Brugada Syndrome Clinical Presentation

  • Author: Jose M Dizon, MD; Chief Editor: Jeffrey N Rottman, MD   more...
 
Updated: May 8, 2012
 

History

Syncope and cardiac arrest are the most common clinical manifestations leading to the diagnosis of Brugada syndrome. Nightmares or thrashing at night may occur. However, some patients remain asymptomatic, and the diagnosis of Brugada syndrome is suggested by a routine ECG showing ST-segment elevation in leads V1 through V3.

A family history of sudden cardiac death is common, though not universal, as the syndrome can occur sporadically. In about 20% of patients, atrial fibrillation is an associated arrhythmia.[20]

The context of the cardiac event is important. In many cases, cardiac arrest occurs during sleep or rest. Cases occurring during physical activity are rare. Fever is often reported to trigger or exacerbate the clinical manifestations of Brugada syndrome.

A 2012 study suggests that the quality of symptoms prior to syncope can predict a benign or malignant cause in patients with Brugada syndrome. Specifically, the lack of a prodrome was more common in patients with ventricular fibrillation documented as the cause of syncope.[21]

Physical Examination

The physical examination is usually normal in patients with Brugada syndrome. Nevertheless, physical examination is required to rule out other possible cardiac causes of syncope or cardiac arrest in an otherwise healthy patient (eg, heart murmurs from hypertrophic cardiomyopathy or from a valvular or septal defect).

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Contributor Information and Disclosures
Author

Jose M Dizon, MD  Associate Professor of Medicine and Surgery, Clinical Electrophysiology Laboratory, Division of Cardiology, Columbia University College of Physicians and Surgeons; Consulting Staff, Department of Medicine, New York-Presbyterian Hospital, Columbia University Medical Center

Jose M Dizon, MD is a member of the following medical societies: American College of Cardiology and Heart Rhythm Society

Disclosure: Nothing to disclose.

Coauthor(s)

Tamim M Nazif, MD  Fellow, Division of Cardiology, Columbia University College of Physicians and Surgeons

Tamim M Nazif, MD, is a member of the following medical societies: American College of Cardiology

Disclosure: Nothing to disclose.

Specialty Editor Board

Justin D Pearlman, MD, ME, PhD, FACC, MA  Chief, Division of Cardiology, Director of Cardiology Consultative Service, Director of Cardiology Clinic Service, Director of Cardiology Non-Invasive Laboratory, Director of Cardiology Quality Program KMC, Dartmouth-Hitchcock Medical Center, Dartmouth Medical School

Justin D Pearlman, MD, ME, PhD, FACC, MA is a member of the following medical societies: American College of Cardiology, American College of Physicians, American Federation for Medical Research, International Society for Magnetic Resonance in Medicine, and Radiological Society of North America

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Ronald J Oudiz, MD, FACP, FACC, FCCP  Professor of Medicine, University of California, Los Angeles, David Geffen School of Medicine; Director, Liu Center for Pulmonary Hypertension, Division of Cardiology, LA Biomedical Research Institute at Harbor-UCLA Medical Center

Ronald J Oudiz, MD, FACP, FACC, FCCP is a member of the following medical societies: American College of Cardiology, American College of Chest Physicians, American College of Physicians, American Heart Association, and American Thoracic Society

Disclosure: Actelion Grant/research funds Clinical Trials + honoraria; Encysive Grant/research funds Clinical Trials + honoraria; Gilead Grant/research funds Clinical Trials + honoraria; Pfizer Grant/research funds Clinical Trials + honoraria; United Therapeutics Grant/research funds Clinical Trials + honoraria; Lilly Grant/research funds Clinical Trials + honoraria; LungRx Clinical Trials + honoraria; Bayer Grant/research funds Consulting; Medtronic Consulting fee Consulting; Novartis Consulting fee Consulting

Chief Editor

Jeffrey N Rottman, MD  Professor of Medicine and Pharmacology, Vanderbilt University School of Medicine; Chief, Department of Cardiology, Nashville Veterans Affairs Medical Center

Jeffrey N Rottman, MD is a member of the following medical societies: American Heart Association and North American Society of Pacing and Electrophysiology (NASPE)

Disclosure: Nothing to disclose.

