Journal of Rare Cardiovascular Diseases

ISSN: 2299-3711 (Print) e-ISSN: 2300-5505 (Online)

Brugada syndrome: new concepts and algorithms in management (RCD code: V‐1A.1)

Paweł Tomasz Matusik, Anna Rydlewska, Joanna Pudło, Jakub Podolec, Jacek Lelakowski, Piotr Podolec

Full Text:

PDF

Abstract

Clinical manifestation of Brugada syndrome (BrS) mainly results from polymorphic ventricular arrhythmias and includes sudden cardiac
arrest (SCA). The Brugada sign, besides being present in true BrS, may result from different causes. Moreover, electrocardiogram findings in some clinical situations may resemble the BrS electrocardiographic pattern. Thus, differential diagnosis is crucial in the proper management of patients suspected of having BrS. Lifestyle modifications and close follow-up with or without pharmacologic treatment and/or implantable cardioverter-defibrillator placement constitute the most common approach to managing BrS patients. However, the role of ablation in BrS treatment is increasing. Due to diagnostic and therapeutic difficulties, the management of BrS is often challenging. This review provides new concepts and algorithms in the diagnostics and treatment of patients suspected of having BrS. JRCD 2017; 3 (5): 151–160

Keywords

Brugada syndrome; diagnostics; treatment; algorithms

References

Noc M, Fajadet J, Lassen JF, et al. Invasive coronary treatment strategies for out-of-hospital cardiac arrest: a consensus statement from the European association for percutaneous cardiovascular interventions (EAPCI)/stent for life (SFL) groups. EuroIntervention 2014; 10: 31–37.

Matusik PT. Insights into channelopathies: progress in clinical practice and research. J Electrocardiol 2017; 50: 534–535.

Matusik PT, Pudło J, Rydlewska A, Podolec J, Lelakowski J, Podolec P. Brugada syndrome: current diagnostics, epidemiology, genetic data and novel mechanisms (RCD code: V‐1A.1). J Rare Cardiovasc Dis 2017; 3: 73–80.

Tarin N, Farre J, Rubio JM, et al. Brugada-like electrocardiographic pattern in a patient with a mediastinal tumor. Pacing Clin Electrophysiol 1999; 22: 1264–1266.

Jastrzebski M, Kukla P. Ventricular fibrillation with a 2:1 conduction block over the right ventricle in a Brugada syndrome patient. Kardiol Pol 2013; 71: 991.

Dybich P, Bakowski D, Wozakowska-Kaplon B. [Syncope in male–let us think about Brugada syndrome! Presentation of 3 cases]. Kardiol Pol 2010; 68: 1397–1400; discussion 1401.

Agostini D, Scanu P, Loiselet P, et al. Iodine-123-metaiodobenzylguanidine SPECT of regional cardiac adrenergic denervation in Brugada syndrome. J Nucl Med 1998; 39: 1129–1132.

Rudic B, Schimpf R, Veltmann C, et al. Brugada syndrome: clinical presentation and genotype-correlation with magnetic resonance imaging parameters. Europace 2016; 18: 1411–1419.

Catalano O, Antonaci S, Moro G, et al. Magnetic resonance investigations in Brugada syndrome reveal unexpectedly high rate of structural abnormalities. Eur Heart J 2009; 30: 2241–2248.

Tessa C, Del Meglio J, Ghidini Ottonelli A, et al. Evaluation of Brugada syndrome by cardiac magnetic resonance. Int J Cardiovasc Imaging 2012; 28:1961–1970.

Iacoviello M, Forleo C, Puzzovivo A, et al. Altered two-dimensional strain measures of the right ventricle in patients with Brugada syndrome and arrhythmogenic right ventricular dysplasia/cardiomyopathy. Eur J Echocardiogr 2011; 12: 773–781.

Fauchier L, Isorni MA, Clementy N, et al. Prognostic value of programmed ventricular stimulation in Brugada syndrome according to clinical presentation: an updated meta-analysis of worldwide published data. Int J Cardiol 2013; 168: 3027–3029.

Priori SG, Gasparini M, Napolitano C, et al. Risk stratification in Brugada syndrome: results of the PRELUDE (PRogrammed ELectrical stimUlation preDictive valuE) registry. J Am Coll Cardiol 2012; 59: 37–45.

Probst V, Veltmann C, Eckardt L, et al. Long-term prognosis of patients diagnosed with Brugada syndrome: Results from the FINGER Brugada Syndrome Registry. Circulation 2010; 121: 635–643.

