Brugada syndrome,Brugada phenocopy or none?

Brugada syndrome is a form of inherited arrhythmia syndrome characterized by a distinct ST‐segment elevation in the right precordial leads. Brugada phenocopies are clinical entities that present with an electrocardiographic pattern identical to Brugada syndrome and may obey to various clinical conditions. We present a case of a suicidal attempt using a high dose of propafenone causing a Brugada‐type electrocardiographic pattern. Is this a Brugada syndrome case, a Brugada phenocopy or something else?     

Electrical storm in Brugada syndrome successfully treated using isoprenaline.

A case of an electrical storm occurring in a patient implanted with a cardioverter-defibrillator for Brugada syndrome is reported. Recurrent ventricular fibrillation was initiated by short-coupled isolated monomorphic ventricular premature beats probably originating from the right ventricular outflow tract, associated with a manifest electrocardiographic pattern of Brugada syndrome. Infusion of atropine accelerated the heart rate but did not prevent ventricular fibrillation, however, low doses of isoprenaline quickly obviated any recurrence of ventricular fibrillation. This was associated with the disappearance of the short-coupled premature beats together with a normalization of the electrocardiographic pattern. Possible mechanisms are discussed according to the accepted pathophysiological hypothesis.     

Brugada syndrome

The Brugada syndrome is an autosomal dominant disease with incomplete penetrance that may cause syncope and sudden cardiac death in young individuals with a normal heart. It is characterized by an electrocardiographic pattern of complete or incomplete right bundle branch block and ST segment elevation in leads V1-V3. One of the genes linked to this syndrome is SCN5A, the gene encoding for the cardiac sodium channel. Mutations in SCN5A cause a functional reduction in the availability of cardiac sodium current in Brugada syndrome. However, only 20-25% of patients affected by this syndrome have mutations on this gene. A novel gene locus on chromosome 3, distinct from SCN5A, has been identified recently. The relative male preponderance of the phenotype, despite equal inheritance of the gene in males and females, has led to the speculation of a role for testosterone in the phenotype. The disease could manifest at first time as cardiac arrest without any previous symptom, and the electrocardiographic pattern could be intermittent, requiring a pharmacological challenge with Class I antiarrhythmic drugs to unmask ST elevation. Several conditions producing Brugada-like electrocardiographic patterns should be borne in mind and excluded while making a diagnosis of the Brugada syndrome. The management is difficult as pharmacological agents are not universally effective. The mode of treatment recommended by the majority of cardiac electrophysiologists is the implantation of a cardioverter defibrillator. Symptomatic patients with inducible ventricular arrhythmias and a positive family history should be considered for prophylactic implantation of a cardioverter defibrillator.     

Brugada综合征和特发性J波的临床分析

目的：了解Brugada综合征和特发性J波的临床和心电图特点及其异同点,方法：对收集的10例Brugada综合征和8例特发性J波患者临床和心电图资料进行对比并结合文献。结果：两病有 相似的临床和心电图表现,但心电图表现仍有一定区别,Brugada综合征R’波大于R波,多见于V1－V3导联,伴ST段持续抬高,T波倒置,图形较恒定,特发性J波中J波小于R波,在Ⅱ,Ⅲ,aVF和V4－V6导联多见,无T波倒置,J波可因药物,运动等原因在短时间内明显改变。结论：Brugada综合征和特发性J波临床表现相似,可能有相同的发病机制,只是影响的部位不同,其区别主要在心电图表现。     

Spectrum of cardiovascular lesions in mucocutaneous lymph node syndrome: Analysis of eight cases

Eight cases of mucocutaneous lymph node syndrome with cardiovascular complications are described. The clinical presentation in all but one patient conformed to the previously described features of this syndrome. Five patients had cardiac enlargement, six had electrocardiographic abnormalities and three had a murmur of mitral insufficiency. Each of the four patients who died had aneurysms of the coronary arteries, usually associated with organizing thrombi. In addition to involvement of the coronary arteries, the renal, mesenteric, vertebral, splenic, hepatic and iliac arteries were affected in individual cases. One patient required triple aortocoronary bypass surgery and one patient underwent excision of a brachial arterial aneurysm. Further long-term follow-up studies of the patients who have survived the acute stage of the syndrome are indicated to establish the spectrum of late complications and sequelae.     

Early repolarization syndrome and Brugada syndrome: is there any linkage?

