Skip to main content

Brugada-Syndrom

  • Living reference work entry
  • First Online:
  • 457 Accesses

Part of the book series: Springer Reference Medizin ((SRM))

Zusammenfassung

Das Brugada-Syndrom ist eine erbliche, heterogene Ionenkanalerkrankung (MIM-Eintrag: 601144), die durch charakteristische rechtspräkordiale EKG-Veränderungen, dem Fehlen von strukturellen Herzauffälligkeiten und einem erhöhten Risiko für den plötzlichen Herztod gekennzeichnet ist. Typische Symptome sind Synkopen, Kammerflimmern bzw. überlebter plötzlicher Herztod, nächtliche agonale Respiration, Palpitationen und/oder Missempfindungsstörungen im Brustkorbbereich. Diese Symptome entstehen überwiegend in Ruhe bzw. Schlaf, während eines Fieberanstiegs oder unter vagotonen Bedingungen. Das Syndrom wird durch eine Typ-1-ST-Segment-Elevation (Typ-1-EKG) diagnostiziert. Eine spezifische dauerhafte Medikation zur Behandlung des Brugada-Syndroms ist nicht verfügbar. Eine ICD-Behandlung ist derzeit die einzige effektive Strategie zur Verhinderung des plötzlichen Herztodes bei Patienten mit Brugada-Syndrom.

This is a preview of subscription content, log in via an institution.

Literatur

  • Ackerman MJ, Priori SG, Willems S, Berul C, Brugada R, Calkins H, Camm AJ, Ellinor PT, Gollob M, Hamilton R, Hershberger RE, Judge DP, Le MH, McKenna WJ, Schulze-Bahr E, Semsarian C, Towbin JA, Watkins H, Wilde A, Wolpert C, Zipes DP (2011) HRS/EHRA expert consensus statement on the state of genetic testing for the channelopathies and cardiomyopathies this document was developed as a partnership between the Heart Rhythm Society (HRS) and the European Heart Rhythm Association (EHRA). Heart Rhythm 8:1308–1339

    Article  PubMed  Google Scholar 

  • Amin AS, de Groot EA, Ruijter JM, Wilde AA, Tan HL (2009) Exercise-induced ECG changes in Brugada syndrome. Circ Arrhythm Electrophysiol 2:531–539

    Article  PubMed  Google Scholar 

  • Antzelevitch C, Brugada P, Borggrefe M, Brugada J, Brugada R, Corrado D, Gussak I, LeMarec H, Nademanee K, Perez Riera AR, Shimizu W, Schulze-Bahr E, Tan H, Wilde A (2005) Brugada syndrome: report of the second consensus conference. Heart Rhythm 2:429–440

    Article  PubMed  Google Scholar 

  • Benito B, Sarkozy A, Mont L, Henkens S, Berruezo A, Tamborero D, Arzamendi D, Berne P, Brugada R, Brugada P, Brugada J (2008) Gender differences in clinical manifestations of Brugada syndrome. J Am Coll Cardiol 52:1567–1573

    Article  PubMed  Google Scholar 

  • Bezzina CR, Shimizu W, Yang P, Koopmann TT, Tanck MW, Miyamoto Y, Kamakura S, Roden DM, Wilde AA (2006) Common sodium channel promoter haplotype in asian subjects underlies variability in cardiac conduction. Circulation 113:338–344

    Article  CAS  PubMed  Google Scholar 

  • Bezzina CR, Barc J, Mizusawa Y, Remme CA, Gourraud JB, Simonet F, Verkerk AO, Schwartz PJ, Crotti L, Dagradi F, Guicheney P, Fressart V, Leenhardt A, Antzelevitch C, Bartkowiak S, Borggrefe M, Schimpf R, Schulze-Bahr E, Zumhagen S, Behr ER, Bastiaenen R, Tfelt-Hansen J, Olesen MS, Kaab S, Beckmann BM, Weeke P, Watanabe H, Endo N, Minamino T, Horie M, Ohno S, Hasegawa K, Makita N, Nogami A, Shimizu W, Aiba T, Froguel P, Balkau B, Lantieri O, Torchio M, Wiese C, Weber D, Wolswinkel R, Coronel R, Boukens BJ, Bezieau S, Charpentier E, Chatel S, Despres A, Gros F, Kyndt F, Lecointe S, Lindenbaum P, Portero V, Violleau J, Gessler M, Tan HL, Roden DM, Christoffels VM, Le MH, Wilde AA, Probst V, Schott JJ, Dina C, Redon R (2013) Common variants at SCN5A-SCN10A and HEY2 are associated with Brugada syndrome, a rare disease with high risk of sudden cardiac death. Nat Genet 45:1044–1049