Sroubek J, Probst V, Mazzanti A, et al. Programmed Ventricular Stimulation for Risk Stratification in the Brugada Syndrome: A Pooled Analysis. Circulation 2016; 133: 622–630.

Sieira J, Brugada P. Management of Brugada Syndrome 2016: Should All High Risk Patients Receive an ICD? All High-Risk Patients Should Receive an Implantable Cardiac Defibrillator. Circ Arrhythm Electrophysiol 2016; 9.

Priori SG, Napolitano C, Gasparini M, et al. Natural history of Brugada syndrome: insights for risk stratification and management. Circulation 2002; 105: 1342–1347.

Priori SG, Blomstrom-Lundqvist C, Mazzanti A, et al. 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). Eur Heart J 2015; 36: 2793–2867.

Kim SH, Nam GB, Yun SC, et al. Variants of Brugada Syndrome and Early Repolarization Syndrome: An Expanded Concept of J-Wave Syndrome. Pacing Clin Electrophysiol 2017; 40: 162–174.

Antzelevitch C, Yan GX. J wave syndromes. Heart Rhythm 2010; 7: 549–558.

Antzelevitch C, Yan GX, Ackerman MJ, et al. J-Wave syndromes expert consensus conference report: Emerging concepts and gaps in knowledge: Endorsed by the Asia Pacific Heart Rhythm Society (APHRS), the European Heart Rhythm Association (EHRA), the Heart Rhythm Society (HRS), and the Latin American Society of Cardiac Pacing and Electrophysiology (Sociedad Latinoamericana de Estimulacifin Cardiaca y Electro fi siologia [SOLAECE]). Europace 2016.

Gonzalez Corcia MC, Sieira J, Sarkozy A, et al. Brugada syndrome in the young: an assessment of risk factors predicting future events. Europace 2016.

Matusik PT, Lelakowski J, Malecka B, et al. Management of Patients with Atrial Fibrillation: Focus on Treatment Options. J Atr Fibrillation 2016; 9: 62–69.

Enriquez A, Antzelevitch C, Bismah V, et al. Atrial fibrillation in inherited cardiac channelopathies: From mechanisms to management. Heart Rhythm 2016; 13: 1878–1884.

Lenarczyk R, Mitrega K, Mazurek M, et al. Polish and European management strategies in patients with atrial fibrillation. Data from the EURObservational Research Programme-Atrial Fibrillation General Registry Pilot Phase (EORP-AF Pilot). Pol Arch Med Wewn 2016; 126: 138–148.

Kolodziej M, Sledz J, Janion M. [Brugada syndrome–underestimated cause of sudden cardiac death in patients without an organic cardiac disease–a case report]. Kardiol Pol 2009; 67: 159–161.

Gehi AK, Duong TD, Metz LD, et al. Risk stratification of individuals with the Brugada electrocardiogram: a meta-analysis. J Cardiovasc Electrophysiol 2006; 17: 577–583.

Fernandez-Falgueras A, Sarquella-Brugada G, Brugada J, et al. Cardiac Channelopathies and Sudden Death: Recent Clinical and Genetic Advances. Biology (Basel) 2017; 6.

Yokokawa M, Okamura H, Noda T, et al. Neurally mediated syncope as a cause of syncope in patients with Brugada electrocardiogram. J Cardiovasc Electrophysiol 2010; 21: 186–192.

Patruno N, Pontillo D. Brugada syndrome and vasovagal syncope. Pacing Clin Electrophysiol 2006; 29: 215; author reply 216.

Stryjewski PJ, Nessler B, Kuczaj A, et al. The role of NT-proBNP in the diagnostics and differentiation of cardiac and reflex syncope in adults: relative importance to clinical presentation and medical examinations. J Interv Card Electrophysiol 2014; 41: 1–8.

Makimoto H, Nakagawa E, Takaki H, et al. Augmented ST-segment elevation during recovery from exercise predicts cardiac events in patients with Brugada syndrome. J Am Coll Cardiol 2010; 56: 1576–1584.

Subramanian M, Prabhu MA, Harikrishnan MS, et al. The Utility of Exercise Testing in Risk Stratification of Asymptomatic Patients With Type 1 Brugada Pattern. J Cardiovasc Electrophysiol 2017; 28: 677–683.

Enriquez A, Brugada J, Baranchuk A. Exercise-Induced Brugada Phenocopy. J Cardiovasc Electrophysiol 2016; 27: 360–361.

Kurpesa M, Jerka K, Bortkiewicz A. [Cardiopulmonary exercise testing–its application in cardiology and occupational medicine]. Med Pr 2014; 65: 665–674. 160 Matusik, et al.

