72. Michowitz Y, Milman A, Andorin A, et al. Characterization and Management of Arrhythmic Events in Young Patients With Brugada Syndrome. J Am Coll Cardiol 2019; 73:1756. 73. Probst V, Denjoy I, Meregalli PG, et al. Clinical aspects and prognosis of Brugada syndrome in children. Circulation 2007; 115:2042. 74. Conte G, de Asmundis C, Ciconte G, et al. Follow-up from childhood to adulthood of individuals with family history of Brugada syndrome and normal electrocardiograms. JAMA 2014; 312:2039. Topic 106759 Version 31.0 https://www.uptodate.com/contents/brugada-syndrome-clinical-presentation-diagnosis-and-evaluation/print 25/40 7/6/23, 11:28 AM Brugada syndrome: Clinical presentation, diagnosis, and evaluation - UpToDate GRAPHICS 12-lead electrocardiogram Brugada pattern Patient with Brugada pattern ECG. Note the presence of pseudo-right bundle branch block and persistent ST segment elevation in leads V1 to V2. ECG: electrocardiogram Courtesy of Ann C Garlitski, MD, FACC, FHRS. Graphic 122897 Version 1.0 https://www.uptodate.com/contents/brugada-syndrome-clinical-presentation-diagnosis-and-evaluation/print 26/40 7/6/23, 11:28 AM Brugada syndrome: Clinical presentation, diagnosis, and evaluation - UpToDate Sinus tachycardia with ST segment changes due to fever in an 11- month-old child with Brugada syndrome (A) Electrocardiography demonstrating sinus tachycardia and ST segment changes triggered by fever in an 11-month-old child with Brugada syndrome. (B) Electrocardiography reverts to normal with the resolution of fever in the same patient. Graphic 100119 Version 3.0 https://www.uptodate.com/contents/brugada-syndrome-clinical-presentation-diagnosis-and-evaluation/print 27/40 7/6/23, 11:28 AM Brugada syndrome: Clinical presentation, diagnosis, and evaluation - UpToDate Drugs that can induce Brugada-like patterns on the surface electrocardiogram (ECG) Antiarrhythmic or antianginal drugs Cardiac sodium channel blockers (some have been used for drug challenge in patients with Brugada type 2 or 3 ECG pattern) Class IC drugs (flecainide, pilsicainide, propafenone) Class IA drugs (ajmaline, procainamide, disopyramide, cibenzoline) Lithium Calcium channel blockers Beta blockers Nitrates Nicorandil (a potassium channel opener) Psychotropic drugs Tricyclic antidepressants Amitriptyline Nortriptyline Desipramine Clomipramine Tetracyclic antidepressants Maprotiline Phenothiazines Perphenazine Cyamemazine Selective serotonin reuptake inhibitors Fluoxetine Other Dimenhydrinate Cocaine intoxication Alcohol intoxication Adapted from: Antzelevitch C, Brugada P, Borggrefe M, et al. Brugada syndrome: report of the second consensus conference: endorsed by the Heart Rhythm Society and the European Heart Rhythm Association. Circulation 2005; 111:659. https://www.uptodate.com/contents/brugada-syndrome-clinical-presentation-diagnosis-and-evaluation/print 28/40 7/6/23, 11:28 AM Brugada syndrome: Clinical presentation, diagnosis, and evaluation - UpToDate Graphic 73611 Version 5.0 https://www.uptodate.com/contents/brugada-syndrome-clinical-presentation-diagnosis-and-evaluation/print 29/40 7/6/23, 11:28 AM Brugada syndrome: Clinical presentation, diagnosis, and evaluation - UpToDate Diagnostic approach to patients with intermediate or high suspicion for Brugada syndrome* ECG: electrocardiogram; VT: ventricular tachycardia. Brugada syndrome is suspected based on the combination of symptoms and typical Brugada pattern ECG changes (either type 1 or 2). Refer to related UpToDate content for further information. This is usually done with stress testing, echocardiography, and sometimes with cardiac magnetic resonance imaging. The presence of structural heart disease makes Brugada syndrome less likely and points to the structural heart disease as the probable diagnosis. A drug challenge typically involves giving a sodium channel- blocking medicine in an attempt to elicit the type 1 Brugada ECG pattern. Refer to related UpToDate content for further