Andrade JG, Verma A, Mitchell LB, et al; CCS Atrial Fibrillation Guidelines Committee. 2018 Focused Update of the Canadian Cardiovascular Society Guidelines for the Management of Atrial Fibrillation. Can J Cardiol. 2018 Nov;34(11):1371-1392. doi: 10.1016/j.cjca.2018.08.026. PubMed PMID: 30404743.
January CT, Wann LS, Alpert JS, et al; ACC/AHA Task Force Members. 2014 AHA/ACC/HRS guideline for the management of patients with atrial fibrillation: a report of the American College of Cardiology/American Heart Association Task Force on practice guidelines and the Heart Rhythm Society. Circulation. 2014 Dec 2;130(23):e199-267. doi: 10.1161/CIR.0000000000000041. Epub 2014 Mar 28. Erratum in: Circulation. 2014 Dec 2;130(23):e272-4. PubMed PMID: 24682347; PubMed Central PMCID: PMC4676081.
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 Dec;10(12):1932-63. doi: 10.1016/j.hrthm.2013.05.014. Epub 2013 Aug 30. Review. PubMed PMID: 24011539.
Brignole M, Auricchio A, Baron-Esquivias G, et al. 2013 ESC Guidelines on cardiac pacing and cardiac resynchronization therapy: the Task Force on cardiac pacing and resynchronization therapy of the European Society of Cardiology (ESC). Developed in collaboration with the European Heart Rhythm Association (EHRA). Eur Heart J. 2013 Aug;34(29):2281-329. doi: 10.1093/eurheartj/eht150. Epub 2013 Jun 24. PubMed PMID: 23801822.
American College of Cardiology Foundation; American Heart Association; European Society of Cardiology; Heart Rhythm Society, Wann LS, Curtis AB, Ellenbogen KA, et al. Management of patients with atrial fibrillation (compilation of 2006 ACCF/AHA/ESC and 2011 ACCF/AHA/HRS recommendations): a report of the American College of Cardiology/American Heart Association Task Force on practice guidelines. Circulation. 2013 May 7;127(18):1916-26. doi: 10.1161/CIR.0b013e318290826d. Epub 2013 Apr 1. PubMed PMID: 23545139.
Camm AJ, Lip GY, De Caterina R, et al; ESC Committee for Practice Guidelines (CPG). 2012 focused update of the ESC Guidelines for the management of atrial fibrillation: an update of the 2010 ESC Guidelines for the management of atrial fibrillation. Developed with the special contribution of the European Heart Rhythm Association. Eur Heart J. 2012 Nov;33(21):2719-47. doi: 10.1093/eurheartj/ehs253. Epub 2012 Aug 24. Erratum in: Eur Heart J. 2013 Mar;34(10):790. Eur Heart J. 2013 Sep;34(36):2850-1. PubMed PMID: 22922413.
Tracy CM, Epstein AE, Darbar D, et al; American College of Cardiology Foundation; American Heart Association Task Force on Practice Guidelines; Heart Rhythm Society. 2012 ACCF/AHA/HRS focused update of the 2008 guidelines for device-based therapy of cardiac rhythm abnormalities: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines and the Heart Rhythm Society. [corrected]. Circulation. 2012 Oct 2;126(14):1784-800. doi: 10.1161/CIR.0b013e3182618569. Epub 2012 Sep 10. Erratum in: Circulation. 2013 Jan 22;127(3):e357-9. Heart Rhythm Society [added]. PubMed PMID: 22965336.
Pediatric and Congenital Electrophysiology Society (PACES); Heart Rhythm Society (HRS); American College of Cardiology Foundation (ACCF); American Heart Association (AHA); American Academy of Pediatrics (AAP); Canadian Heart Rhythm Society (CHRS), Cohen MI, Triedman JK, Cannon BC, et al. PACES/HRS expert consensus statement on the management of the asymptomatic young patient with a Wolff-Parkinson-White (WPW, ventricular preexcitation) electrocardiographic pattern: developed in partnership between the Pediatric and Congenital Electrophysiology Society (PACES) and the Heart Rhythm Society (HRS). Endorsed by the governing bodies of PACES, HRS, the American College of Cardiology Foundation (ACCF), the American Heart Association (AHA), the American Academy of Pediatrics (AAP), and the Canadian Heart Rhythm Society (CHRS). Heart Rhythm. 2012 Jun;9(6):1006-24. doi: 10.1016/j.hrthm.2012.03.050. Epub 2012 May 10. PubMed PMID: 22579340.
European Heart Rhythm Association; European Association for Cardio-Thoracic Surgery, Camm AJ, Kirchhof P, Lip GY, et al. Guidelines for the management of atrial fibrillation: the Task Force for the Management of Atrial Fibrillation of the European Society of Cardiology (ESC). Eur Heart J. 2010 Oct;31(19):2369-429. doi: 10.1093/eurheartj/ehq278. Epub 2010 Aug 29. Erratum in: Eur Heart J. 2011 May;32(9):1172. PubMed PMID: 20802247.
Brugada syndrome (BrS) is a rare autosomal dominant disease that is 8 times more prevalent in men than in women; it is also more frequent in eastern Asia, where it affects 0.05% to 0.1% of the general population. Disease onset is usually between the age of 20 and 40 years but sometimes may occur earlier, particularly in patients with malignant arrhythmias.
Key clinical features include syncope caused by fast polymorphic ventricular tachycardia (VT), cardiac arrest, or sudden cardiac death (SCD). SCD typically occurs at night. Syncope may be a preliminary symptom.
Electrocardiography (ECG) during sinus rhythm has a distinctive pattern and is characterized by an ST-segment elevation of ≥2 mm with a negative T wave in ≥1 right precordial leads (V1-V3) positioned in the fourth, third, or second intercostal space. This Brugada ECG pattern can be transient and is typically apparent in patients with high fever. Sodium channel blocker antiarrhythmic drugs (flecainide, procainamide, ajmaline) can unmask the pattern.
There are 3 described ECG patterns but only the type 1 morphology is diagnostic of BrS. In case of clinical suspicion of BrS in the absence of a spontaneous type 1 ST-segment elevation, a pharmacologic challenge using a sodium channel blocker is recommended. When a type 1 ST-segment elevation is unmasked using a sodium channel blocker, the diagnosis of BrS should require that the patient also present with 1 of the following: documented ventricular fibrillation (VF) or polymorphic VT, syncope of a probable arrhythmic cause, family history of SCD at <45 years with negative autopsy, coved-type ECG in family members, or nocturnal gasping. The diagnosis of BrS can be challenging.
Differential diagnosis of right precordial ST-segment elevation includes atypical right bundle branch block, early repolarization, acute myocardial ischemia, arrhythmogenic right ventricular cardiomyopathy, and pectus excavatum.
Classification of antiarrhythmic drugs: see Table 3.4-1.
Antiarrhythmic agents: see Table 3.4-2.
1. Implantable cardioverter-defibrillator (ICD) is the first-line therapy for patients with BrS presenting with aborted SCD or documented VT or VF. ICDs can be used prophylactically in BrS patients with a spontaneous manifest ECG pattern (type 1) and unexplained syncope.
2. Asymptomatic patients with a Brugada ECG pattern (type 1), either spontaneous or induced by sodium channel blockers, can be closely followed without an ICD after seeking expert advice.
3. Quinidine may be considered, if available, in BrS patients presenting with electrical storm or frequent ICD shocks. It may also be useful in patients who qualify for an ICD but the device is unavailable, refused, or contraindicated.