Patients with Brugada syndrome are at risk of life-threatening ventricular arrhythmias. Epicardial substrate ablation for Brugada syndrome has been described as a means of controlling these arrhythmias and recent reports describe elimination of the Brugada phenotype with ablation. We describe a unique case in which a patient developed inferior J waves with an early repolarization-type electroca...

BACKGROUND Brugada syndrome and congenital long-QT syndrome (LQTS) type 3 (LQT3) are 2 inherited conditions of abnormal cardiac excitability characterized clinically by an increased risk of ventricular tachyarrhythmias. SCN5A gene that encodes the cardiac sodium channel α subunit is responsible for the 2 diseases, and more work is needed to improve correlations between SCN5A genotypes and assoc...

OBJECTIVES We carried out a complete screening of the SCN5A gene in 38 Japanese patients with Brugada syndrome to investigate the genotype-phenotype relationship. BACKGROUND The gene SCN5A encodes the pore-forming alpha-subunit of voltage-gated cardiac sodium (Na) channel, which plays an important role in heart excitation/contraction. Mutations of SCN5A have been identified in 15% of patients...

The vast majority of sudden cardiac arrests occur in patients with structural heart disease and in approximately 10% of the cases, it can occur in those with structurally normal hearts. Brugada syndrome is an autosomal dominant sodium channelopathy that has been implicated in sudden deaths. Given their low prevalence, our knowledge about Brugada syndrome is still evolving. Apart from schizophre...

(I-MIBG) Imaging in Brugada Syndrome To the Editor: We read with interest a recent article in Circulation by Wichter et al1 on cardiac autonomic dysfunction in Brugada syndrome. The authors demonstrated that regionally reduced iodine-123-metaiodobenzylguanidine (I-MIBG) uptake in the inferior and septal left ventricular wall was present in 8 (47%) of 17 patients with Brugada syndrome but in non...

S ince Brugada syndrome was identified in 1992, its therapeutic management of patients has presented a great challenge for clinicians. In this issue of Circulation Arrhythmia and Electrophysiology, Belhassen et al 2 suggest both risk strati-fication of arrhythmia and therapeutic improvements using electrophysiological study (EPS) and quinidine. Although gradual progress in risk stratification a...

The prevalence of the Brugada-type ECG and its natural history are still unclear. The Brugada syndrome is usually identified by a characteristic Brugada-type ECG that consists of ST elevation of a coved type in the precordial leads V1 to V3 and ventricular fibrillation that can lead to sudden cardiac death, although affected individuals may have a normal ECG. Mutations in the cardiac sodium cha...

In the 2001 edition of the textbook Heart Disease (1) Brugada syndrome is defined as a “distinct form of idiopathic ventricular fibrillation (VF) in which patients have right bundle branch block (RBBB) and ST elevation in the anterior precordial leads without evidence of structural heart disease.” Although patients with such characteristics were described before the Brugada brothers reported th...

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 b...

Two leading groups of investigators are reporting differing conclusions regarding the value of electrophysiologic studies (EPS) in the Brugada syndrome. First, Brugada et al.1 reported that EPS has excellent accuracy for predicting outcome in the Brugada syndrome. Soon thereafter, however, Priori et al.2 concluded quite the opposite. Based on a study of 86 patients with Brugada syndrome who und...