Brugada syndrome with ventricular tachycardia and fibrillation related to hypokalemia.

A 60-year-old man with asymptomatic Brugada syndrome and neither a history of syncope nor family history of sudden death was admitted because of bronchial asthma. Serum potassium concentration was 3.8 mmol/L on admission, and decreased to 3.1 mmol/L on the 6th day, probably as a side effect of steroid therapy. The patient was found unconscious on the 7th day, and his serum potassium concentration was 3.4 mmol/L immediately after the episode. On the 8th day, the patient was again found unconscious, and polymorphic ventricular tachycardia and fibrillation (VT/VF) was documented on electrocardiographic (ECG) monitoring. The coved type of ST-segment elevation in leads V(1-3) was observed on the ECG after spontaneous recovery of sinus rhythm, and VT/VF associated with Brugada syndrome was diagnosed. The serum potassium concentration decreased to 2.9 mmol/L immediately after the episode, but QT prolongation was not observed during the clinical course. After the correcting the serum potassium concentration, there was no further recurrence of the malignant ventricular arrhythmia and syncope. An implantable cardioverter defibrillator was inserted to prevent sudden death. Hypokalemia that does not induce QT prolongation may contribute to the occurrence of VT/VF in Brugada syndrome.     

Manifestation of Brugada syndrome after pacemaker implantation in a patient with sick sinus syndrome

A 49-year-old woman experienced syncope 10 months after DDD pacemaker implantation for sick sinus syndrome. ECG revealed abnormal ST elevation in leads V1 to V3 during a paced rhythm. Multifocal premature ventricular contractions followed by ventricular fibrillation were documented. Saddleback-type ST elevation was confirmed after a mode change to AAI. The diagnosis of Brugada syndrome was made, and the DDD pacemaker was upgraded to an implantable cardioverter defibrillator. Brugada syndrome can be easily overlooked if the classic ECG findings are not initially noted but may be observed even during pacing therapy.     

Electrocardiographic pattern of Brugada syndrome disclosed by a febrile illness: clinical and therapeutic implications.

BACKGROUND: Recent studies have identified a direct link between the ionic mechanisms responsible for the electrocardiographic (ECG) pattern of the Brugada syndrome (BS) and the in vitro experimental temperature, pointing to the possibility that some BS patients may display the ECG phenotype only during a febrile state, being in this setting at risk of lethal arrhythmias. CASE REPORT: A 53-year-old man referred to the emergency room for abdominal pain and fever. The ECG showed dome-shaped ST-segment elevation in V1-V3, as in the typical BS. The personal and family history were unremarkable for syncope and sudden death and physical, laboratory and ultrasound examinations were negative. On day 3, at normal body temperature, the patient's ECG returned to normal and the ECG abnormalities were later reproduced with intravenous flecainide. The patient refused the implantation of a loop recorder and was discharged after 6 days. He has remained asymptomatic during 2 years of follow-up. CONCLUSIONS: The typical ECG phenotype of BS disclosed by a febrile illness confirms the in vitro experimental data that previously established a correlation between ECG pattern of BS and temperature variations. The clinical and therapeutic implications of these findings are discussed.     

Brugada syndrome associated with ventricular fibrillation induced by administration of pilsicainide: a case report

A 56-year-old man with Brugada syndrome presented with ventricular fibrillation induced by administration of pilsicainide. He had syncope at age 46 years, and his uncle suddenly died of unknown cause. He had taken pilsicainide (150 mg/day) for paroxysmal atrial fibrillation, and suffered from syncope due to ventricular fibrillation. Coved type ST elevation was observed in the V1 lead, and saddle back type ST elevation was observed in the V2 lead. The ST elevation gradually recovered after stopping pilsicainide therapy. No structural heart disease was found. After intravenous injection of pilsicainide, the ST segment elevated in the V1 and V2 leads. Ventricular fibrillation was induced by triple extrastimulation at the right ventricular apex. The diagnosis was Brugada syndrome, and a cardioverter-defibrillator was implanted. Brugada syndrome should be considered before administering pilsicainide.     