References

  1. Frustaci A, Priori SG, Pieroni M, Chimenti C, Napolitano C, Rivolta I, et al. Cardiac histological substrate in patients with clinical phenotype of Brugada syndrome. Circulation. Dec 13 2005;112(24):3680-7. [Medline].

  2. Martini B, Nava A, Thiene G, Buja GF, Canciani B, Scognamiglio R, et al. Ventricular fibrillation without apparent heart disease: description of six cases. Am Heart J. Dec 1989;118(6):1203-9. [Medline].

  3. Brugada J, Brugada R, Brugada P. Determinants of sudden cardiac death in individuals with the electrocardiographic pattern of Brugada syndrome and no previous cardiac arrest. Circulation. Dec 23 2003;108(25):3092-6. [Medline].

  4. 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. Nov 15 1992;20(6):1391-6. [Medline].

  5. Vorobiof G, Kroening D, Hall B, Brugada R, Huang D. Brugada syndrome with marked conduction disease: dual implications of a SCN5A mutation. Pacing Clin Electrophysiol. May 2008;31(5):630-4. [Medline].

  6. Kusano KF, Taniyama M, Nakamura K, Miura D, Banba K, Nagase S, et al. Atrial fibrillation in patients with Brugada syndrome relationships of gene mutation, electrophysiology, and clinical backgrounds. J Am Coll Cardiol. Mar 25 2008;51(12):1169-75. [Medline].

  7. Alings M, Wilde A. "Brugada" syndrome: clinical data and suggested pathophysiological mechanism. Circulation. Feb 9 1999;99(5):666-73. [Medline].

  8. Meregalli PG, Wilde AA, Tan HL. Pathophysiological mechanisms of Brugada syndrome: depolarization disorder, repolarization disorder, or more?. Cardiovasc Res. Aug 15 2005;67(3):367-78. [Medline].

  9. Nagase S, Kusano KF, Morita H, Nishii N, Banba K, Watanabe A, et al. Longer repolarization in the epicardium at the right ventricular outflow tract causes type 1 electrocardiogram in patients with Brugada syndrome. J Am Coll Cardiol. Mar 25 2008;51(12):1154-61. [Medline].

  10. Antzelevitch C, Brugada P, Brugada J, Brugada R. Brugada syndrome: from cell to bedside. Curr Probl Cardiol. Jan 2005;30(1):9-54. [Medline]. [Full Text].

  11. Postema PG, van Dessel PF, Kors JA, Linnenbank AC, van Herpen G, Ritsema van Eck HJ, et al. Local depolarization abnormalities are the dominant pathophysiologic mechanism for type 1 electrocardiogram in brugada syndrome a study of electrocardiograms, vectorcardiograms, and body surface potential maps during ajmaline provocation. J Am Coll Cardiol. Feb 23 2010;55(8):789-97. [Medline].

  12. Kapplinger JD, Tester DJ, Alders M, Benito B, Berthet M, Brugada J, et al. An international compendium of mutations in the SCN5A-encoded cardiac sodium channel in patients referred for Brugada syndrome genetic testing. Heart Rhythm. Jan 2010;7(1):33-46. [Medline]. [Full Text].

  13. Antzelevitch C, Pollevick GD, Cordeiro JM, Casis O, Sanguinetti MC, Aizawa Y, et al. Loss-of-function mutations in the cardiac calcium channel underlie a new clinical entity characterized by ST-segment elevation, short QT intervals, and sudden cardiac death. Circulation. Jan 30 2007;115(4):442-9. [Medline]. [Full Text].

  14. London B, Michalec M, Mehdi H, Zhu X, Kerchner L, Sanyal S, et al. Mutation in glycerol-3-phosphate dehydrogenase 1 like gene (GPD1-L) decreases cardiac Na+ current and causes inherited arrhythmias. Circulation. Nov 13 2007;116(20):2260-8. [Medline].