Early repolarization syndrome (ERS) is characterized by the presence, in most cases in mid-to-lateral precordial leads, of a J wave on the downsloping portion of the QRS complex, followed by an elevation of the ST-segment with upward concavity. ERS is considered a benign electrocardiographic pattern of ventricular repolarization and, thus far, clinical interest in this syndrome has been confined to its differential diagnosis from myocardial infarction and pericarditis. Brugada syndrome (BS), an inherited cardiac disease first described in 1992, exhibits a characteristic electrocardiographic pattern consisting of a J wave mimicking a right bundle branch block with typical ST-segment elevation in the right precordial leads. Believed to be a normal repolarization variant for more than three decades, the syndrome is now known instead to be associated with a high incidence of life-threatening ventricular tachyarrhythmias and is responsible for a number of sudden deaths in young adults worldwide. Although clinical findings seem to differentiate the two syndromes, similarities between BS and ERS in terms of response to heart rate, pharmacologic agents, and neuromodulation could suggest a linkage in their pathophysiological mechanism. The authors review the clinical and experimental data in order to test this hypothesis.     

Anterior myocardial infarction patterns in the mitral valve prolapse-systolic click syndrome

Two young women with the mitral valve prolapse-systolic click (MVP-SC) syndrome had electrocardiographic and vectorcardiographic evidence of transmural anterior myocardial infarction in the absence of clinical and arteriographic evidence of coronary disease.     

Congenital long QT syndrome: A case report

The congenital long QT syndrome (LQTS) is characterized by abnormally prolonged ventricular repolarization due to inherited defects in cardiac sodium and potassium channels, which predispose the patients to syncope, seizure like activity, ventricular arrhythmias, and sudden cardiac death. Early diagnosis and preventive treatment are instrumental in preventing sudden cardiac deaths in patients with the congenital LQTS. The diagnostic criteria for congenital LQTS are based on certain electrocardiographic findings, clinical findings and findings of epinephrine stress test. Recently genotype specific electrocardiographic pattern in the congenital LQTS has also been described. Recent studies suggest feasibility of genotype specific treatment of LQTS and, in near future, mutation specific treatment will probably become a novel approach to this potentially fatal syndrome. We describe one case that fulfilled the electrocardiographic, historical diagnostic criteria and epinephrine stress test suggestive of LQT syndrome.     

Sudden death during ambulatory monitoring. Analysis of six cases

The ambulatory electrocardiographic recordings of six patients with coronary artery disease who died during monitoring were analyzed. In four patients, sinus rhythm was interrupted by sinoatrial, atrioventricular, nodal, or infra-His conduction abnormalities leading to bradyarrhythmic sudden death. Two patients died of sustained ventricular tachycardia or ventricular fibrillation. These data emphasize that the arrhythmias involved in the sudden death syndrome may be more heterogenous than currently appreciated.     

Multiple changes of the morphology of ST segment in a patient with Brugada syndrome

The Brugada syndrome is characterized by in a electrocardiographic pattern of right bundle branch block and ST-segment elevation in the right precordial leads, absence of any structural heart disease and syncope episodes or sudden death. We report the case of a 50 year-old men with Brugada syndrome and manifold changes of the precordial morphology of ST segment.     

Status epilepticus as an initial presentation of Brugada syndrome: a case report

Patients presenting with convulsions are sometimes incorrectly treated for epilepsy, as these symptoms may be manifestations of underlying cardiac disease. Brugada syndrome, associated with an elevated risk of developing fatal arrhythmic events, is a rare disorder characterized by a unique electrocardiographic pattern. The typical clinical presentation mainly involves syncope of unclear cause or sudden death. Seizures are uncommon clinical manifestations of Brugada syndrome, and reports of status epilepticus as initial presentation of Brugada syndrome are extremely rare. In this case report, we present a male patient with a typical pattern on 12-lead electrocardiography, following resuscitation for generalized convulsive status epilepticus.     

Transient ascending ST‐segment depression and widening of the S wave in 3‐channel Holter monitoring—A sign of dromotropic disturbance in the right ventricular outflow tract in the Brugada syndrome: A report of five cases

BackgroundBrugada syndrome (BrS) is somewhat a challenging diagnosis, due to its dynamic pattern. One of the aspects of this disease is a significant conduction disorder located in the right ventricular outflow tract (RVOT), which can be explained as a consequence of low expression of Connexin‐43. This decreased conduction speed is responsible for the typical electrocardiographic pattern. Opposite leads located preferably in inferior leads of the electrocardiogram may show a deep and widened S wave associated with ascending ST segment depression. Holter monitoring electrocardiographic (ECG) aspects is still a new frontier of knowledge in BrS, especially in intermittent clinical presentations.MethodsWe describe, as an exploratory analysis, five case series of intermittent type 1 BrS to demonstrate the appearance of ascending ST segment depression and widening of the S wave, during 3‐channel 24h‐Holter monitoring (C1, C2 and C3) with bipolar leads.ResultsIn the five cases described, the ST segment depression was observed mainly in C2, but in some cases also in C1 and C3. Only case 1 presented concomitant intermittent elevation of the ST segment in C1. All cases were intermittent.ConclusionThe recognition of an ECG pattern with ascending ST‐segment depression and widening of the S wave in 3‐channel Holter described in this case series should raise a suspicion of the BrS and suggests the counterpart of a dromotropic disturbance registered in the RVOT and/or reciprocal changes.     