information. https://www.uptodate.com/contents/brugada-syndrome-clinical-presentation-diagnosis-and-evaluation/print 30/40 7/6/23, 11:28 AM Brugada syndrome: Clinical presentation, diagnosis, and evaluation - UpToDate Tests such as electrophysiologic studies and signal-averaged ECG can be used for further risk stratification and evaluation. Refer to related UpToDate content for further information. Graphic 141729 Version 1.0 https://www.uptodate.com/contents/brugada-syndrome-clinical-presentation-diagnosis-and-evaluation/print 31/40 7/6/23, 11:28 AM Brugada syndrome: Clinical presentation, diagnosis, and evaluation - UpToDate ECG patterns of Brugada syndrome in leads V1-V2 (A) This typical coved pattern present in V1-V2 shows the following: 1. At the end of QRS, an ascending and quick slope with a high take-off 2 mm followed by concave or rectilinear downsloping ST. There are few cases of coved pattern with a high take-off between 1 and 2 mm. 2. There is no clear r' wave. 3. The high take-off often does not correspond with the J point. 4. At 40 milliseconds of high take-off, the decrease in amplitude of ST is 4 mm. In RBBB and athletes, it is much higher. 5. ST at high take-off N ST at 40 milliseconds N ST at 80 milliseconds. 6. ST is followed by negative and symmetric T wave. 7. The duration of QRS is longer than in RBBB, and there is a mismatch between V1 and V6. (B) This typical saddle-back pattern present in V1-V2 shows the following: 1. High take-off of r' (that often does not coincide with J point) 2 mm. 2. Descending arm of r' coincides with beginning of ST (often is not well seen). 3. Minimum ST ascent 0.5 mm. 4. ST is followed by positive T wave in V2 (T peak N ST minimum N 0) and of variable morphology in V1. 5. The characteristics of triangle formed by r' allow to define different criteria useful for diagnosis. angle. Duration of the base of the triangle of r' at 5 mm from the high take- off greater than 3.5 mm. 6. The duration of QRS is longer in BrP type 2 than in other cases with r' in V1, and there is a mismatch between V1 and V6. RBBB: right bundle branch block. https://www.uptodate.com/contents/brugada-syndrome-clinical-presentation-diagnosis-and-evaluation/print 32/40 7/6/23, 11:28 AM Brugada syndrome: Clinical presentation, diagnosis, and evaluation - UpToDate Reproduced from: Bay s de Luna A, Brugada J, Baranchuk A, et al. Current electrocardiographic criteria for diagnosis of Brugada pattern: a consensus report. J Electrocardiol 2012; 45:433. Illustration used with the permission of Elsevier Inc. All rights reserved. Graphic 91152 Version 2.0 https://www.uptodate.com/contents/brugada-syndrome-clinical-presentation-diagnosis-and-evaluation/print 33/40 7/6/23, 11:28 AM Brugada syndrome: Clinical presentation, diagnosis, and evaluation - UpToDate Patterns of ST abnormalities in leads V1 to V3 in Brugada syndrome* Feature Type 1 Type 2 2 mm 2 mm J wave amplitude T wave Negative Positive or biphasic ST-T configuration Coved type Saddle back Elevated 1 mm ST segment (terminal portion) Gradually descending ECG: electrocardiographic. In contemporary studies of Brugada syndrome, the focus has generally been on ECG findings in leads V1 and V2 only. Modi ed with permission from: Wilde AA, Antzelevitch C, Borggrefe M, et al. Proposed diagnostic criteria for the Brugada syndrome. Eur Heart J 2002; 23:1648. Copyright 2002 Elsevier Science. Graphic 71258 Version 7.0 https://www.uptodate.com/contents/brugada-syndrome-clinical-presentation-diagnosis-and-evaluation/print 34/40 7/6/23, 11:28 AM Brugada syndrome: Clinical presentation, diagnosis, and evaluation - UpToDate Electrocardiogram (ECG) showing common right bundle branch block (RBBB) Electrocardiogram showing characteristic changes in the precordial leads in complete RBBB. The asynchronous activation of the two ventricles increases the QRS duration (0.13 seconds). The terminal forces are rightward and anterior due to the delayed activation of the right ventricle, resulting in an rsR' pattern in the anterior-posterior lead V1 