Electrocardiographic findings typical of Brugada syndrome unmasked by cocaine consumption

Brugada syndrome is characterized by the presence of right bundle branch block on electrocardiography and by ST-segment elevation in the right precordial leads (V1-V3), by the absence of structural cardiac abnormalities, and by episodes of syncope or sudden death. On occasion, diagnosis is made difficult by temporary normalization of the ECG. The condition can be unmasked by potent sodium channel blockers, such as flecainide. Our patient presented with a Brugada syndrome-type ECG after intake of a large amount of cocaine.     

ST segment elevation in the right precordial leads induced with class IC antiarrhythmic drugs: insight into the mechanism of Brugada syndrome

We evaluated two patients without previous episodes of syncope who showed characteristic ECG changes similar to Brugada syndrome following administration of Class IC drugs, flecainide and pilsicainide, but not following Class IA drugs. Patient 1 had frequent episodes of paroxysmal atrial fibrillation resistant to Class IA drugs. After treatment with flecainide, the ECG showed a marked ST elevation in leads V2 and V3, and the coved-type configuration of ST segment in lead V2. A signal-averaged ECG showed late potentials that became more prominent after flecainide. Pilsicainide, a Class IC drug, induced the same ST segment elevation as flecainide, but procainamide did not. Patient 2 also had frequent episodes of paroxysmal atrial fibrillation. Pilsicainide changed atrial fibrillation to atrial flutter with 2:1 ventricular response, and the ECG showed right bundle branch block and a marked coved-type ST elevation in leads V1 and V2. After termination of atrial flutter, ST segment elevation in leads V1 and V2 continued. In this patient, procainamide and quinidine did not induce this type of ECG change. In conclusion, strong Na channel blocking drugs induce ST segment elevation similar to Brugada syndrome even in patients without any history of syncope or ventricular fibrillation.     

Paradoxical effect of ajmaline in a patient with Brugada syndrome.

AIMS: The typical Brugada ECG pattern consists of a prominent J-wave associated with ST-segment elevation localized in the right precordial leads V1-V3. In many patients, the ECG presents periods of transient normalization and the Brugada-phenotype can be unmasked by the administration of class-I antiarrhythmics. Reports have documented the heterogeneity of the Brugada syndrome ECG-phenotype characterized by unusual localization of the ECG abnormalities in the inferior leads. Case report A 51-year-old man, without detectable structural heart disease, was referred to us because of a history of syncope, dizziness, and palpitations. The ECG showed a J-wave and ST-segment elevation in the right precordial leads, suggesting Brugada syndrome. As other causes of the ECG abnormalities were excluded, the patient underwent an electrophysiological study that documented easy induction of ventricular fibrillation. During infusion of ajmaline, new prominent J-waves and ST-segment elevation appeared in the inferior leads, whereas the basal ECG abnormalities in the right precordial leads normalized. After infusion of isoprenaline, the ECG-pattern resumed the typical Brugada pattern. An implantable cardioverter-defibrillator was recommended. CONCLUSION: In our patient, the double localization of the typical Brugada-pattern and the paradoxical effect of ajmaline on the ECG abnormalities confirmed the possibility of a phenotype heterogeneity in the Brugada syndrome.     

Prolonged QRS duration in lead V2 and risk of life-threatening ventricular Arrhythmia in patients with Brugada syndrome

Brugada syndrome is an inherited disorder that predisposes some patients to sudden cardiac death. It is not well established which Brugada syndrome patients are at risk of life-threatening arrhythmias. We investigated whether standard 12-lead electrocardiograms (ECG) can identify such patients. The subjects were 35 men with Brugada syndrome (mean age, 50.1 ± 12.4 years). Documented ventricular fibrillation or aborted sudden cardiac arrests were judged to be related to the Brugada syndrome. Ten patients (mean age, 49.6 ± 14.9 years) were symptomatic, and 25 (mean age, 50.3 ± 11.5 years) were asymptomatic. We determined the PR interval, QRS duration, and QT interval from baseline 12-lead ECG leads II and V2 as well as the J point elevation amplitude of lead V2. The QRS interval was measured from QRS onset to the J point in leads II and V2. The only significant difference between the symptomatic and asymptomatic patients was the QRS duration measured from lead V2. The mean QRS interval was 129.0 ± 23.9 ms in symptomatic patients versus 108.3 ± 15.9 ms in asymptomatic patients (P = 0.012). A QRS interval in lead V2 ≥ 120 ms was found to be a possible predictor of a life-threatening ventricular arrhythmia and/or syncope (P = 0.012). Prolonged QRS duration as measured on a standard 12-lead ECG is associated with ventricular arrhythmia and could serve as a simple noninvasive marker of vulnerability to life-threatening cardiac events in patients with Brugada syndrome.     