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  • Brugada P, Brugada J (1992) Right bundle branch block, persistent ST segment elevation and sudden cardiac death: a distinct clinical and electrocardiographic syndrome. J Am Coll Cardiol 20:1391–1396

    Article  CAS  PubMed  Google Scholar 

  • Brugada J, Brugada R, Brugada P (1998) Syndrome of right bundle branch block, ST segment evaluation from V1 to V3 and sudden cardiac death (editorial). Rev Esp Cardiol 51:169–170

    Article  CAS  PubMed  Google Scholar 

  • Brugada J, Brugada R, Antzelevitch C, Towbin J, Nademanee K, Brugada P (2002) Long-term follow-up of individuals with the electrocardiographic pattern of right bundle-branch block and ST-segment elevation in precordial leads V1 to V3. Circulation 105:73–78

    Article  PubMed  Google Scholar 

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

    Article  PubMed  Google Scholar 

  • 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  PubMed  Google Scholar 

  • Di Diego JM, Cordeiro JM, Goodrow RJ, Fish JM, Zygmunt AC, Perez GJ, Scornik FS, Antzelevitch C (2002) Ionic and cellular basis for the predominance of the Brugada syndrome phenotype in males. Circulation 106:2004–2011

    Article  PubMed  Google Scholar 

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

    Article  PubMed  Google Scholar 

  • Gehi AK, Duong TD, Metz LD, Gomes JA, Mehta D (2006) Risk stratification of individuals with the Brugada electrocardiogram: a meta-analysis. J Cardiovasc Electrophysiol 17:577–583

    Article  PubMed  Google Scholar 

  • Giustetto C, Drago S, Demarchi PG, Dalmasso P, Bianchi F, Masi AS, Carvalho P, Occhetta E, Rossetti G, Riccardi R, Bertona R, Gaita F (2009) Risk stratification of the patients with Brugada type electrocardiogram: a community-based prospective study. Europace 11:507–513

    Article  PubMed  Google Scholar 

  • Ikeda T, Sakurada H, Sakabe K, Sakata T, Takami M, Tezuka N, Nakae T, Noro M, Enjoji Y, Tejima T, Sugi K, Yamaguchi T (2001) Assessment of noninvasive markers in identifying patients at risk in the Brugada syndrome: insight into risk stratification. J Am Coll Cardiol 37:1628–1634

    Article  CAS  PubMed  Google Scholar 

  • Kakishita M, Kurita T, Matsuo K, Taguchi A, Suyama K, Shimizu W, Aihara N, Kamakura S, Yamamoto F, Kobayashi J, Kosakai Y, Ohe T (2000) Mode of onset of ventricular fibrillation in patients with Brugada syndrome detected by implantable cardioverter defibrillator therapy. J Am Coll Cardiol 36:1646–1653

    Article  CAS  PubMed  Google Scholar 

  • Kamakura S, Ohe T, Nakazawa K, Aizawa Y, Shimizu A, Horie M, Ogawa S, Okumura K, Tsuchihashi K, Sugi K, Makita N, Hagiwara N, Inoue H, Atarashi H, Aihara N, Shimizu W, Kurita T, Suyama K, Noda T, Satomi K, Okamura H, Tomoike H (2009) Long-term prognosis of probands with Brugada-pattern ST-elevation in leads V1-V3. Circ Arrhythm Electrophysiol 2:495–503

    Article  PubMed  Google Scholar 

  • Kusano KF, Taniyama M, Nakamura K, Miura D, Banba K, Nagase S, Morita H, Nishii N, Watanabe A, Tada T, Murakami M, Miyaji K, Hiramatsu S, Nakagawa K, Tanaka M, Miura A, Kimura H, Fuke S, Sumita W, Sakuragi S, Urakawa S, Iwasaki J, Ohe T (2008) Atrial fibrillation in patients with Brugada syndrome relationships of gene mutation, electrophysiology, and clinical backgrounds. J Am Coll Cardiol 51:1169–1175

    Article  PubMed  Google Scholar 

  • Makimoto H, Nakagawa E, Takaki H, Yamada Y, Okamura H, Noda T, Satomi K, Suyama K, Aihara N, Kurita T, Kamakura S, Shimizu W (2010) Augmented ST-segment elevation during recovery from exercise predicts cardiac events in patients with Brugada syndrome. J Am Coll Cardiol 56:1576–1584