and a wide negative S wave in the left-right lead V6 (and, not shown, in lead I). Courtesy of Ary Goldberger, MD. Graphic 64393 Version 7.0 Normal ECG https://www.uptodate.com/contents/brugada-syndrome-clinical-presentation-diagnosis-and-evaluation/print 35/40 7/6/23, 11:28 AM Brugada syndrome: Clinical presentation, diagnosis, and evaluation - UpToDate Normal electrocardiogram showing normal sinus rhythm at a rate of 75 beats/minute, a PR interval of 0.14 seconds, a QRS interval of 0.10 seconds, and a QRS axis of approximately 75 . Courtesy of Ary Goldberger, MD. Graphic 76183 Version 4.0 https://www.uptodate.com/contents/brugada-syndrome-clinical-presentation-diagnosis-and-evaluation/print 36/40 7/6/23, 11:28 AM Brugada syndrome: Clinical presentation, diagnosis, and evaluation - UpToDate ECG Brugada ajmaline challenge The panels display leads V1 to V3 (25 mm/ssecond, 1 cm/mV) acquired at baseline ("pre-test") and up to the 4th minute during intravenous administration of the sodium-channel blocker ajmaline in a 15-year-old female patient with suspected Brugada syndrome and a nondiagnostic baseline ECG. A type 1 Brugada pattern ECG is unmasked, which is important for prognosis and risk stratification purposes. ECG: electrocardiogram. Graphic 116958 Version 2.0 https://www.uptodate.com/contents/brugada-syndrome-clinical-presentation-diagnosis-and-evaluation/print 37/40 7/6/23, 11:28 AM Brugada syndrome: Clinical presentation, diagnosis, and evaluation - UpToDate Irregular beat-to-beat variations in the shape and amplitude of the ST-T wave d a positive diagnostic ajmaline test The figure demonstrates visible irregular beat-to-beat variations in the shape and amplitude of the ST-T wave during a positive diagnostic ajmaline test in a 58-year-old female patient with no previous history of arrhythm events. Only leads V1 and V2 recorded from their standard positions, from the 3rd intercostal space (leads V1 and V23, respectively), and 2nd intercostal space (V12 and V22) are presented (12.5 mm/second, 1 cm/mV). N the irregular pattern of ST-T wave variability. Graphic 116875 Version 1.0 https://www.uptodate.com/contents/brugada-syndrome-clinical-presentation-diagnosis-and-evaluation/print 38/40 7/6/23, 11:28 AM Brugada syndrome: Clinical presentation, diagnosis, and evaluation - UpToDate High- and intermediate-risk factors for arrhythmia or sudden cardiac arrest in patients with Brugada syndrome or pattern High-risk symptoms Intermediate-risk factors Sudden cardiac arrest Syncope that may be nonarrhythmic in origin* Syncope Family history of sudden cardiac arrest and/or Brugada syndrome Sustained ventricular tachycardia Spontaneous type 1 ECG pattern Atrial fibrillation This symptom is the most concerning of the intermediate-risk factors. Graphic 141594 Version 1.0 https://www.uptodate.com/contents/brugada-syndrome-clinical-presentation-diagnosis-and-evaluation/print 39/40 7/6/23, 11:28 AM Brugada syndrome: Clinical presentation, diagnosis, and evaluation - UpToDate Contributor Disclosures John V Wylie, MD, FACC No relevant financial relationship(s) with ineligible companies to disclose. Samuel Asirvatham, MD Grant/Research/Clinical Trial Support: Medtronic [Defibrillators]; St Jude's [Sudden Cardiac Death]. Consultant/Advisory Boards: BioTronik [Defibrillators]; Boston Scientific [Sudden Cardiac Death]. All of the relevant financial relationships listed have been mitigated. Nisha Parikh, MD, MPH No relevant financial relationship(s) with ineligible companies to disclose. Contributor disclosures are reviewed for conflicts of interest by the editorial group. When found, these are addressed by vetting through a multi-level review process, and through requirements for references to be provided to support the content. Appropriately referenced content is required of all authors and must conform to UpToDate standards of evidence. Conflict of interest policy https://www.uptodate.com/contents/brugada-syndrome-clinical-presentation-diagnosis-and-evaluation/print 40/40