Síncope e padrão de Brugada intermitente

Brugada syndrome is a rare syndrome, with an estimated prevalence in Europe of 1-5/10 000 population, whose initial clinical presentation can be sudden death. Although it has a characteristic electrocardiographic pattern, this can be intermittent. The authors present the case of a 32-year-old man, with no family history of syncope or sudden death, who went to the emergency department for syncope without prodromes. The initial electrocardiogram (ECG) in sinus rhythm documented an isolated and non-specific ST-segment elevation in V2. During further diagnostic studies, a repeat ECG revealed type 1 Brugada pattern. This pattern was later seen in a more marked form during a respiratory infection. The patient subsequently underwent electrophysiological study, followed by implantation of an implantable cardioverter-defibrillator (ICD), with an episode of ventricular fibrillation converted via ICD shock two months after implantation.     

Long-term follow-up in patients with Brugada Syndrome in South China

ObjectiveTo evaluate the presence of Brugada electrocardiogram (ECG) pattern, clinical characteristics, treatment, and long‐term prognosis of Brugada syndrome in southern Chinese population.MethodsThis prospective study consisted of a consecutive series of patients with diagnostic coved type I Brugada ECG pattern at baseline between January 2007 and February 2020. Histories of symptoms including ventricular tachycardia (VT)/ventricular fibrillation (VF) episode, syncope, and family history of Brugada Syndrome (BrS) or unexplained sudden cardiac death were collected. Electrophysiological study and implantable cardioverter‐defibrillator (ICD) were performed. All patients included in this study were followed up in the outpatient department every 6 months after baseline evaluation. Occurrences of syncope, VF, and sudden death were independently analyzed by two cardiologists.Results45 (56.3%) patients were diagnosed with BrS. During a mean follow‐up of 7.9 ± 3.6 years, six patients had experienced documented VF/sudden cardiac death (SCD) or recurrent syncope. Two patients experienced episodes of syncope more than once. Two patients experienced onset of electrical storm with a total of 11 episodes of VF. There were 50% of these events occurring in fever status. One of patient with BrS died of SCD.ConclusionThere was a very low prevalence of Brugada syndrome in southern Chinese population. The risk of arrhythmic events was low in asymptomatic patients. ICD was high effective in preventing SCD without adverse device outcome in long‐term follow‐up. Fever can lead to predispose to malignant arrhythmia, and aggressive treatment of febrile state in Brugada syndrome was recommended.     

The Brugada syndrome--a clinical case]

A case report of a patient with syncope and family history of sudden death is presented. The precordial recordings in the standard 12-lead ECG showed a right bundle-branch block pattern with persistent ST elevation in V1 and V2-V3. After a thorough evaluation, we found no underlying organic cardiomyopathy. The diagnosis of symptomatic Brugada syndrome was made. A cardioverter-defibrillator was implanted.     

Isolated and asymptomatic Brugada syndrome. A case report

The case report of a 32-year-old man with a Brugada syndrome is presented. He was asymptomatic and without familial history of sudden death or syncope. Diagnosis criteria for Brugada syndrome were 1--a pattern of right bundle branch block and ST-segment elevation in leads V1 and V2 on the ECG, 2--no cardiac structural anomalies. Symptomatic patients with this electrical anomaly are at high risk of sudden death and need an automatic implantable defibrillator. The outcome and the treatment of asymptomatic patients are a matter of debate and are discussed in this report.     