    Article  PubMed  Google Scholar 

  • Makimoto H, Kamakura S, Aihara N, Noda T, Nakajima I, Yokoyama T, Doi A, Kawata H, Yamada Y, Okamura H, Satomi K, Aiba T, Shimizu W (2012) Clinical impact of the number of extrastimuli in programmed electrical stimulation in patients with Brugada type 1 electrocardiogram. Heart Rhythm 9:242–248

    Article  PubMed  Google Scholar 

  • Marquez MF, Bonny A, Hernandez-Castillo E, De SA, Gomez-Flores J, Nava S, Hidden-Lucet F, Iturralde P, Cardenas M, Tonet J (2012) Long-term efficacy of low doses of quinidine on malignant arrhythmias in Brugada syndrome with an implantable cardioverter-defibrillator: a case series and literature review. Heart Rhythm 9:1995–2000

    Article  PubMed  Google Scholar 

  • Meregalli PG, Tan HL, Probst V, Koopmann TT, Tanck MW, Bhuiyan ZA, Sacher F, Kyndt F, Schott JJ, Albuisson J, Mabo P, Bezzina CR, Le Marec H, Wilde AA (2009) Type of SCN5A mutation determines clinical severity and degree of conduction slowing in loss-of-function sodium channelopathies. Heart Rhythm 6:341–348

    Article  PubMed  Google Scholar 

  • Miyamoto K, Yokokawa M, Tanaka K, Nagai T, Okamura H, Noda T, Satomi K, Suyama K, Kurita T, Aihara N, Kamakura S, Shimizu W (2007) Diagnostic and prognostic value of a type 1 Brugada electrocardiogram at higher (third or second) V1 to V2 recording in men with Brugada syndrome. Am J Cardiol 99:53–57

    Article  PubMed  Google Scholar 

  • Mizusawa Y, Wilde AA (2012) Brugada syndrome. Circ Arrhythm Electrophysiol 5:606–616

    Article  PubMed  Google Scholar 

  • Morita H, Kusano-Fukushima K, Nagase S, Fujimoto Y, Hisamatsu K, Fujio H, Haraoka K, Kobayashi M, Morita ST, Nakamura K, Emori T, Matsubara H, Hina K, Kita T, Fukatani M, Ohe T (2002) Atrial fibrillation and atrial vulnerability in patients with Brugada syndrome. J Am Coll Cardiol 40:1437–1444

    Article  PubMed  Google Scholar 

  • 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 of Brugada syndrome. Circulation 118:1697–1704

    Article  PubMed  Google Scholar 

  • Nagase S, Hiramatsu S, Morita H, Nishii N, Murakami M, Nakamura K, Kusano KF, Ito H, Ohe T (2010) Electroanatomical correlation of repolarization abnormalities in Brugada syndrome: detection of type 1 electrocardiogram in the right ventricular outflow tract. J Am Coll Cardiol 56:2143–2145

    Article  PubMed  Google Scholar 

  • Poelzing S, Forleo C, Samodell M, Dudash L, Sorrentino S, Anaclerio M, Troccoli R, Iacoviello M, Romito R, Guida P, Chahine M, Pitzalis M, Deschenes I (2006) SCN5A polymorphism restores trafficking of a Brugada syndrome mutation on a separate gene. Circulation 114:368–376

    Article  CAS  PubMed  Google Scholar 

  • Priori SG, Napolitano C, Gasparini M, Pappone C, Della BP, 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  PubMed  Google Scholar 

  • Priori SG, Gasparini M, Napolitano C, Della BP, Ottonelli AG, Sassone B, Giordano U, Pappone C, Mascioli G, Rossetti G, De NR, Colombo M (2012) Risk stratification in Brugada syndrome: results of the PRELUDE (PRogrammed ELectrical stimUlation preDictive valuE) registry. J Am Coll Cardiol 59:37–45

    Article  PubMed  Google Scholar 

  • Priori SG, Wilde AA, Horie 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, Ackerman M, Belhassen B, Estes NA III, Fatkin D, Kalman J, Kaufman E, Kirchhof P, Schulze-Bahr E, Wolpert C, Vohra J, Refaat M, Etheridge SP, Campbell RM, Martin ET, Quek SC (2013) Executive summary: HRS/EHRA/APHRS expert consensus statement on the diagnosis and management of patients with inherited primary arrhythmia syndromes. Europace 15:1389–1406