Brugada-like electrocardiographic pattern unmasked by fever

Brugada syndrome is characterized by right bundle branch block morphology and ST-segment elevation in the right precordial leads and a propensity to develop ventricular arrhythmias. Mutations in a cardiac sodium channel gene have been linked to this syndrome, and the ionic mechanisms responsible for the electrocardiographic phenotype are temperature-dependent. This case report describes a patient in whom a typical Brugada ECG pattern developed during fever and could be reproduced at normal body temperature by administration of pilsicainide.     

Detailed analysis of 24 hour ambulatory electrocardiographic recordings during ventricular fibrillation or torsade de pointes

Although the terminal cardiac rhythm is often well documented in many cases of sudden cardiac death, the antecedent or premonitory arrhythmias are usually not retrievable. The ambulatory electrocardiographic recordings of 12 patients who sustained ventricular fibrillation or torsade de pointes while wearing a long-term electrocardiographic monitor were analyzed in detail. A printout of the entire electrocardiographic recording was made and hand counts of ventricular arrhythmias were correlated with heart rate, QTc interval, RR interval preceding ventricular fibrillation or torsade de pointes and (RR')/QT initiating ventricular fibrillation or torsade de pointes. Common ambulatory electrocardiographic features in these 12 patients experiencing ventricular fibrillation or torsade de pointes included: 1) a period of high density of increasingly frequent or complex ventricular arrhythmias, or both, preceding ventricular fibrillation or torsade de pointes (11 patients); 2) R on T beats frequently initiating ventricular fibrillation or torsade de pointes (9 patients); and 3) repolarization abnormalities present for several hours before ventricular fibrillation or torsade de pointes (7 patients). No consistent relation between the RR and RR' interval initiating ventricular fibrillation or torsade de pointes was found; no consistent alteration in heart rate occurred before ventricular fibrillation or torsade de pointes. Thus, ventricular arrhythmias leading to sudden death in an ambulatory population do not occur in isolation but are preceded by a period of increased ventricular ectopic activity. Future guidelines for assessment of antiarrhythmic drug efficacy should include an evaluation of a drug's impact not only on ectopic beat frequency but also on arrhythmia density.     

Variable morphologies of preexcitation due to a concomitant Brugada pattern

Since the first publication of the Brugada syndrome in 1992 several variants of this ECG pattern have been described. We report a very unusual case of preexcitation with changing electrocardiographic morphologies which appeared to be an association of a variable Brugada pattern with a persistent antegrade preexcitation.     

Brugada-like electrocardiographic pattern induced by an episode of anemia

The mechanisms responsible for the undulating pattern of ST-segment elevations in the Brugada syndrome are still a matter of discussion. This report describes a young man with a Brugada-like electrocardiographic pattern. The specific ST-segment elevations were unmasked during an episode of anemia due to a duodenal ulcer.     

Acute occlusion of the left main trunk presenting as ST-elevation acute coronary syndrome

Acute obstruction of the left main coronary artery (LMCA) is not frequently encountered. Electrocardiographic findings are important to early diagnosis in determining an acute obstruction of the LMCA, which requires immediate aggressive treatment, in this extremely unstable condition. However, there is no single typical electrocardiographic pattern representing acute occlusion of the LMCA. We describe a rare electrocardiographic finding that suggested ST-elevation acute coronary syndrome of the anterior zone due to left main trunk total occlusion.     

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.     

Persistent Abnormal Value of Late Potential in Brugada Syndrome Associated with Hypokalemia

Hypokalemia accentuates the electrocardiographic (ECG) pattern of Brugada syndrome. We report two patients with Brugada syndrome and hypokalemia‐induced lethal events. Despite concealing the typical ECG pattern with normalization of serum potassium levels, late potentials were persistently detected by signal‐averaged ECG, even at the 18‐month follow‐up. An implantable cardioverter defibrillator was inserted to prevent sudden cardiac death. Ann Noninvasive Electrocardiol 2011;16(1):104–106