  15. Watanabe H, Koopmann TT, Le Scouarnec S, Yang T, Ingram CR, Schott JJ, et al. Sodium channel beta1 subunit mutations associated with Brugada syndrome and cardiac conduction disease in humans. J Clin Invest. Jun 2008;118(6):2260-8. [Medline]. [Full Text].

  16. Donohue D, Tehrani F, Jamehdor R, Lam C, Movahed MR. The prevalence of Brugada ECG in adult patients in a large university hospital in the western United States. Am Heart Hosp J. Winter 2008;6(1):48-50. [Medline].

  17. Nademanee K, Veerakul G, Nimmannit S, Chaowakul V, Bhuripanyo K, Likittanasombat K, et al. Arrhythmogenic marker for the sudden unexplained death syndrome in Thai men. Circulation. Oct 21 1997;96(8):2595-600. [Medline].

  18. Bezzina CR, Shimizu W, Yang P, Koopmann TT, Tanck MW, Miyamoto Y, et al. Common sodium channel promoter haplotype in asian subjects underlies variability in cardiac conduction. Circulation. Jan 24 2006;113(3):338-44. [Medline].

  19. [Guideline] Antzelevitch C, Brugada P, Borggrefe M, Brugada J, Brugada R, Corrado D, et al. Brugada syndrome: report of the second consensus conference: endorsed by the Heart Rhythm Society and the European Heart Rhythm Association. Circulation. Feb 8 2005;111(5):659-70. [Medline].

  20. Bordachar P, Reuter S, Garrigue S, Caï X, Hocini M, Jaïs P, et al. Incidence, clinical implications and prognosis of atrial arrhythmias in Brugada syndrome. Eur Heart J. May 2004;25(10):879-84. [Medline].

  21. Take Y, Morita H, Toh N, Nishii N, Nagase S, Nakamura K, et al. Identification of high-risk syncope related to ventricular fibrillation in patients with Brugada syndrome. Heart Rhythm. May 2012;9(5):752-9. [Medline].

  22. Wilde AA, Antzelevitch C, Borggrefe M, Brugada J, Brugada R, Brugada P, et al. Proposed diagnostic criteria for the Brugada syndrome: consensus report. Circulation. Nov 5 2002;106(19):2514-9. [Medline].

  23. Sangwatanaroj S, Prechawat S, Sunsaneewitayakul B, Sitthisook S, Tosukhowong P, Tungsanga K. New electrocardiographic leads and the procainamide test for the detection of the Brugada sign in sudden unexplained death syndrome survivors and their relatives. Eur Heart J. Dec 2001;22(24):2290-6. [Medline].

  24. Takagi M, Yokoyama Y, Aonuma K, Aihara N, Hiraoka M. Clinical characteristics and risk stratification in symptomatic and asymptomatic patients with brugada syndrome: multicenter study in Japan. J Cardiovasc Electrophysiol. Dec 2007;18(12):1244-51. [Medline].

  25. Junttila MJ, Brugada P, Hong K, Lizotte E, DE Zutter M, Sarkozy A, et al. Differences in 12-lead electrocardiogram between symptomatic and asymptomatic Brugada syndrome patients. J Cardiovasc Electrophysiol. Apr 2008;19(4):380-3. [Medline].

  26. Sarkozy A, Chierchia GB, Paparella G, Boussy T, De Asmundis C, Roos M, et al. Inferior and lateral electrocardiographic repolarization abnormalities in Brugada syndrome. Circ Arrhythm Electrophysiol. Apr 2009;2(2):154-61. [Medline].

  27. Kamakura S, Ohe T, Nakazawa K, Aizawa Y, Shimizu A, Horie M, et al. Long-term prognosis of probands with Brugada-pattern ST-elevation in leads V1-V3. Circ Arrhythm Electrophysiol. Oct 2009;2(5):495-503. [Medline].

  28. Brugada P, Geelen P, Brugada R, Mont L, Brugada J. Prognostic value of electrophysiologic investigations in Brugada syndrome. J Cardiovasc Electrophysiol. Sep 2001;12(9):1004-7. [Medline].

