brugada syndrome

Summary

Summary: An autosomal dominant defect of cardiac conduction that is characterized by an abnormal ST-segment in leads V1-V3 on the ELECTROCARDIOGRAM resembling a right BUNDLE-BRANCH BLOCK; high risk of VENTRICULAR TACHYCARDIA; or VENTRICULAR FIBRILLATION; SYNCOPAL EPISODE; and possible sudden death. This syndrome is linked to mutations of gene encoding the cardiac SODIUM CHANNEL alpha subunit.

Top Publications

  1. Yakut K, Erdogan I, Varan B, Atar I. A Report of Brugada Syndrome Presenting with Cardiac Arrest Triggered by Verapamil Intoxication. Balkan Med J. 2017;34:576-579 pubmed publisher
    b>Brugada syndrome is a disease characterized by a specific electrocardiographic pattern and an increased risk of sudden cardiac death...
  2. Leong K, Ng F, Yao C, Roney C, Taraborrelli P, Linton N, et al. ST-Elevation Magnitude Correlates With Right Ventricular Outflow Tract Conduction Delay in Type I Brugada ECG. Circ Arrhythm Electrophysiol. 2017;10: pubmed publisher
    ..location and underlying electrophysiological mechanisms that contribute to the characteristic ECG pattern of Brugada syndrome (BrS) are still debated...
  3. Hata H, Sumitomo N, Nakai T, Amano A. Retrosternal Implantation of the Cardioverter-Defibrillator Lead in an Infant. Ann Thorac Surg. 2017;103:e449-e451 pubmed publisher
    ..The redundant retrosternal part of the lead straightened during growth, while cardiac coverage by the cardioversion energy field and the defibrillation vector were maintained appropriately. ..
  4. Zhao Y, Wang J, Huang G, Ding C. Significance of ST-segment elevation in lead aVR. Chin Med J (Engl). 2014;127:3034 pubmed
  5. Mascia G, Arbelo E, Hernandez Ojeda J, Solimene F, Brugada R, Brugada J. Brugada Syndrome and Exercise Practice: Current Knowledge, Shortcomings and Open Questions. Int J Sports Med. 2017;38:573-581 pubmed publisher
    Since its recognition as a clinical entity in 1992, the Brugada Syndrome (BrS), a hereditary disease characterized by a typical electrocardiogram (ECG) pattern potentially predisposing to sudden cardiac death (SCD), has attracted the ..
  6. Veerman C, Podliesna S, Tadros R, Lodder E, Mengarelli I, de Jonge B, et al. The Brugada Syndrome Susceptibility Gene HEY2 Modulates Cardiac Transmural Ion Channel Patterning and Electrical Heterogeneity. Circ Res. 2017;121:537-548 pubmed publisher
    ..3 (near HEY2) with Brugada syndrome. The causal gene and underlying mechanism remain unresolved...
  7. Persyn E, Karakachoff M, Le Scouarnec S, Le Clézio C, Campion D, Consortium F, et al. DoEstRare: A statistical test to identify local enrichments in rare genomic variants associated with disease. PLoS ONE. 2017;12:e0179364 pubmed publisher
    ..DoEstRare was also applied to Brugada syndrome and early-onset Alzheimer's disease data and provided complementary results to other existing tests...
  8. De Maria E, Cappelli S, Cappato R. Shock efficacy of the entirely subcutaneous defibrillator for termination of spontaneous ventricular fibrillation in Brugada syndrome. Heart Rhythm. 2013;10:1807-9 pubmed publisher
  9. Silverblatt C, Wasserman F, Baum G, Wolcott M, GREENBERGER A, TRAITZ J. Factors associated with the development of ectopic rhythms during surgery. Am J Surg. 1962;103:102-15 pubmed

