, Canada (A.D.K.).
¹⁹ Division of Cardiovascular Medicine, Department of Internal Medicine and Abboud Cardiovascular Research Center, University of Iowa Carver College of Medicine, Iowa City (B.L.).
²⁰ Demoulas Center for Cardiac Arrhythmias, Massachusetts General Hospital, Boston (S.A.L.).
²¹ Department of Medicine, Division of Cardiology, University Hospitals Harrington Heart and Vascular Institute, Case Western Reserve University School of Medicine, Cleveland, OH (J.A.M.).
²² Center of Excellence in Arrhythmia Research, Department of Medicine, Faculty of Medicine, Chulalongkorn University (K.N.).
²³ Pacific Rim Electrophysiology Research Institute at Bumrungrad Hospital, Bangkok, Thailand (K.N.).
²⁴ Stanford Center for Inherited Cardiovascular Diseases, Stanford University, CA (M.V.P.).
²⁵ Université Nantes, CHU Nantes, CNRS, INSERM, Service de Cardiologie, l'institut du thorax, Nantes, France (V.P.).
²⁶ Departments of Medicine, Pharmacology and Biomedical Informatics, Vanderbilt University Medical Center, Nashville, TN (D.M.R.).
²⁷ Arrhythmia Department, Bordeaux University Hospital, IHU LIRYC, Pessac, France (F.S.).
²⁸ Pediatric Arrhythmias, Inherited Cardiac Diseases and Sudden Death Unit, Hospital Sant Joan de Déu, Universitat de Barcelona (G.S.-B.).
²⁹ Arrítmies Pediàtriques, Cardiologia Genètica i Mort sobtada, Malalties Cardiovasculars en el Desenvolupament, Institut de Recerca Sant Joan de Déu, Esplugues de Llobregat, Barcelona, Spain (G.S.-B.).
³⁰ Section of Cardiac Electrophysiology, Division of Cardiology, University of California-San Francisco (M.M.S.).
³¹ Department of Cardiovascular Medicine, Graduate School of Medicine, Nippon Medical School, Tokyo, Japan (W.S.).
³² Department of Medicine, Division of Cardiovascular Medicine, Vanderbilt University Medical Center, Nashville, TN (B.S.).
³³ Faculty of Medicine and Heath, The University of Sydney (R.W.S.).
³⁴ Department of Cardiology, Royal Prince Alfred Hospital, Sydney, Australia (R.W.S.).
³⁵ Department of Cardiology, National Hospital Organization Okayama Medical Center, Japan (A.W.).
³⁶ Department of Cardiology, University of Amsterdam (A.A.M.W.).
³⁷ Amsterdam Cardiovascular Sciences, Heart Failure and Arrhythmias, Amsterdam, the Netherlands (A.A.M.W.).

^# Contributed equally.

PMID: 38099441
PMCID: PMC10824563
DOI: 10.1161/CIRCEP.123.012072

Review

Clinical Management of Brugada Syndrome: Commentary From the Experts

Michael J Cutler et al. Circ Arrhythm Electrophysiol. 2024 Jan.

. 2024 Jan;17(1):e012072.

doi: 10.1161/CIRCEP.123.012072. Epub 2023 Dec 15.