  29. Priori SG, Napolitano C, Gasparini M, Pappone C, Della Bella P, Giordano U, et al. Natural history of Brugada syndrome: insights for risk stratification and management. Circulation. Mar 19 2002;105(11):1342-7. [Medline].

  30. Gehi AK, Duong TD, Metz LD, Gomes JA, Mehta D. Risk stratification of individuals with the Brugada electrocardiogram: a meta-analysis. J Cardiovasc Electrophysiol. Jun 2006;17(6):577-83. [Medline].

  31. Probst V, Veltmann C, Eckardt L, Meregalli PG, Gaita F, Tan HL, et al. Long-term prognosis of patients diagnosed with Brugada syndrome: Results from the FINGER Brugada Syndrome Registry. Circulation. Feb 9 2010;121(5):635-43. [Medline].

  32. 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. Jan 3 2012;59(1):37-45. [Medline].

  33. Raju H, Papadakis M, Govindan M, et al. Low prevalence of risk markers in cases of sudden death due to brugada syndrome relevance to risk stratification in brugada syndrome. J Am Coll Cardiol. Jun 7 2011;57(23):2340-5. [Medline].

  34. Pelliccia A, Fagard R, Bjørnstad HH, Anastassakis A, Arbustini E, Assanelli D, 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. Jul 2005;26(14):1422-45. [Medline].

  35. Antzelevitch C, Brugada P, Brugada J, Brugada R, Nademanee K, Towbin J. The Brugada syndrome. In: Clinical approaches to tachyarrhythmias. Armonk NY: Futura Publishing Company; 1999.

  36. Antzelevitch C. The Brugada syndrome: ionic basis and arrhythmia mechanisms. J Cardiovasc Electrophysiol. Feb 2001;12(2):268-72. [Medline].

  37. Márquez MF, Salica G, Hermosillo AG, Pastelín G, Gómez-Flores J, Nava S, et al. Ionic basis of pharmacological therapy in Brugada syndrome. J Cardiovasc Electrophysiol. Feb 2007;18(2):234-40. [Medline].

  38. Márquez MF, Salica G, Hermosillo AG, Pastelín G, Cárdenas M. Drug therapy in Brugada syndrome. Curr Drug Targets Cardiovasc Haematol Disord. Oct 2005;5(5):409-17. [Medline].

  39. Yang F, Hanon S, Lam P, Schweitzer P. Quinidine revisited. Am J Med. Apr 2009;122(4):317-21. [Medline].

  40. Postema PG, Wolpert C, Amin AS, Probst V, Borggrefe M, Roden DM, et al. Drugs and Brugada syndrome patients: review of the literature, recommendations, and an up-to-date website (www.brugadadrugs.org). Heart Rhythm. Sep 2009;6(9):1335-41. [Medline]. [Full Text].

 
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Schematics show the 3 types of action potentials in the right ventricle: endocardial (End), mid myocardial (M), and epicardial (Epi). A, Normal situation on V2 ECG generated by transmural voltage gradients during the depolarization and repolarization phases of the action potential. B-E, Different alterations of the epicardial action potential that produce the ECG changes observed in patients with Brugada syndrome. Adapted from Antzelevitch, 2005.
Three types of ST-segment elevation in Brugada syndrome, as shown in the precordial leads on ECG in the same patient at different times. Left panel shows a type 1 ECG pattern with pronounced elevation of the J point (arrow), a coved-type ST segment, and an inverted T wave in V1 and V2. The middle panel illustrates a type 2 pattern with a saddleback ST-segment elevated by >1 mm. The right panel shows a type 3 pattern in which the ST segment is elevated < 1 mm. According to a consensus report (Antzelevitch, 2005), the type 1 ECG pattern is diagnostic of Brugada syndrome. Modified from Wilde, 2002.
Table. ECG Patterns in Brugada Syndrome
Characteristic Type 1 Type 2 Type 3
J wave amplitude≥2 mm≥2 mm≥2 mm
T waveNegativePositive or biphasicPositive
ST-T configurationCover-typeSaddlebackSaddleback
ST segment, terminal portionGradually descendingElevated by ≥1 mmElevated by < 1 mm
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