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Brugada-Syndrom

Risikostratifikation und Prävention des plötzlichen Herztods

Brugada syndrome

Risk stratification and prevention of sudden cardiac death

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Zusammenfassung

Das Brugada-Syndrom zählt zu den Ionenkanalerkrankungen. Elektrokardiographisch finden sich transiente oder persistierende bogenförmige ST-Streckenhebungen in mindestens einer rechtspräkordialen EKG-Ableitung. Die Klinik der Patienten ist durch Kammertachykardien, einen plötzlichen Herztod oder Synkopen charakterisiert, wobei aber die Mehrzahl der Betroffenen wahrscheinlich zeitlebens asymptomatisch bleibt. Die richtige EKG-Diagnose ist eine besondere klinische Herausforderung. Der implantierbare Kardioverter-Defibrillator (ICD) ist der einzige etablierte Schutz vor einem plötzlichen Herztod. Insbesondere in der Primärprophylaxe ist eine individuelle Risikostratifikation von großer Bedeutung. Das Ziel dieses Artikels ist es, einen Überblick über den aktuellen Stand der Risikostratifikation und der therapeutischen Möglichkeiten beim Brugada-Syndrom zu geben.

Abstract

Brugada syndrome is ion channelopathy defined by coved type ST-elevation in at least one right precordial ECG lead. Patients may suffer from ventricular tachycardia/fibrillation, which may cause syncope or sudden cardiac death. The majority of patients are likely to remain asymptomatic throughout life. A correct ECG diagnosis remains challenging. The implantable cardioverter/defibrillator (ICD) is the only established therapy to protect against sudden cardiac death. Thus, individual risk stratification is of major clinical relevance in primary prevention. The present article gives an update on current risk stratification and novel therapeutic options apart from ICD therapy.

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Literatur

  1. Brugada J, Campuzano O, Arbelo E, Sarquella-Brugada G, Brugada R (2018) Present status of Brugada syndrome. J Am Coll Cardiol 72:1046–1059

    Article  Google Scholar 

  2. Watanabe H, Minamino T (2016) Genetics of Brugada syndrome. J Hum Genet 61:57–60

    Article  CAS  Google Scholar 

  3. Bayes de Luna A, Brugada J, Baranchuk A, Borggrefe M, Breithardt G, Goldwasser D, Lambiase P, Riera AP, Garcia-Niebla J, Pastore C, Oreto G, McKenna W, Zareba W, Brugada R, Brugada P (2012) Current electrocardiographic criteria for diagnosis of Brugada pattern: a consensus report. J Electrocardiol 45:433–442

    Article  Google Scholar 

  4. Osher HL, Wolff L (1953) Electrocardiographic pattern simulating acute myocardial injury. Am J Med Sci 226:541–545

    Article  CAS  Google Scholar 

  5. Chevallier S, Forclaz A, Tenkorang J, Ahmad Y, Faouzi M, Graf D, Schlaepfer J, Pruvot E (2011) New electrocardiographic criteria for discriminating between Brugada types 2 and 3 patterns and incomplete right bundle branch block. J Am Coll Cardiol 58:2290–2298

    Article  Google Scholar 

  6. Smits JP, Eckardt L, Probst V, Bezzina CR, Schott JJ, Remme CA, Haverkamp W, Breithardt G, Escande D, Schulze-Bahr E, LeMarec H, Wilde AA (2002) Genotype-phenotype relationship in Brugada syndrome: electrocardiographic features differentiate SCN5A-related patients from non-SCN5A-related patients. J Am Coll Cardiol 40:350–356

    Article  CAS  Google Scholar 

  7. Catalano O, Antonaci S, Moro G, Mussida M, Frascaroli M, Baldi M, Cobelli F, Baiardi P, Nastoli J, Bloise R, Monteforte N, Napolitano C, Priori SG (2009) Magnetic resonance investigations in Brugada syndrome reveal unexpectedly high rate of structural abnormalities. Eur Heart J 30:2241–2248