Reeves GR, Gupta S, Forman DE. Evolving Role of Exercise Testing in Contemporary Cardiac Rehabilitation. J Cardiopulm Rehabil Prev 2016; 36: 309–319.

Antzelevitch C, Patocskai B. Brugada Syndrome: Clinical, Genetic, Molecular, Cellular, and Ionic Aspects. Curr Probl Cardiol 2016; 41: 7–57.

Masrur S, Memon S, Thompson PD. Brugada syndrome, exercise, and exercise testing. Clin Cardiol 2015; 38: 323–326.

Antzelevitch C, Brugada P, Borggrefe M, 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.

Mont L, Pelliccia A, Sharma S, et al. Pre-participation cardiovascular evaluation for athletic participants to prevent sudden death: Position paper from the EHRA and the EACPR, branches of the ESC. Endorsed by APHRS, HRS, and SOLAECE. Europace 2017; 19: 139–163.

Pelliccia A, Fagard R, Bjornstad HH, et al. Recommendations for competitive sports participation in athletes with cardiovascular disease: a consensus document from the Study Group of Sports Cardiology of the Working Group of Cardiac Rehabilitation and Exercise Physiology and the Working Group of Myocardial and Pericardial Diseases of the European Society of Cardiology. Eur Heart J 2005; 26: 1422–1445.

Ackerman MJ, Zipes DP, Kovacs RJ, et al. Eligibility and Disqualification Recommendations for Competitive Athletes With Cardiovascular Abnormalities: Task Force 10: The Cardiac Channelopathies: A Scientific Statement From the American Heart Association and American College of Cardiology. J Am Coll Cardiol 2015; 66: 2424–2428.

Mascia G, Arbelo E, Ojeda JH, et al. Brugada Syndrome and Exercise Practice: Current Knowledge, Shortcomings and Open Questions. Int J Sports Med 2017.

Matusik PT, Komar M, Podolec J, Karkowski G, Lelakowski J, Podolec P. Exercise ECG unmasked Brugada sign: manifestation of the risk of sports‐associated sudden cardiac arrest (RCD code: V‐1A.1). J Rare Cardiovasc Dis 2017; 3: 92–97.

Zipes DP, Link MS, Ackerman MJ, et al. Eligibility and Disqualification Recommendations for Competitive Athletes With Cardiovascular Abnormalities: Task Force 9: Arrhythmias and Conduction Defects: A Scientific Statement From the American Heart Association and American College of Cardiology. J Am Coll Cardiol 2015; 66: 2412–2423.

Belhassen B, Rahkovich M, Michowitz Y, et al. Management of Brugada Syndrome: Thirty-Three-Year Experience Using Electrophysiologically Guided Therapy With Class 1A Antiarrhythmic Drugs. Circ Arrhythm Electrophysiol 2015; 8: 1393–1402.

Bouzeman A, Traulle S, Messali A, et al. Long-term follow-up of asymptomatic Brugada patients with inducible ventricular fibrillation under hydroquinidine. Europace 2014; 16: 572–577.

Nademanee K, Veerakul G, Mower M, et al. Defibrillator Versus beta-Blockers for Unexplained Death in Thailand (DEBUT): a randomized clinical trial. Circulation 2003; 107: 2221–2226.

Havakuk O, Viskin S. A Tale of 2 Diseases: The History of Long-QT Syndrome and Brugada Syndrome. J Am Coll Cardiol 2016; 67: 100–108.

Olde Nordkamp LR, Postema PG, Knops RE, et al. Implantable cardioverter-defibrillator harm in young patients with inherited arrhythmia syndromes: A systematic review and meta-analysis of inappropriate shocks and complications. Heart Rhythm 2016; 13: 443–454.

Miyazaki S, Uchiyama T, Komatsu Y, et al. Long-term complications of implantable defibrillator therapy in Brugada syndrome. Am J Cardiol 2013; 111: 1448–1451.

Sacher F, Probst V, Maury P, et al. Outcome after implantation of a cardioverter-defibrillator in patients with Brugada syndrome: a multicenter study-part 2. Circulation 2013; 128: 1739–1747.

Conte G, Sieira J, Ciconte G, et al. Implantable cardioverter-defibrillator therapy in Brugada syndrome: a 20-year single-center experience. J Am Coll Cardiol 2015; 65: 879–888.

Burke MC, Gold MR, Knight BP, et al. Safety and Efficacy of the Totally Subcutaneous Implantable Defibrillator: 2-Year Results From a Pooled Analysis of the IDE Study and EFFORTLESS Registry. J Am Coll Cardiol 2015; 65: 1605–1615.

