Brugada Sign Research Articles

A Case of Brugada Phenocopy in Adrenal Insufficiency-Related Pericarditis

This is a report of a 27-year-old man with known history of Addison’s disease, noncompliant with medications, and hypothyroidism who presented with shortness of breath, nausea, vomiting, fever, and chest pain as well as Brugada sign seen on electrocardiogram. Echocardiogram revealed a moderate pericardial effusion and laboratory findings were suggestive of adrenal insufficiency. Patient was determined to have Type I Brugada phenocopy, which is a Brugada sign seen on EKG with a reversible cause. In this instance, the Brugada phenocopy was caused by adrenal insufficiency with associated pericarditis. Treatment with high-dose steroids led to resolution of both the pericardial effusion and Brugada sign, providing further evidence of Brugada phenocopy.

Posterior reversible encephalopathy syndrome in spotted fever group of rickettsial infection with brugada syndrome

Rickettsioses are one of the common causes of undifferentiated febrile illness. Patients present commonly with fever, rash, and eschar. Posterior Reversible Encephalopathy Syndrome (PRES) also known as reversible posterior encephalopathy syndrome is a rare condition, in which parts of the brain are affected by swelling as a result of an underlying cause such as infection. However, PRES caused by spotted fever group of rickettsiosis is rarely reported. We are here with reporting such a case. A 55-year-old male was admitted to MVJ Medical College and Research Hospital, Bengaluru, with complaints of fever for 3 days and one episode of Generalized Tonic Clonic Seizures (GTCS) following which he was in altered sensorium. On examination, there was hepatosplenomegaly. Weil-Felix OX19 and OX2 were 1:320. Lumbar puncture was done which showed 15 cells 98% lymphocytes and 2% neutrophils, proteins-119 mg/dl, and sugar- 153 mg/dl. Magnetic resonance imaging of the brain showed bilateral symmetric hyperintensities on T2-weighted imaging in the parietal and occipital lobes. The calcarine and occipital lobes were spared suggestive of posterior reversible encephalopathy syndrome. Electrocardiogram showed ST elevation in V1 and V2 along with T-wave inversion (Brugada sign). Posterior reversible encephalopathy syndrome is usually commonly seen in hypertensive encephalopathy and in infections such as dengue and few cases of scrub typhus. This is one of the rare cases where spotted fever group of rickettsia has caused posterior reversible encephalopathy syndrome and Brugada syndrome.

Brugada syndrome: new concepts and algorithms in management (RCD code: V 1A.1)

Clinical manifestation of Brugada syndrome (BrS) mainly results from polymorphic ventricular arrhythmias and includes sudden cardiac arrest (SCA). The Brugada sign, besides being present in true BrS, may result from different causes. Moreover, electrocardiogram findings in some clinical situations may resemble the BrS electrocardiographic pattern. Thus, differential diagnosis is crucial in the proper management of patients suspected of having BrS. Lifestyle modifications and close follow-up with or without pharmacologic treatment and/or implantable cardioverter-defibrillator placement constitute the most common approach to managing BrS patients. However, the role of ablation in BrS treatment is increasing. Due to diagnostic and therapeutic difficulties, the management of BrS is often challenging. This review provides new concepts and algorithms in the diagnostics and treatment of patients suspected of having BrS. JRCD 2017; 3 (5): 151–160

Exercise ECG unmasked Brugada sign: manifestation of the risk of sports-associated sudden cardiac arrest (RCD code: V-1A.1)

Sports‑associated sudden cardiac arrest (SCA) constitutes an important problem. Causes of SCA during sport activities include Brugada syndrome (BrS) among others. We describe a 29‑year‑old male, without a history of cardiovascular disease, who suffered from SCA during football training and despite intensive treatment, after almost 4 weeks of hospitalization, unfortunately died. Detailed medical documentation review showed grade I atrioventricular block, intraventricular conduction abnormalities and BrS type 2 morphology of ST-segment elevation on resting 12‑lead electrocardiogram (ECG). The patient had a history of syncope during physical activity. Further echocardiographic study did not reveal significant structural heart disease. Submaximal exercise testing was performed and showed ventricular extrasystoles during physical activity and type 1 BrS morphology of ST-segment elevation during recovery phase. We compared observed ECG changes to those present in a healthy football player and described noticeable similarities. Exercise ECG testing, especially in patients after syncope of probable arrhythmic etiology, may unmask BrS ECG pattern and lead to BrS diagnosis. JRCD 2017; 3 (3): 92–97.