More Information

Publications41

  1. Probst V, Gourraud J. Quinidine in Brugada Syndrome: Still a Long Way to Go…. Circ Arrhythm Electrophysiol. 2015;8:1309-10 pubmed publisher
  2. Ragab A, Houck C, van der Does L, Lanters E, Burghouwt D, Muskens A, et al. Usefulness of the R-Wave Sign as a Predictor for Ventricular Tachyarrhythmia in Patients With Brugada Syndrome. Am J Cardiol. 2017;120:428-434 pubmed publisher
    b>Brugada syndrome (BrS) is an autosomal dominant channelopathy which is responsible for a large number of sudden cardiac deaths in young subjects without structural abnormalities...
  3. Wang Y, Zhao X, O Neil A, Turner A, Liu X, Berk M. Altered cardiac autonomic nervous function in depression. BMC Psychiatry. 2013;13:187 pubmed publisher
    ..Individuals with depression appear to be susceptible to premature atrial and/or ventricular disease. ..
  4. Suktitipat B, Sathirareuangchai S, Roothumnong E, Thongnoppakhun W, Wangkiratikant P, Vorasan N, et al. Molecular investigation by whole exome sequencing revealed a high proportion of pathogenic variants among Thai victims of sudden unexpected death syndrome. PLoS ONE. 2017;12:e0180056 pubmed publisher
    ..WES can help to identify the genetic causes in victims of SUDS and may help to further guide investigations into their relatives to prevent additional SUDS victims. ..
  5. Obeyesekere M, Klein G, Modi S, Leong Sit P, Gula L, Yee R, et al. How to perform and interpret provocative testing for the diagnosis of Brugada syndrome, long-QT syndrome, and catecholaminergic polymorphic ventricular tachycardia. Circ Arrhythm Electrophysiol. 2011;4:958-64 pubmed publisher
  6. Faulkner M, Haley M, Littmann L. Propofol infusion syndrome with severe and dynamic Brugada electrocardiogram but benign clinical outcome. J Cardiovasc Electrophysiol. 2011;22:827-8 pubmed publisher
  7. Yagi S, Soeki T, Aihara K, Fukuda D, Ise T, Kadota M, et al. Low Serum Levels of Eicosapentaenoic Acid and Docosahexaenoic Acid are Risk Factors for Cardiogenic Syncope in Patients with Brugada Syndrome. Int Heart J. 2017;58:720-723 pubmed publisher
    ..unknown whether low serum levels of n-3 PUFAs are risk factors for ventricular fibrillation in patients with Brugada syndrome (BrS)...
  8. Li Q, Wang D, Chen B. Electrocardiogram in patients with acute inferior myocardial infarction due to occlusion of circumflex artery. Medicine (Baltimore). 2017;96:e6095 pubmed publisher
    ..05 for all). The changes of STD I, STE III < STEII, STD AVL < STD I could discriminate between LCX and RCA in AIMI patients with high sensitivity and specificity.ECG may be an effective tool to predict the IRA in patient with AIMI. ..
  9. Liao W, Juo L, Shih Y, Chen Y, Yan Y. HSPB7 prevents cardiac conduction system defect through maintaining intercalated disc integrity. PLoS Genet. 2017;13:e1006984 pubmed publisher
    ..In conclusion, our findings characterize HSPB7 as an intercalated disc protein and suggest it has an essential role in maintaining intercalated disc integrity and conduction function in the adult heart. ..
  10. Ozyilmaz I, Akyol B, Ergul Y. Sudden Cardiac Arrest While Eating a Hot Dog: A Rare Presentation of Brugada Syndrome in a Child. Pediatrics. 2017;140: pubmed publisher
    Patients who are diagnosed with Brugada syndrome (BS) usually experience sudden cardiac arrest (SCA) and arrhythmia when they have a high fever, consume alcohol, and, more frequently, during their night sleep...
  11. Pappone C, Brugada J, Vicedomini G, Ciconte G, Manguso F, Saviano M, et al. Electrical Substrate Elimination in 135 Consecutive Patients With Brugada Syndrome. Circ Arrhythm Electrophysiol. 2017;10:e005053 pubmed publisher
    ..electric activity in the epicardial right ventricular outflow tract may be beneficial in patients with Brugada syndrome. A total of 135 symptomatic Brugada syndrome patients having implantable cardiac defibrillator were enrolled: ..
  12. Corrado D, Zorzi A, Cerrone M, Rigato I, Mongillo M, Bauce B, et al. Relationship Between Arrhythmogenic Right Ventricular Cardiomyopathy and Brugada Syndrome: New Insights From Molecular Biology and Clinical Implications. Circ Arrhythm Electrophysiol. 2016;9:e003631 pubmed publisher
  13. Remme C, Wilde A. SCN5A overlap syndromes: no end to disease complexity?. Europace. 2008;10:1253-5 pubmed publisher
  14. Benito B, Berruezo A. Brugada syndrome and pregnancy: delving into the role of sex hormones in ion channelopathies. Rev Esp Cardiol (Engl Ed). 2014;67:165-7 pubmed publisher
  15. Forsblad d Elia H, Wallberg H, Klingberg E, Carlsten H, Bergfeldt L. Cardiac conduction system abnormalities in ankylosing spondylitis: a cross-sectional study. BMC Musculoskelet Disord. 2013;14:237 pubmed publisher