Weiss R, Knight BP, Gold MR, et al. Safety and efficacy of a totally subcutaneous implantable-cardioverter defibrillator. Circulation 2013; 128: 944–953.

Lambiase PD, Barr C, Theuns DA, et al. Worldwide experience with a totally subcutaneous implantable defibrillator: early results from the EFFORTLESS S-ICD Registry. Eur Heart J 2014; 35: 1657–1665.

Hai JJ, Lim ET, Chan CP, et al. First clinical experience of the safety and feasibility of total subcutaneous implantable defibrillator in an Asian population. Europace 2015; 17 Suppl 2: ii63-68.

Olde Nordkamp LR, Conte G, Rosenmoller BR, et al. Brugada Syndrome and the Subcutaneous Implantable Cardioverter-Defibrillator. J Am Coll Cardiol 2016; 68: 665–666.

Haissaguerre M, Extramiana F, Hocini M, et al. Mapping and ablation of ventricular fibrillation associated with long-QT and Brugada syndromes. Circulation 2003; 108: 925–928.

Nademanee K, Hocini M, Haissaguerre M. Epicardial substrate ablation for Brugada syndrome. Heart Rhythm 2017; 14: 457–461.

Shah AJ, Hocini M, Lamaison D, et al. Regional substrate ablation abolishes Brugada syndrome. J Cardiovasc Electrophysiol 2011; 22: 1290–1291.

Nademanee K, Veerakul G, Chandanamattha P, et al. Prevention of ventricular fibrillation episodes in Brugada syndrome by catheter ablation over the anterior right ventricular outflow tract epicardium. Circulation 2011; 123: 1270–1279.

Wilde AA, Nademanee K. Epicardial Substrate Ablation in Brugada Syndrome: Time for a Randomized Trial! Circ Arrhythm Electrophysiol 2015; 8: 1306–1308.

Brugada J, Pappone C, Berruezo A, et al. Brugada Syndrome Phenotype Elimination by Epicardial Substrate Ablation. Circ Arrhythm Electrophysiol 2015; 8: 1373–1381.

Priori SG, Wilde AA, Horie M, et al. HRS/EHRA/APHRS expert consensus statement on the diagnosis and management of patients with inherited primary arrhythmia syndromes: document endorsed by HRS, EHRA, and APHRS in May 2013 and by ACCF, AHA, PACES, and AEPC in June 2013. Heart Rhythm 2013; 10: 1932–1963.

Arnalsteen-Dassonvalle E, Hermida JS, Kubala M, et al. Ajmaline challenge for the diagnosis of Brugada syndrome: which protocol? Arch Cardiovasc Dis 2010; 103: 570–578.

Dendramis G. Brugada syndrome and Brugada phenocopy. The importance of a differential diagnosis. Int J Cardiol 2016; 210: 25–27.

Hong K, Brugada J, Oliva A, et al. Value of electrocardiographic parameters and ajmaline test in the diagnosis of Brugada syndrome caused by SCN5A mutations. Circulation 2004; 110: 3023–3027.

Veltmann C, Papavassiliu T, Konrad T, et al. Insights into the location of type I ECG in patients with Brugada syndrome: correlation of ECG and cardiovascular magnetic resonance imaging. Heart Rhythm 2012; 9: 414–421.

Papavassiliu T, Veltmann C, Doesch C, et al. Spontaneous type 1 electrocardiographic pattern is associated with cardiovascular magnetic resonance imaging changes in Brugada syndrome. Heart Rhythm 2010; 7: 1790–1796.

Papavassiliu T, Wolpert C, Fluchter S, et al. Magnetic resonance imaging findings in patients with Brugada syndrome. J Cardiovasc Electrophysiol 2004; 15: 1133–1138.

Shinohara T, Ebata Y, Ayabe R, et al. Combination therapy of cilostazol and bepridil suppresses recurrent ventricular fibrillation related to J-wave syndromes. Heart Rhythm 2014; 11: 1441–1445.

Belhassen B, Glick A, Viskin S. Efficacy of quinidine in high-risk patients with Brugada syndrome. Circulation 2004; 110: 1731–1737.

Rubiś P. Brugada syndrome: 2017 update (RCD code: V-1A.1). J Rare Cardiovasc Dis 2017; 3 (4): 108–109.

DOI: http://dx.doi.org/10.20418%2Fjrcd.vol3no5.298

Refbacks

  • There are currently no refbacks.