Brugada sign in a patient with hyperkalemia due to rhabdomyolysis in hypothermia

The Brugada sign may appear as an indication of severe hyperkalemia. This phenomena has recently been called as the “Brugada phenocopy”. Hyperthermia and hypothermia may lead to rhabdomyolysis. Rhabdomyolysis is a syndrome characterized by muscle necrosis and the release of intracellular muscle constituents into the circulation. We present a case where rhabdomyolysis-related delayed hyperkalemia showed Brugada sign on the ECG mimicking ventricular tachycardia.

When and Why Is a Patient with a Brugada Sign in the ECG at Risk?

When and Why Is a Patient with a Brugada Sign in the ECG at Risk?

Low prevalence of Brugada-type electrocardiogram in a prospective large cohort of Egyptians

Abstract Background Brugada syndrome is a cardiac channelopathy that is associated with a high risk of VF and SCD and characterized by ECG pattern of transient or persistent, coved type ST-segment elevation in leads V 1 – V 3 . Aim To prospectively determine the prevalence of Brugada-type ECG pattern (Brugada sign) among unselected non cardiac individuals served at Cairo university teaching-hospital. Methods This study was conducted from October 2011 to September 2012, and included 4000 unselected noncardiac individuals (2078 males 52%, mean age 39 ± 14.44 years) admitted or presented to our hospital for different reasons. A 12-lead ECG was recorded for each of them; these ECGs were reviewed independently by two electrophysiologists for criteria of the three types of Brugada ECG pattern, and diagnosis of Brugada sign was made when both investigators agreed on the classification of the ECG findings according to the criteria of the Consensus Report of the Study Group of the Molecular Basis of Arrhythmia of the European Society of Cardiology. Results Twenty-two (0.55%) subjects had Brugada ECG pattern. All of them were of Mediterranean ethnic group. 18 males (0.45%), and 4 females (0.1%). No subjects showed type-1, while 11 (0.275%) of the 22 subjects had type-2, and the other 11 had type-3 Brugada ECG pattern. Eight cases (0.2%) among the 22 subjects were between 10 and 24 years old. Of the 22 subjects, 4 cases (18.2%) were symptomatic (palpitation and syncope), and 3 cases (13%) had positive F.H of SCD. Conclusion The frequency of type-1 Brugada-type ECG pattern was 0%, while type-2 and type-3 (saddleback type) was 0.55% among Egyptian hospital-based population, and it was more prevalent in middle-aged males.

ECG is not a reliable predictor of sudden cardiac death in the general population

Objectives: To determine the predictive value of the ECG for sudden death in the general population. Design: In the Copenhagen City Heart Study, a randomly selected population sample in Copenhagen, Denmarkhas been followed prospectively since 1976. From this population sample, we analyzed ECGs of individuals who had suffered sudden cardiac death (SCD) before the age of 50 years and compared them with ECGs of a randomly selected control individuals from the same population sample. Specific ECG signs that could point toward a condition associated with a risk of SCD were noted. Results: From a total of 18,974 individuals in the cohort, 207 had died at an age younger than 50 years. Among these, 24 persons with SCD were identified. The most prevalent ECG abnormality was QRS fragmentation. We found no ECGs with long or short QTc, Brugada sign or WPW. The prevalence of signs of left ventricular hyper-trophy, early repolarization, or fragmentation was not different from the prevalence of these signs in the control group. Conclusion: In the Copenhagen City Heart Study, the ECG failed to predict SCD in persons who died before the age of 50 years.

Hyperkalemia-Induced Brugada Pattern with Electrical Alternans

The Brugada syndrome is a genetic disease (mutation of the SCN5A gene) that predisposes to syncope and life-threatening sudden cardiac death. The case highlights the importance of recognizing hyperkalemia, as potential triggers of the acquired Brugada sign with electrical alternans.