    ..Even relatively mild conduction system abnormalities might, however, indirectly affect morbidity and mortality. ..
  16. Brugada P. Commentary on the Brugada ECG pattern: a marker of channelopathy, structural heart disease, or neither? Toward a unifying mechanism of the Brugada syndrome. Circ Arrhythm Electrophysiol. 2010;3:280-2 pubmed publisher
  17. Ripley A, Castro J, Gadsden J. Local anesthetics, neuraxial anesthesia, and the Brugada syndrome. J Clin Anesth. 2013;25:78-9 pubmed publisher
  18. Dendramis G, Paleologo C, Sgarito G, Giordano U, Verlato R, Baranchuk A, et al. Anesthetic and Perioperative Management of Patients With Brugada Syndrome. Am J Cardiol. 2017;120:1031-1036 pubmed publisher
    b>Brugada syndrome (BrS) is an arrhythmogenic disease reported to be one among the leading causes of cardiac death in subjects under the age of 40 years...
  19. Garcia Fuertes D, Villanueva Fernández E, Crespín Crespín M, Puchol A, Pachon M, Arias M. Type 1 Brugada Pattern Unmasked During the Recovery Period of an Exercise Stress Test. Arq Bras Cardiol. 2016;106:447-9 pubmed publisher
  20. Ackerman M, Priori S, Willems S, Berul C, Brugada R, Calkins H, et al. 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. 2011;8:1308-39 pubmed publisher
  21. Mugnai G, Hunuk B, Hernandez Ojeda J, Ströker E, Velagic V, Ciconte G, et al. Role of Electrocardiographic Tpeak-Tend for the Prediction of Ventricular Arrhythmic Events in the Brugada Syndrome. Am J Cardiol. 2017;120:1332-1337 pubmed publisher
    ..Tpeak-Tend (TpTe) as a possible predictor of ventricular arrhythmic events in patients with Brugada syndrome (BrS)...
  22. Mizusawa Y, Wilde A. Brugada syndrome. Circ Arrhythm Electrophysiol. 2012;5:606-16 pubmed publisher
  23. Cerrone M, Cummings S, Alansari T, Priori S. A clinical approach to inherited arrhythmias. Circ Cardiovasc Genet. 2012;5:581-90 pubmed publisher
  24. Lown B, Ganong W, Levine S. The syndrome of short P-R interval, normal QRS complex and paroxysmal rapid heart action. Circulation. 1952;5:693-706 pubmed
  25. Frustaci A, Russo M, Chimenti C. Structural myocardial abnormalities in asymptomatic family members with Brugada syndrome and SCN5A gene mutation. Eur Heart J. 2009;30:1763 pubmed publisher
  26. Hernandez Ojeda J, Arbelo E, Borràs R, Berne P, Tolosana J, Gomez Juanatey A, et al. Patients With Brugada Syndrome and Implanted Cardioverter-Defibrillators: Long-Term Follow-Up. J Am Coll Cardiol. 2017;70:1991-2002 pubmed publisher
    Implantable cardioverter-defibrillator (ICD) indications for primary prevention in Brugada syndrome (BrS) are still debated.
  27. Reddy N, Moudgil R, Lopez Mattei J, Karimzad K, Mouhayar E, Somaiah N, et al. Progressive and Reversible Conduction Disease With Checkpoint Inhibitors. Can J Cardiol. 2017;33:1335.e13-1335.e15 pubmed publisher
    ..Prompt medical intervention resulted in full recovery. Despite the anticancer efficacy, the newer antineoplastic agents pose a significant and often life-threatening risk of cardiotoxicity. ..
  28. Ottolini M, Barker B, Gaykema R, Meisler M, Patel M. Aberrant Sodium Channel Currents and Hyperexcitability of Medial Entorhinal Cortex Neurons in a Mouse Model of SCN8A Encephalopathy. J Neurosci. 2017;37:7643-7655 pubmed publisher
    ..6 may increase excitatory drive into the hippocampus, culminating in seizure activity and SUDEP. ..
  29. Garg P, Nelson T, Sahu J, Sheridan P. Is this ST-elevation because of myocardial ischaemia or a Brugada pattern? An interesting case review. Intern Emerg Med. 2015;10:249-50 pubmed publisher
  30. Gonzalez Corcia M, Sieira J, Pappaert G, De Asmundis C, Chierchia G, Sarkozy A, et al. A Clinical Score Model to Predict Lethal Events in Young Patients (?19 Years) With the Brugada Syndrome. Am J Cardiol. 2017;120:797-802 pubmed publisher
    Risk stratification in Brugada syndrome in young patients remains challenging. We investigated the clinical characteristics, prognosis, and risk in young patients with the Brugada syndrome...
  31. Prall M, Lehmann H, Prokesch H, Richter D, Graeff C, Kaderka R, et al. Treatment of arrhythmias by external charged particle beams: a Langendorff feasibility study. Biomed Tech (Berl). 2015;60:147-56 pubmed publisher
    ..Second, our pilot study seems to suggest that no adverse effects have to be expected immediately during heavy ion irradiation in performing subsequent experiments with doses of 30-60 Gy and intact pigs. ..
  32. Campuzano O, Brugada R. Age, Genetics, and Fibrosis in the Brugada Syndrome. J Am Coll Cardiol. 2015;66:1987-1989 pubmed publisher