Brugada phenocopy in a patient with unstable angina and three-vessel coronary artery disease

A 52-year-old male was admitted with unstable angina and three-vessel coronary artery disease. Electrocardiography (ECG) changes consistent with type-1 Brugada ECG pattern were noted during admission. The patient was asymptomatic for syncope and had no family history of sudden cardiac death, ICD implantation, and Brugada syndrome. After coronary by-pass graft the Brugada ECG pattern resolved, and ajmaline test did not elicit type-1 ECG pattern, confirming the suspicion of Brugada phenocopy.     

Síncope em contexto febril – caso clínico de síndrome de Brugada

In 1992, Brugada and Brugada first described a new entity, which became known as Brugada syndrome, that is associated with a high risk of ventricular arrhythmias and sudden cardiac death in patients without structural heart disease. This syndrome is characterized by a distinct electrocardiographic phenotype, type 1 Brugada pattern, consisting of a coved ST‐segment elevation (≥0.2 mV) followed by a negative T wave in more than one right precordial lead. This pattern is dynamic, and can be spontaneous or concealed, but is unmasked under certain circumstances, like febrile states.The authors report a case in which the diagnosis of Brugada syndrome was made in the course of etiologic investigation of recurrent syncope in a febrile state.     

Spontaneous T wave alternans in a patient with Brugada syndrome--responses to intravenous administration of class I antiarrhythmic drug, glucose tolerance test, and atrial pacing

Spontaneous T wave alternans in Brugada syndrome. A 43-year-old man with an episode of syncope showed ECG patterns of coved-type ST elevation in leads V1-V3 and right bundle branch block pattern. The patient had spontaneous T wave alternans at baseline, and T wave alternans diminished with distinct development of ST elevation after administration of Na+ channel blocker, and during oral glucose load and atrial pacing. Na+ channel mutation may contribute to the genesis of his ECG changes.     

Syncope in pharmacologically unmasked Brugada syndrome: indication for an implantable defibrillator or an unresolved dilemma?

A 30-year-old Caucasian male was referred for evaluation of a 2-year history of recurrent post-exertion lightheadedness and near syncopal spells in the setting of a family history of unexplained sudden cardiac death. Cardiac evaluation demonstrated normal heart structure, but the 12-lead surface ECG was suggestive of but not diagnostic of Brugada syndrome. An exercise stress test reproduced the patient's usual symptoms during the recovery period, and was consistent with a typical vasovagal faint. The same symptoms were observed during a head-up tilt table test. However, given the family history and ECG, pharmacological testing with procainamide, isoprenaline and metoprolol, as well as programmed ventricular stimulation, were undertaken. Pharmacological provocation further supported a diagnosis of Brugada syndrome, whereas programmed ventricular stimulation was considered non-diagnostic regarding ventricular tachyarrhythmia susceptibility. Consequently, despite ECG and pharmacological findings suggestive of Brugada syndrome, there appeared to be sufficient evidence to believe that this patient's symptoms were the result of neurally mediated syncope and not due to ventricular tachyarrhythmias. The patient was treated with midodrine, and has remained symptom-free for 16 months. Thus, given the frequency with which vasovagal syncope occurs in young patients, its occurrence is not unexpected in individuals with concomitant diagnoses such as Brugada syndrome. In as much as current recommendations favour implantable defibrillators in symptomatic Brugada syndrome, the identification of other causes of syncope in such patients poses an uncomfortable, and currently unsettled dilemma.     

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

AIMS: 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. METHODS AND RESULTS: 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). CONCLUSION: Frequency of Brugada sign in an Iranian population presenting with palpitation is greater than some European countries and lower than a Japanese urban population.     