Exercise-induced Brugada sign

The Brugada syndrome has been described as a genetic disorder involving aborted sudden cardiac death in patients with apparently structurally normal hearts. Exercise has been shown to unmask the electrocardiographic (ECG) findings in this disease; however, this is yet to be validated as a useful testing method. This is a case of exercise-induced typical Brugada syndrome ECG changes with no occurrence of arrhythmias during exercise and no ECG evidence prior to testing.

Hyperkalemia and cocaine induced dynamic Brugada-type electrocardiogram

Brugada syndrome is a well-defined clinical entity with the typical electrocardiographic changes in the right precordial leads (V1 and V2), attributed to mutation in SCN5A gene. Brugada-like electrocardiographic pattern can be replicated by sodium channel-blocking drugs and electrolyte abnormalities. We describe a 46-year-old individual, who presented with hyperkalemia secondary to muscle damage and renal insufficiency, after abusing cocaine. The electrocardiogram showed Brugada sign. The electrocardiogram changes disappeared after sodium bicarbonate administration and normalization of serum potassium. The case highlights the importance of recognizing cocaine and hyperkalemia, as potential triggers of the acquired Brugada sign.

Mechanism of right precordial ST-segment elevation in structural heart disease: Excitation failure by current-to-load mismatch

The Brugada sign has been associated with mutations in SCN5A and with right ventricular structural abnormalities. Their role in the Brugada sign and the associated ventricular arrhythmias is unknown. The purpose of this study was to delineate the role of structural abnormalities and sodium channel dysfunction in the Brugada sign. Activation and repolarization characteristics of the explanted heart of a patient with a loss-of-function mutation in SCN5A (G752R) and dilated cardiomyopathy were determined after induction of right-sided ST-segment elevation by ajmaline. In addition, right ventricular structural discontinuities and sodium channel dysfunction were simulated in a computer model encompassing the heart and thorax. In the explanted heart, disappearance of local activation in unipolar electrograms at the basal right ventricular epicardium was followed by monophasic ST-segment elevation. The local origin of this phenomenon was confirmed by coaxial electrograms. Neither early repolarization nor late activation correlated with ST-segment elevation. At sites of local ST-segment elevation, the subepicardium was interspersed with adipose tissue and contained more fibrous tissue than either the left ventricle or control hearts. In computer simulations entailing right ventricular structural discontinuities, reduction of sodium channel conductance or size of the gaps between introduced barriers resulted in subepicardial excitation failure or delayed activation by current-to-load mismatch and in the Brugada sign on the ECG. Right ventricular excitation failure and activation delay by current-to-load mismatch in the subepicardium can cause the Brugada sign. Therefore, current-to-load mismatch may underlie the ventricular arrhythmias in patients with the Brugada sign.

Drug-induced Brugada syndrome

Brugada syndrome is an inherited cardiac arrhythmia condition characterized by (i) coved ST-elevation and J point elevation of at least 2 mm in at least two of the right precordial ECG leads (V1-V3) and (ii) ventricular arrhythmias, syncope, and sudden death. Patients with Brugada syndrome or suspected mutation carriers can have normal ECG recordings at other times. In these cases, a diagnostic challenge with a sodium channel blocker such as ajmaline, flecainide, or pilsicainide may induce the full-blown type 1 ECG pattern and support the diagnosis. However, recently, many other pharmacological agents not related to class I anti-arrhythmic agents have been reported to induce Brugada ECG patterns including tricyclic antidepressants, fluoxetine, lithium, trifluoperazine, antihistamines, and cocaine. As published reports of the drug-induced Brugada sign have become increasingly prevalent, there is growing interest in the mechanisms responsible for this acquired ECG pattern and its clinical significance. It is possible that drug-induced Brugada syndrome may be due to an individual susceptibility that favours drug-induced ECG abnormalities, possibly as a result of an increase in a latent ion channel dysfunction similar to that in drug-induced long QT syndrome. However, further evidence is needed to confirm this postulation. In this paper, we will review the cases and evidence of drug-induced Brugada syndrome reported in the literature.