    Article  Google Scholar 

  8. Rolf S, Bruns HJ, Wichter T, Kirchhof P, Ribbing M, Wasmer K, Paul M, Breithardt G, Haverkamp W, Eckardt L (2003) The ajmaline challenge in Brugada syndrome: diagnostic impact, safety and recommended protocol. Eur Heart J 24:1104–1112

    Article  CAS  Google Scholar 

  9. Frommeyer G, Eckardt L (2016) Drug-induced proarrhythmia: risk factors and electrophysiological mechanisms. Nat Rev Cardiol 13:36–47

    Article  CAS  Google Scholar 

  10. Brugada P, Brugada J (1992) 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 20:1391–1396

    Article  CAS  Google Scholar 

  11. Paul M, Gerss J, Schulze-Bahr E, Wichter T, Vahlhaus C, Wilde AA, Breithardt G, Eckardt L (2007) Role of programmed ventricular stimulation in patients with Brugada syndrome: a meta-analysis of worldwide published data. Eur Heart J 28:2126–2233

    Article  Google Scholar 

  12. Brugada J, Brugada R (2003) Determinants of 68 sudden cardiac death in individuals with the electrocardiographic pattern of Brugada syndrome and no previous cardiac arrest. Circulation 108:3092–3096

    Article  Google Scholar 

  13. Priori SG, Napolitano C, Gasparini M, Pappone C, Della Bella P, Giordano U, Bloise R, Giustetto C, De Nardis R, Grillo M, Ronchetti E, Faggiano G, Nastoli J (2002) Natural history of Brugada syndrome: insights for risk stratification and management. Circulation 105:1342–1347

    Article  Google Scholar 

  14. Eckardt L, Probst V, Smits JP, Bahr ES, Wolpert C, Schimpf R, Wichter T, Boisseau P, Heinecke A, Breithardt G, Borggrefe M, LeMarec H, Böcker D, Wilde AA (2005) Long-term prognosis of individuals with right precordial St-segment-elevation. Circulation 111:257–263

    Article  Google Scholar 

  15. Probst V, Veltmann C, Eckardt L, Meregalli PG, Gaita F, Tan HL, Babuty D, Sacher F, Giustetto C, Schulze-Bahr E, Borggrefe M, Haissaguerre M, Mabo P, Le Marec H, Wolpert C, Wilde AA (2010) Long-term prognosis of patients diagnosed with Brugada syndrome: results from the FINGER Brugada syndrome registry. Circulation 105:635–643

    Article  Google Scholar 

  16. Sacher F, Probst V, Maury P, Babuty D, Mansoourati J, Komatsu Y, Marquie C, Rosa A, Diallo A, Cassagneau R, Loizeau C, Martins R, Field ME, Derval N, Miyazaki S, Denis A, Nogami A, Ritter P, Gourraud JB, Ploux S, Rollin A, Zemmoura A, Lamaison D, Bordachar P, Pierre B, Jais P, Pasquie JL, Hocini M, Legal F, Defaye P, Boveda S, Lesaka Y, Mabo P, Haisaguerre M (2013) Qutcome after implantation of a cardioverter-defibrillator in patients with Brugada syndrome: a multi-center study-part 2. Circulation 128:1739–1747

    Article  Google Scholar 

  17. Priori SG, Gasparini M, Napolitano C, Della Bella P, Ottonelli AG, Sassone B, Giordano U, Pappone C, Mascioli G, Rossetti G, De Nardis R, Colombo M (2012) Risk stratification in Brugada syndrome: results of the PRELUDE registry. J Am Coll Cardiol 59:37–45

    Article  Google Scholar 

  18. Morita H, Kusano KF, Miura D, Nagase S, Nakamura K, Morita ST, Ohe T, Zipes DP, Wu J (2008) Fragmented QRS as a marker of conduction abnormality and a predictor of prognosis in Brugada syndrome. Circulation 118:1697–1704