Brugada syndrome--an under-recognized electrical disease in patients with sudden cardiac death

In 1992, Brugada and Brugada described 8 patients with a history of aborted sudden death and a distinct ECG pattern of right bundle-branch block with ST segment elevation in leads V1-V3 and normal QT interval in the absence of any structural heart disease. It is called Brugada syndrome now and is believed to be responsible for 4-12% of all sudden deaths and around 20% of deaths in patients with structurally normal hearts. Although this syndrome is observed worldwide and the exact prevalence is unknown, it is more common in the Southeast Asian countries. Repeated syncope, ventricular fibrillation, and sudden cardiac death have been reported in patients with Brugada syndrome. The clinical presentation of Brugada syndrome is distinguished by a male predominance and the appearance of arrhythmic events at an average age of 40 years. The Brugada syndrome is inherited in an autosomal dominant manner with incomplete penetrance and an incidence ranging between 5 and 66 per 10,000. The surface ECG manifestations of the syndrome can transiently disappear, but can be unmasked by potent sodium channel blockers in some cases. Mutations of the cardiac sodium channel SCN5A have been detectable in <20% of patients with Brugada syndrome. Recent genetic studies have confirmed the genetic heterogeneity of the disorder. Antiarrhythmic drugs appear to be of little use in prolonging survival and in preventing recurrences of ventricular arrhythmias. To date, implantable cardioverter defibrillator remains the best therapy to prevent sudden death in these patients.     

Sepsis-induzierter elektrischer Sturm als Erstmanifestation eines Brugada-Syndrom

Brugada syndrome (BS) is characterized by an electrocardiographic (ECG) pattern consisting of elevated ST segments in leads V1–V3 and a morphology similar to right bundle branch block. BS is associated with a risk of sudden cardiac death. A genetic defect (e.g., SCN5A) seems to be a requirement for occurrence of this syndrome. Together with factors like electrolyte disturbances, bradycardia or febrile illness, this defect leads to increased ventricular vulnerability resulting in ventricular arrhythmias. A 38-year-old so far healthy male suffered a second to third degree burn over 40% of his body surface. The patient was in an excellent physical condition before the trauma. Burns were treated with multiple split-thickness skin grafts. The patient was ventilated artificially on the ICU of the local casualty hospital. After several days, patient developed septic shock requiring low dose catecholamine infusion. During sepsis with fever up to 40°C even under calculated antibiosis, recidivating polymorphic ventricular tachycardias which degenerated to ventricular fibrillation in terms of an electrical storm had to be treated electrically several times a day. Therapy according to the guidelines of advanced life support was ineffective. Sinus rhythm ECG revealed a Brugada pattern and repolarization abnormalities suspicious for cardiac ischemia. Therefore BS and cardiac ischemia were taken into account as the trigger for the ventricular tachyarrhythmias. By reason of the inconspicuous anamnesis we assessed a low feasibility of a coronary heart disease but acute myocardial ischemia during sepsis could not be fully excluded. However, because of the large area of burned skin, transfer to the cath lab would have been a considerable effort for our patient. Fully aware of fever as a predisposing factor of electrocardiographic and arrhythmic manifestation of a BS, fever treatment was our first therapeutical effort. After effective fever treatment including physical cooling and intravenous paracetamol, Brugada pattern and tachyarrhythmias disappeared within a few hours. No further ventricular arrhythmias were registered during ongoing inflammation as long as body temperature was normal. Four months after these acute events, we invasively excluded coronary heart disease. A supplementary positive ajmaline test ensured the correct diagnosis of BS. No currently described mutation typical for BS was found in the genetic screening. No history of syncope, sudden cardiac death or lifethreatening arrhythmias was reported from blood relatives. According to current guidelines, a cardioverter-defibrillator (ICD) was implanted. At the 12-month follow-up, interrogation of the ICD revealed no further tachyarrhythmias. ECG has completely normalized. Sufficient documentation of ECG and body temperature is a necessity in the ICU, especially in a patient with a clinical picture of sepsis and ventricular tachycardia. Unfortunately, registration of ECGs of distinctively morbid patients showing Brugada pattern is often difficult although the ECG pattern is quite characteristic. If a BS is diagnosed, antiarrhythmic therapy diverges from the usual therapeutical approach, instead of adjustment of serum electrolytes and a medicamentous antiarrhythmic regime, normalization of body temperature and sepsis treatment is urgent and can be life-saving.