Prevalence of Brugada sign and syndrome in patients presenting with arrhythmic symptoms at a Heart Rhythm Clinic in Singapore

Southeast Asia is endemic for Brugada syndrome; however, its prevalence in Singapore is unknown. We aimed to determine the prevalence of Brugada sign [Types 1, 2, and 3 Brugada electrocardiogram (ECG)] and syndrome at an arrhythmia clinic in Singapore. Patients who presented with pre-syncope, syncope, and/or palpitations without a known cause at an arrhythmia clinic between 2005 and 2007 were included in the study. The study criteria were met by 392 patients (age: 49.6 +/- 19.1 years, 55.9% male); 675 patients were excluded because they were referred for the management of known diagnoses. The 12-lead ECGs of these patients were analysed and clinical information was abstracted. Brugada sign was observed in 28 patients, and 12 patients were determined to have Brugada syndrome (age: 36.3 +/- 15.4 years, 100% male), yielding a prevalence of 7.1% [95% confidence interval (CI): 4.8-10.2] and 3.1% (95% CI: 1.6-5.3), respectively. The prevalence rates of patients with Type 1, Type 2, and Type 3 Brugada signs were 4.8% (95% CI: 2.9-7.5), 1.8% (95% CI: 0.7-3.6), and 0.5% (95% CI: 0.06-1.8), respectively. Over 30.4 person-years of follow-up, there was one inappropriate defibrillation shock but no appropriate shocks or sudden cardiac deaths among patients with Brugada sign or syndrome. The prevalence of both Brugada sign and syndrome in a hospital-based population in Singapore was found to be high. Further population-based investigation is warranted to confirm these findings.

Prevalence of Brugada sign in a Greek tertiary hospital population

The purpose of the present study was to determine for the first time the prevalence of Brugada-type electrocardiographic (ECG) pattern (Brugada sign) in unselected individuals served by an urban Greek tertiary hospital during a 4-year time period. Among 11,488 individuals (6640 males, 4848 females), 25 (23 males, 2 females, aged 36.8 +/- 19.2 years) were found to display the Brugada sign (0.22%). Two cases exhibited the diagnostic type 1 ECG pattern (0.02%) and 23 subjects fulfilled the ECG criteria for type 2 or 3 patterns (0.2%). The incidence of Brugada sign was higher among men (0.34%) than in women (0.04%). Structural heart disease was established in four cases (one of them exhibiting a type 1 ECG pattern). Twenty-one individuals (19 males, 2 females, aged 29.7 +/- 10.7 years) without structural heart disease displaying Brugada-type ECG features (4 cases with spontaneous or procainamide-induced type 1 ECG pattern) were subsequently selected and closely followed up for 24 +/- 12 months. No mortality or life-threatening ventricular arrhythmias were recorded during this period. The Brugada-type ECG pattern is infrequently seen in a Greek hospital-based population. All subjects with Brugada sign and structurally normal hearts displayed a benign clinical course without arrhythmic events during a relatively long follow-up period.

Steep restitution of ventricular action potential duration and conduction slowing in human Brugada syndrome

Brugada syndrome is an increasingly recognized cause of sudden cardiac arrest, defined by characteristic electrocardiogram (ECG) findings and features suggesting a direct or family history of ventricular tachyarrhythmias.1 However, the mechanisms for arrhythmogenesis remain controversial. Animal studies suggest that the ECG “Brugada sign” reflects loss of action potential dome in the epicardium but not in the endocardium, enabling extra beats (from phase 2 reentry) to interact with transmural repolarization dispersion to initiate reentry.

Prevalence of Brugada sign in patients presenting with palpitation in southern Iran

Brugada syndrome is a cardiac channel abnormality that is associated with a high risk of ventricular fibrillation and sudden cardiac death and characterized by an electrocardiographic pattern of right bundle branch block and transient or persistent ST-segment elevation in leads V1-V3. No data regarding the frequency of Brugada syndrome exist in an Iranian population. The aim of this study was to determine the frequency of Brugada-type ECG pattern in southern Iran. All patients presenting with palpitation were enrolled in the study. A Brugada-type ECG pattern was determined according to the criteria recommended by European Heart Association Molecular Basis of Arrhythmias Study Group. A total of 3895 patients (mean age 38.2 +/- 11.9 years, 54% women) met all study criteria. One hundred patients (2.56%) had Brugada-type ECG pattern. Of these, 21 patients (0.54%) had definite Brugada sign (Type 1 or Types 2 and 3 with conversion to Type 1 following procainamide test). Of 21 patients with definite Brugada sign, eight had Brugada syndrome, four had history of syncope, two had coved-type ECG in the family, one had polymorphic ventricular tachycardia, and one had history of sudden cardiac death in the family. Five patients underwent ICD implantation. The incidence of a Brugada-type ECG pattern was 2.43% in subjects between 17 and 30 years and 0.13% in subjects >30 years (P = 0.01). Frequency of Brugada sign in an Iranian population presenting with palpitation is greater than some European countries and lower than a Japanese urban population.

