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Brugada Syndrome: A Rare Life-Threatening Condition.

The authors declare that there is no conflict of interest regarding the publication of this paper.


Brugada Syndrome is a rare inherited arrhythmic disorder that can lead to sudden cardiac death (SCD) due to ventricular fibrillation (VF), which can be the first manifestation. We are reporting a case of a 61-year-old male who presented to the ED with a sore throat and was found to have ST elevations in V1 and V2 leads, characteristic for Brugada pattern (BrP) type 1.

Case Description

A 61-year-old male patient with a history of hypertension, diabetes mellitus, COPD and recent history of unexplained syncope. He presented to the ED complaining of a sore throat and general weakness for one week. He denied chest pain, palpitations, or shortness of breath. His vital signs showed a heart rate of 110, the rest of his vital signs are within normal limits. Physical exam was unremarkable except for a diffusely erythematous pharynx. Initial workup was notable only for elevated WBCs. Due to Tachycardia and general weakness an EKG was done. It showed ST elevations in V1 and V2 leads, which is characteristic of Brugada pattern type 1, as shown in Figure 1. As the ED physician was not familiar with the Brugada pattern, he diagnosed him with an ST elevation MI. The patient was transferred to a near hospital for left heart catheterization (LHC). The heart catheterization showed mild non-obstructive CAD with preserved ejection fraction. The patient had no significant medical history other than a recent history of hospitalization due to unexplained syncope during which a comprehensive workup was done that was unrevealing. He denied any history of ventricular fibrillation, ventricular tachycardia, or family history of sudden cardiac death. Repeat ECG showed no ST elevation, as shown in Figure 2. The patient was diagnosed with Brugada Syndrome triggered by viral pharyngitis based on the characteristic Brugada type 1 pattern on ECG along with the history of unexplained syncope. He was discharged home with ICD.


Brugada Syndrome is an arrhythmic genetic syndrome inherited as autosomal dominant with incomplete penetrance and variable expressivity. (1,2) Genetic analysis in these patients had shown >100 SCN5A mutations which encode for sodium channels. These mutations result in an accelerated inactivation of sodium channels, which predisposes them to ventricular arrhythmias. (3) The prevalence of asymptomatic Brugada Syndrome in the general population is 0.05%. (3,4) Typically this syndrome occurs in patients who are in the age group of 30 to 60 years, and 87% are males. (3) Patients with Brugada Syndrome are at risk for sudden cardiac death (SCD) due to ventricular fibrillation (VF), which can be the first manifestation. Patients may present with syncope or can be asymptomatic for life. It is characterized by persistent or transient ST elevation with successive negative T wave in the right precordial leads (V1, V2) with or without right bundle branch block in the absence of structural heart abnormalities. (1-3) These ECG changes are dynamic, often hidden, and may reveal themselves in the presence of triggers like fever, intoxication (alcohol, cocaine, or cannabis), vagal stimulation, electrolyte imbalance, anesthetics (propofol, bupivacaine), psychotropic agents (amitriptyline, lithium), or sodium channel blockers. (3,5) In our case, the trigger was the viral pharyngitis in the absence of documented fever. Type I Brugada pattern is 20 times more likely to occur in febrile patients than afebrile patients. (3,6) The criteria for the diagnosis of Brugada syndrome (BrS) include the presence one of the typical ECG patterns (mainly Coved-type ST-segment elevation and negative T wave in the right precordial leads with or without a drug challenge test). One of these patterns in conjunction with the presence one of the following criteria: a history of documented ventricular fibrillation, a history of documented ventricular tachycardia, family history of sudden cardiac death (SCD) before the age of 45, a family history of type 1 Brugada pattern ECG changes, nocturnal agonal respiration during sleep, unexplained syncope suggestive of tachyarrhythmia, or inducible ventricular tachycardia/ventricular fibrillation during electrophysiological study. (3,4) ECG patterns described in Brugada Syndrome: Type 1 (coved-type ST-segment elevation) defined as ST-segment elevation of [greater than or equal to]2 mm (0.2 mV), followed by a negative T wave with little or no isoelectric separation in the right precordial leads. Type 2 (saddle-back type ST-segment elevation) defined as a J wave amplitude of [greater than or equal to]2 mm (0.2 mV), gives rise to a gradually descending ST-segment elevation (remaining [greater than or equal to]1 mm above the baseline) followed by a positive or biphasic T wave that results in a saddle-back configuration. Type 3 is a right precordial ST-segment elevation (saddle-back type, coved-type, or both) without meeting the standard criteria for type 1 or 2. (3,4,7,8) Type 2 Brugada pattern has a benign outcome according to multiple studies. On the other hand, patients with Type 1 Brugada pattern are almost 300 times more likely to have sudden cardiac death as compared with the general population. (4)