Authors

Affiliations

¹ Intermountain Heart Institute, Intermountain Medical Center, Salt Lake City, UT (M.J.C.).
² Cellular and Molecular Arrhythmia Research Program, Division of CVM, Department of Medicine, University of Wisconsin-Madison (L.L.E.).
³ Heart and Vascular Center, MetroHealth Campus, Case Western Reserve University, Cleveland, OH (E.S.K.).
⁴ Arrhythmia Section, Cardiology Department, Hospital Clínic, Universitat de Barcelona (E.A.).
⁵ Centro de Investigacion Biomedica en Red de Enfermedades Cardiovasculares (CIBERCV), Madrid (E.A.).
⁶ IDIBAPS, Institut d'Investigacio August Pi I Sunyer, Barcelona, Spain (E.A.).
⁷ Cardiovascular Clinical Academic Group, Cardiology Section, St. George's, University of London and St. George's University Hospitals NHS Foundation Trust (E.R.B.).
⁸ Mayo Clinic Healthcare, London, United Kingdom (E.R.B.).
⁹ Cardiovascular Division, UZ Brussel-VUB, Belgium (P.B.).
¹⁰ Arrhythmia Unit, Helicopteros Sanitarios Hospital (HSH), Puerto Banús, Marbella, Malaga, Spain (P.B.).
¹¹ New York Univ Grossman School of Medicine, Leon H. Charney Division of Cardiology (M.C.).
¹² Department of Medicine and Surgery, University of Milano-Bicocca (L.C.).
¹³ Istituto Auxologico Italiano IRCCS, Center for Cardiac Arrhythmias of Genetic Origin and Laboratory of Cardiovascular Genetics, Milan, Italy (L.C.).
¹⁴ Heart Rhythm Management Center, Postgraduate Program in Cardiac Electrophysiology and Pacing, Universitair Ziekenhuis Brussel-Vrije Universiteit Brussel, European Reference Networks Guard-Heart, Belgium (C.D.).
¹⁵ Peter Munk Cardiac Center, Division of Cardiology, Toronto General Hospital, University Health Network, Canada (M.H.G.).
¹⁶ Department of Cardiovascular Medicine, Shiga University of Medical Science, Ohtsu, Japan (M.H.).
¹⁷ University of Rochester Medical Center, NY (D.T.H.).
¹⁸ Center for Cardiovascular Innovation, Division of Cardiology, University of British Columbia, Vancouver, Canada (A.D.K.).
¹⁹ Division of Cardiovascular Medicine, Department of Internal Medicine and Abboud Cardiovascular Research Center, University of Iowa Carver College of Medicine, Iowa City (B.L.).
²⁰ Demoulas Center for Cardiac Arrhythmias, Massachusetts General Hospital, Boston (S.A.L.).
²¹ Department of Medicine, Division of Cardiology, University Hospitals Harrington Heart and Vascular Institute, Case Western Reserve University School of Medicine, Cleveland, OH (J.A.M.).
²² Center of Excellence in Arrhythmia Research, Department of Medicine, Faculty of Medicine, Chulalongkorn University (K.N.).
²³ Pacific Rim Electrophysiology Research Institute at Bumrungrad Hospital, Bangkok, Thailand (K.N.).
²⁴ Stanford Center for Inherited Cardiovascular Diseases, Stanford University, CA (M.V.P.).
²⁵ Université Nantes, CHU Nantes, CNRS, INSERM, Service de Cardiologie, l'institut du thorax, Nantes, France (V.P.).
²⁶ Departments of Medicine, Pharmacology and Biomedical Informatics, Vanderbilt University Medical Center, Nashville, TN (D.M.R.).
²⁷ Arrhythmia Department, Bordeaux University Hospital, IHU LIRYC, Pessac, France (F.S.).
²⁸ Pediatric Arrhythmias, Inherited Cardiac Diseases and Sudden Death Unit, Hospital Sant Joan de Déu, Universitat de Barcelona (G.S.-B.).
²⁹ Arrítmies Pediàtriques, Cardiologia Genètica i Mort sobtada, Malalties Cardiovasculars en el Desenvolupament, Institut de Recerca Sant Joan de Déu, Esplugues de Llobregat, Barcelona, Spain (G.S.-B.).
³⁰ Section of Cardiac Electrophysiology, Division of Cardiology, University of California-San Francisco (M.M.S.).
³¹ Department of Cardiovascular Medicine, Graduate School of Medicine, Nippon Medical School, Tokyo, Japan (W.S.).
³² Department of Medicine, Division of Cardiovascular Medicine, Vanderbilt University Medical Center, Nashville, TN (B.S.).
³³ Faculty of Medicine and Heath, The University of Sydney (R.W.S.).
³⁴ Department of Cardiology, Royal Prince Alfred Hospital, Sydney, Australia (R.W.S.).
³⁵ Department of Cardiology, National Hospital Organization Okayama Medical Center, Japan (A.W.).
³⁶ Department of Cardiology, University of Amsterdam (A.A.M.W.).
³⁷ Amsterdam Cardiovascular Sciences, Heart Failure and Arrhythmias, Amsterdam, the Netherlands (A.A.M.W.).

^# Contributed equally.