    Article  PubMed  Google Scholar 

  • 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 121:635–643

    Article  CAS  PubMed  Google Scholar 

  • Rosso R, Glick A, Glikson M, Wagshal A, Swissa M, Rosenhek S, Shetboun I, Khalamizer V, Fuchs T, Boulos M, Geist M, Strasberg B, Ilan M, Belhassen B (2008) Outcome after implantation of cardioverter defibrillator [corrected] in patients with Brugada syndrome: a multicenter Israeli study (ISRABRU). Isr Med Assoc J 10:435–439

    PubMed  Google Scholar 

  • Sacher F, Probst V, Iesaka Y, Jacon P, Laborderie J, Mizon-Gerard F, Mabo P, Reuter S, Lamaison D, Takahashi Y, O'Neill MD, Garrigue S, Pierre B, Jais P, Pasquie JL, Hocini M, Salvador-Mazenq M, Nogami A, Amiel A, Defaye P, Bordachar P, Boveda S, Maury P, Klug D, Babuty D, Haissaguerre M, Mansourati J, Clementy J, Le Marec H (2006) Outcome after implantation of a cardioverter-defibrillator in patients with Brugada syndrome: a multicenter study. Circulation 114:2317–2324

    Article  PubMed  Google Scholar 

  • Sarkozy A, Boussy T, Kourgiannides G, Chierchia GB, Richter S, De Potter T, Geelen P, Wellens F, Dingena SM, Brugada P (2007) Long-term follow-up of primary prophylactic implantable cardioverter-defibrillator therapy in Brugada syndrome. Eur Heart J 28:334–344

    Article  PubMed  Google Scholar 

  • Schweizer PA, Becker R, Katus HA, Thomas D (2010) Successful acute and long-term management of electrical storm in Brugada syndrome using orciprenaline and quinine/quinidine. Clin Res Cardiol 99:467–470

    Article  PubMed  Google Scholar 

  • Shimizu W (2005) Acquired forms of the Brugada syndrome. J Electrocardiol 38:22–25

    Article  PubMed  Google Scholar 

  • Shimizu W, Matsuo K, Kokubo Y, Satomi K, Kurita T, Noda T, Nagaya N, Suyama K, Aihara N, Kamakura S, Inamoto N, Akahoshi M, Tomoike H (2007) Sex hormone and gender difference – role of testosterone on male predominance in Brugada syndrome. J Cardiovasc Electrophysiol 18:415–421

    Article  PubMed  Google Scholar 

  • Van HF, Campian ME, Spijkerboer A, Blom MT, Planken RN, van Rossum AC, de Bakker JM, Wilde AA, Groenink M, Tan HL (2012) SCN5A mutations in Brugada syndrome are associated with increased cardiac dimensions and reduced contractility. PLoS One 7:e42037

    Article  Google Scholar 

  • Viswanathan PC, Benson DW, Balser JR (2003) A common SCN5A polymorphism modulates the biophysical effects of an SCN5A mutation. J Clin Invest 111:341–346

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  • Zipes DP, Camm AJ, Borggrefe M, Buxton AE, Chaitman B, Fromer M, Gregoratos G, Klein G, Moss AJ, Myerburg RJ, Priori SG, Quinones MA, Roden DM, Silka MJ, Tracy C, Smith SC Jr, Jacobs AK, Adams CD, Antman EM, Anderson JL, Hunt SA, Halperin JL, Nishimura R, Ornato JP, Page RL, Riegel B, Priori SG, Blanc JJ, Budaj A, Camm AJ, Dean V, Deckers JW, Despres C, Dickstein K, Lekakis J, McGregor K, Metra M, Morais J, Osterspey A, Tamargo JL, Zamorano JL (2006) ACC/AHA/ESC 2006 guidelines for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death: a report of the American College of Cardiology/American Heart Association Task Force and the European Society of Cardiology Committee for Practice Guidelines (Writing committee to develop guidelines for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death). J Am Coll Cardiol 48:e247–e346

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Eric Schulze-Bahr .

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 2015 Springer-Verlag Berlin Heidelberg

About this entry

Cite this entry

Schulze-Bahr, E. (2015). Brugada-Syndrom. In: Lehnert, H., et al. SpringerReference Innere Medizin. Springer Reference Medizin. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-54676-1_199-1

Download citation

  • DOI: https://doi.org/10.1007/978-3-642-54676-1_199-1

  • Received:

  • Accepted:

  • Published:

  • Publisher Name: Springer, Berlin, Heidelberg

  • Online ISBN: 978-3-642-54676-1

  • eBook Packages: Springer Referenz Medizin

Publish with us

Policies and ethics