The hyperkalemic Brugada sign

Background A few case reports have indicated that hyperkalemia can induce a Brugada pattern in the electrocardiogram. The specific clinical and electrocardiographic features of the hyperkalemic Brugada sign, however, have not been previously described. Methods A case series was collected from hospitalized hyperkalemic patients with a type I Brugada pattern in the electrocardiogram, and a literature review was performed. Electrocardiograms were examined for rhythm and morphology, and clinical characteristics were analyzed. Results Nine new cases with the hyperkalemic Brugada sign were identified with an additional 15 cases found in the literature. Of the 9 cases, 8 were male patients, and all were critically ill; 5 of the 9 died within 48 hours. The mean (±SD) serum potassium level was 7.8 ± 0.5 mEq/L. The mean QRS width was 144 ± 31 milliseconds, and all had abnormal QRS axis. In 6 cases, there was a wide complex rhythm without visible P waves. The clinical and electrocardiographic characteristics of 15 cases found in the literature were remarkably similar to those in our series. Conclusions The hyperkalemic Brugada pattern differs in substantial ways from the electrocardiogram of patients with the genetic Brugada syndrome. Many patients have wide complex rhythms without visible P waves, marked QRS widening, and an abnormal QRS axis. Most patients are male, and many are critically ill. Prompt recognition of this clinical and electrocardiographic entity may expedite the initiation of appropriate treatment for hyperkalemia.

Brugada electrocardiographic pattern due to tricyclic antidepressant overdose

The Brugada syndrome is an arrhythmogenic disease with characteristic coved ST-segment elevation 2 mm or greater in the right precordial leads (type 1 Brugada electrocardiogram [ECG] pattern or “Brugada sign”] and is estimated to be responsible for at least 20% of sudden deaths in patients with structurally normal hearts [ Circulation 2005;111(5):659-70]. The Brugada sign has been described in asymptomatic patients after exposure to various drugs. As published reports of the drug-induced Brugada sign have become increasingly prevalent, there is growing interest in the mechanisms responsible for this acquired ECG pattern and its clinical significance. We report a case of a patient who developed the type 1 Brugada ECG pattern after intentional overdose of a tricyclic antidepressant agent, review the literature concerning tricyclic antidepressant agent–induced Brugada sign, discuss potential mechanisms, and evaluate the clinical significance of this ECG abnormality.

Frequency of Brugada-Type ECG Pattern (Brugada Sign) in Southern Turkey

The frequency of Brugada sign was found to differ among ethnic groups. Yet, there is no data regarding the prevalence of Brugada syndrome and sign in our country. The aim of this study was to determine the frequency of a Brugada-type electrocardiogram (ECG) pattern in southern Turkey. A total of 1,238 subjects (males, 671, females, 567) were included in the study. The previously archived ECGs of 807 subjects without any evidence of structural heart disease were chosen randomly and evaluated. In addition, prospective analysis of the ECGs of 431 subjects (males, 293, females, 138) randomly chosen from healthy university students were also included. The mean age was 38.9 +/- 17.6 years. Six subjects (0.48%) had a Brugada-type ECG pattern. One (0.08%) of them had the coved-type and 5 (0.40%) had the saddleback-type. All subjects were asymptomatic. A Brugada-type ECG pattern was obtained in 1 (0.17%) female and in 5 (0.74%) males (OR: 4.2 CI: 0.5-36.4, P = 0.2). The Brugada-type ECG pattern frequency was 0.12% in subjects >or= 25 years old and 1.16% in subjects between 17-24 years old (OR: 9.4 CI: 1.1-81.2, P = 0.02). Young males between 17-24 years had the highest (1.70%) frequency. The results indicate that the frequency of the Brugada-type ECG pattern was 0.48% in the general population, being more prevalent in young males in our region. These results are similar to the findings of studies performed in other countries.