Brugada Syndrome (BrS) has no known cure. Prevention of malignant arrhythmias is the target of therapy. (4,9) For patients with BrS with a history of ventricular tachycardia/ventricular fibrillation or syncope suggestive of malignant arrhythmia, ICD is the first-line therapy. (3) ICD indication in asymptomatic patients needs careful judgment. Small studies have shown the benefit of quinidine, but overall, drug therapy is not proven to be beneficial. (10)


There are several triggering factors for ECG changes in patients with Brugada Syndrome that have been described in the literature including fever, intoxication (alcohol, cocaine, or cannabis), electrolyte imbalance and many others. Physicians should be aware of BrS and BrP as it can be found in any routine ECG done for patients who present with non-cardiac illnesses.


(1.) 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. Journal of the American College of Cardiology. 1992;20(6):1391-6.

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

(3.) Mizusawa Y, Wilde AA. Brugada syndrome. Circ Arrhythm Electrophysiol. 2012;5(3):606-16.

(4.) Manohar S, Dahal BR, Gitler B. Fever-Induced Brugada Syndrome. J Investig Med-HIgh Impact Case Rep. 2015;3(1):2324709615577414.

(5.) Junttila MJ, Raatikainen MJ, Karjalainen J, Kauma H, Kesaniemi YA, Huikuri HV. Prevalence and prognosis of subjects with Brugada-type ECG pattern in a young and middle-aged Finnish population. Eur Heart J. 2004;25(10):874-8.

(6.) Adler A, Topaz G, Heller K, Zeltser D, Ohayon T, Rozovski U, et al. Fever-induced Brugada pattern: how common is it and what does it mean? Heart Rhythm. 2013;10(9):1375-82.

(7.) Shimizu W, Matsuo K, Takagi M, Tanabe Y, Aiba T, Taguchi A, et al. Body surface distribution and response to drugs of ST-segment elevation in Brugada syndrome: clinical implication of eighty-seven-lead body surface potential mapping and its application to twelve-lead electrocardiograms. J Cardiovasc Electrophysiol. 2000;11(4):396-404.

(8.) 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. 2009;2(2):154-61.

(9.) Schmidt B, Chun KR, Baensch D, Antz M, Koektuerk B, Tilz RR, et al. Catheter ablation for ventricular tachycardia after failed endocardial ablation: epicardial substrate or inappropriate endocardial ablation? Heart Rhythm. 2010;7(12):1746-52.

(10.) Belhassen B, Glick A, Viskin S. Efficacy of quinidine in high-risk patients with Brugada syndrome. Circulation. 2004;110(13):1731-7.

Ahmed Amro, MD

Department of Internal Medicine, Marshall University Joan C. Edwards School of Medicine, Huntington, WV

Alaa Gabi, MD

Haytham Aljoudi, MD

Rameez Sayyed, MD, FACC, FSCAI

Department of Cardiovascular Services, Marshall University Joan C. Edwards School of Medicine, Huntington, WV

Corresponding Author: Ahmed Amro, MD, Dept. of Internal Medicine, JCESOM, 1600 Medical Center Dr., Huntington, WV 25701. Email:
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Title Annotation:Case Report
Author:Amro, Ahmed; Gabi, Alaa; Aljoudi, Haytham; Sayyed, Rameez
Publication:West Virginia Medical Journal
Article Type:Case study
Date:Nov 1, 2017
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