PMID: 38099441
PMCID: PMC10824563
DOI: 10.1161/CIRCEP.123.012072

Abstract

Although there is consensus on the management of patients with Brugada Syndrome with high risk for sudden cardiac arrest, asymptomatic or intermediate-risk patients present clinical management challenges. This document explores the management opinions of experts throughout the world for patients with Brugada Syndrome who do not fit guideline recommendations. Four real-world clinical scenarios were presented with commentary from small expert groups for each case. All authors voted on case-specific questions to evaluate the level of consensus among the entire group in nuanced diagnostic and management decisions relevant to each case. Points of agreement, points of controversy, and gaps in knowledge are highlighted.

Keywords: Brugada Syndrome; sudden cardiac arrest; syncope; ventricular fibrillation.

PubMed Disclaimer

Conflict of interest statement

Disclosures Dr Lubitz is a full-time employee of Novartis Institutes of BioMedical Research as of July 18, 2022. Dr Lubitz previously received sponsored research support from Bristol Myers Squibb, Pfizer, Boehringer Ingelheim, Fitbit, Medtronic, Premier, and IBM and has consulted for Bristol Myers Squibb, Pfizer, Blackstone Life Sciences, and Invitae.

Figures

**Figure 1**
Case 1 ECGs. Panel A. Spontaneous ECG during fever. Panel B. Repeat ECG when afebrile.

**Figure 2**
Case 1 Group Voting. Survey results from questions related to case 1.

**Figure 3**
Case 2 ECG. Spontaneous ECG in the emergency department following syncope.

**Figure 4**
Case 2 Group Voting. Survey results from questions related to case 2.

**Figure 5**
Case 3 ECGs. Panel A. Spontaneous ECG in the emergency department following syncope. Panel B. Baseline ECG during Procainamide challenge. Panel C. Repeat ECG during Procainamide challenge.

**Figure 6**
Case 3 Group Voting. Survey results from questions related to case 3.

**Figure 7**
Case 4 ECGs. Panel A. Spontaneous ECG in the emergency department. Panel B. Repeat ECG three hours after presenting to the emergency department.

**Figure 8**
Case 4 Group Voting. Survey results from questions related to case 4.

See this image and copyright information in PMC

References

1. Brugada P, Brugada J. Right bundle branch block, persistent ST segment elevation and sudden cardiac death: a distinct clinical and electrocardiographic syndrome. A multicenter report. J Am Coll Cardiol. 1992;20:1391–1396. doi: 10.1016/0735-1097(92)90253-j - DOI - PubMed
1. Antzelevitch C, Brugada P, Borggrefe M, Brugada J, Brugada R, Corrado D, Gussak I, LeMarec H, Nademanee K, Perez Riera AR, et al. Brugada syndrome: report of the second consensus conference: endorsed by the Heart Rhythm Society and the European Heart Rhythm Association. Circulation. 2005;111:659–670. doi: 10.1161/01.CIR.0000152479.54298.51 - DOI - PubMed
1. Antzelevitch C, Yan GX, Ackerman MJ, Borggrefe M, Corrado D, Guo J, Gussak I, Hasdemir C, Horie M, Huikuri H, et al. J-Wave syndromes expert consensus conference report: Emerging concepts and gaps in knowledge. Europace. 2017;19:665–694. doi: 10.1093/europace/euw235 - DOI - PMC - PubMed
1. Viskin S, Rosso R, Friedensohn L, Havakuk O, Wilde AA. Everybody has Brugada syndrome until proven otherwise? Heart Rhythm. 2015;12:1595–1598. doi: 10.1016/j.hrthm.2015.04.017 - DOI - PubMed
1. Hasdemir C, Payzin S, Kocabas U, Sahin H, Yildirim N, Alp A, Aydin M, Pfeiffer R, Burashnikov E, Wu Y, Antzelevitch C. High prevalence of concealed Brugada syndrome in patients with atrioventricular nodal reentrant tachycardia. Heart Rhythm. 2015;12:1584–1594. doi: 10.1016/j.hrthm.2015.03.015 - DOI - PubMed

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Clinical Management of Brugada Syndrome: Commentary From the Experts

Affiliations

Clinical Management of Brugada Syndrome: Commentary From the Experts

Authors

Affiliations

Abstract

Conflict of interest statement

Figures

References

Publication types

MeSH terms

Grants and funding

LinkOut - more resources

Full Text Sources

Medical