    Article  Google Scholar 

  19. Calò L, Giustetto C, Martino A, Sciarra L, Cerrato N, Marziali M, Rauzino J, Carlino G, de Ruvo E, Guerra F, Rebecchi M, Lanzillo C, Anselmino M, Castro A, Turreni F, Penco M, Volpe M, Capucci A, Gaita F (2016) A new electrocardiographic marker of sudden death in Brugada syndrome. J Am Coll Cardiol 67:1427–1440

    Article  Google Scholar 

  20. Eckardt L, Kirchhof P, Loh P, Schulze-Bahr E, Johna R, Wichter T, Breithardt G, Haverkamp W, Borggrefe M (2001) Brugada syndrome and supraventricular tachyarrhythmias: a novel association? J Cardiovasc Electrophysiol 12:680–685

    Article  CAS  Google Scholar 

  21. Sroubek J, Probst V, Mazzanti A, Delise P, Hevia JC, Ohkubo K, Zorzi A, Champagne J, Kostopoulou A, Yin X, Napolitano C, Milan DJ, Wilde A, Sacher F, Borggrefe M, Ellinor PT, Theodorakis G, Nault I, Corrado D, Watanabe I, Antzelevitch C, Allocca G, Priori SG, Lubitz SA (2016) Programmed ventricular stimulation for risk stratification in Brugada syndrome: a pooled analysis. Circulation 133:622–630

    Article  Google Scholar 

  22. Eckardt L, Bruns HJ, Paul M, Kirchhof P, Schulze-Bahr E, Wichter T, Breithardt G, Borggrefe M, Haverkamp W (2002) Body surface area of ST elevation and the presence of late potentials correlate to the inducibility of ventricular tachyarrhythmias in Brugada syndrome. Herzschrittmacherther Elektrophysiol 13:157–165

    Article  Google Scholar 

  23. Eckardt L, Kirchhof P, Schulze-Bahr E, Rolf S, Ribbing M, Loh P, Bruns HJ, Witte A, Milberg P, Borggrefe M, Breithardt G, Wichter T, Haverkamp W (2002) Electrophysiologic investigation in Brugada syndrome: yield of programmed ventricular stimulation at two ventricular sites with up to three premature beats. Eur Heart J 23:1394–1401

    Article  CAS  Google Scholar 

  24. Priori SG, Wilde AA, Horide M, Cho Y, Behr ER, Berul C, Blom N, Brugada J, Chiang CE, Huikuri H, Kannankeril P, Krahn A, Leenhardt A, Moss A, Schwartz PJ, Shimizu W, Tomaselli G, Tracy C (2013) HRS/EHRA/APHRS expert consensus statement on the diagnosis and management of patients with inherited primary arrhythmia syndromes. Europace 15:1389–1406

    Article  Google Scholar 

  25. Köbe J, Wasmer K, Reinke F, Eckardt L (2016) Indications for implantable loop recorders in patients with channelopathies and ventricular tachycardias. Herzschrittmacherther Elektrophysiol 27:360–365

    Article  Google Scholar 

  26. Siera J, Conte G, Ciconte G, Brugada P (2017) A score model to predict risk of events in patients with Brugada syndrome. Eur Heart J 38:1756–1763

    Article  Google Scholar 

  27. Olde Nordkamp LR, Postema PG, Knops RE, van Dijk N, Limpens J, Wilde AA, de Groot JR (2016) Implantable cardioverter-defibrillator harm in young patients with inherited arrhythmia syndromes: a systematic review and meta-analysis of inappropriate shocks and complications. Heart Rhythm 13:443–454

    Article  Google Scholar 

  28. Bögeholz N, Willy K, Niehues P, Rath B, Dechering DG, Frommeyer G, Kochhäuser S, Löher A, Köbe J, Reinke F, Eckardt L (2019) Spotlight on S‑ICDTM therapy: 10 years of clinical experience and innovation. Europace 21:1001–1012

    Article  Google Scholar 

  29. Frommeyer G, Reinke F, Andresen D, Kleemann T, Spitzer SG, Jehle J, Brachmann J, Stellbrink C, Hochadel M, Sengers J, Eckardt L (2019) Implantable cardioverter defibrillators in patients with electrical heart disease and hypertrophic cardiomyopathy: data from the German device registry. Clin Res Cardiol. https://doi.org/10.1007/s00392-019-01532-9

    Article  PubMed  Google Scholar 

  30. Veltmann C, Schimpf R, Echternach C, Eckardt L, Kuschyk J, Streitner F, Spehl S, Borggrefe M, Wolpert C (2006) A prospective study on spontaneous fluctuations between diagnostic and non-diagnostic ECGs in Brugada syndrome: implications for correct phenotyping and risk stratification. Eur Heart J 27:2544–2552

    Article  Google Scholar 

  31. Gonzalez Corcia MC, de Asmundis C, Chierchia GM, Brugada P (2016) Brugada syndrome in the paediatric population: a comprehensive approach to clinical manifestations, diagnosis, and management. Cardiol Young 26:1044–1055

    Article  Google Scholar 

  32. Milberg P, Tegelkamp R, Osada N, Schimpf R, Wolpert C, Breithardt G, Borggrefe M, Eckardt L (2007) Reduction of dispersion of repolarization and prolongation of postrepolarization refractoriness explain the antiarrhythmic effects of quinidine in a model of short QT syndrome. J Cardiovasc Electrophysiol 18:658–664

    Article  Google Scholar 

  33. Andorin A, Gourraud JB, Mansourati J, Fouchard S, le Marec H, Maury P, Mabo P, Hermida JS, Deharo JC, Delasalle B, Esnault S, Sadoul N, Davy JM, Leenhardt A, Klug D, Defaye P, Babuty D, Sacher F, Probst V (2017) The QUIDAM study: hydroquinidine therapy for the management of Brugada syndrome patients at high arrhythmie risk. Heart Rhythm 14:1147–1154

    Article  Google Scholar 

  34. Nademanee K, Veerakul G, Chandanamattha P, Ariyachaipanich A, Jirasirirojanakorn K, Likittanasombat K, Bhuripanyo K, Ngarmukos T (2011) Prevention of ventricular fibrillation episodes in Brugada syndrome by catheter ablation over the anterior right ventricular outflow tract epicardium. Circulation 123:1270–1279

    Article  Google Scholar 

  35. Pappone C, Ciconte G, Manguso F, Vicedomini G, Mecarocci V, Conti M, Giannelli L, Pozzi P, Borrelli V, Menicanti L, Calovic Z, Della Ratta G, Brugada J, Santinelli V (2018) Assessing the malignant ventricular arrhythmic substrate in patients with Brugada syndrome. J Am Coll Cardiol 71:1631–1646

    Article  Google Scholar 

  36. Pappone C, Brugada J, Vicedomini G, Ciconte G, Manguso F, Saviano M, Vitale R, Cuko A, Giannelli L, Calovic Z, Conti M, Pozzi P, Natalizia A, Crisà S, Borrelli V, Brugada R, Sarquella-Brugada G, Guazzi M, Frigiola A, Menicanti L, Santinelli V (2017) Electrical substrate elimination in 135 consecutive patients with Brugada syndrome. Circulation 10:e5053. https://doi.org/10.1161/CIRCEP.117.005053

    Article  PubMed  Google Scholar 

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  1. Klinik für Kardiologie II – Rhythmologie, Universitätsklinikum Münster, Albert-Schweitzer-Campus 1, Gebäude A1, 48149, Münster, Deutschland

    Lars Eckardt

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Correspondence to Lars Eckardt.

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Für diesen Beitrag wurden von den Autoren keine Studien an Menschen oder Tieren durchgeführt. Für die aufgeführten Studien gelten die jeweils dort angegebenen ethischen Richtlinien.

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Eckardt, L. Brugada-Syndrom. Herzschr Elektrophys 31, 39–47 (2020). https://doi.org/10.1007/s00399-020-00667-x

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