November 2002 PACE, Vol. 25, No. 11 In 1992 two Spanish cardiologist brothers, Pedro and Josep Brugada reported eight patients with aborted cardiac arrest and no demonstrable heart disease who exhibited in sinus rhythm right bundle branch block (RBBB) with prominent STsegment elevation in precordial leads V1–V3. Despite initial controversy about the diagnosis, especially concerning the possibil...

Brugada syndrome has been linked to mutations in SCN5A. Agents that dissociate slowly from the sodium channel such as flecainide and ajmaline unmask the Brugada syndrome electrocardiogram and precipitate ventricular tachycardia/fibrillation. Lidocaine, an agent with rapid dissociation kinetics, has previously been shown to exert no effect in patients with Brugada syndrome. We characterized a no...

Brugada syndrome is a heritable cause of fatal ventricular arrhythmias in people with otherwise structurally normal hearts. It is diagnosed by the identification of a characteristic ECG pattern in association with certain risk factors [1]. Some risk factors are clinical events likely to be precipitated by ventricular arrhythmias, such as syncope and agonal respirations during sleep. Such patien...

Some mutations of the sodium channel gene Na(V1.5) are multifunctional, causing combinations of LQTS, Brugada syndrome and progressive cardiac conduction system disease (PCCD). The combination of Brugada syndrome and PCCD is uncommon, although they both result from a reduction in the sodium current. We hypothesize that slow conduction is sufficient to cause S-T segment elevation and undertook a...

Loss-of-function mutations in the cardiac sodium channel α-subunit gene SCN5A result in multiple inherited arrhythmic syndromes. This case report describes 2 unrelated probands carrying an identical SCN5A frameshift mutation, V1764fsX1786, who exhibited distinct clinical manifestations: progressive cardiac conduction defect (PCCD)/Brugada syndrome (patient #1) and idiopathic ventricular fibrill...

Objectives-The Brugada syndrome is a heterogeneous genetic disease that predisposes one to lifethreatening ventricular tachyarrhythmia and sudden cardiac death (SCD). In this study, we sought to compare the efficacy of intravenous Procainamide versus oral Flecainide to unmask the typical electrocardiographic changes of this syndrome. Methods-From October 2001 to December 2010, we evaluated pati...

INTRODUCTION Brugada syndrome is a hereditary arrhythmia characterized by a specific electrocardiographic pattern and an increased risk of sudden cardiac death, with an apparent absence of structural abnormalities or ischemic heart disease. To date, mutations in the sodium channel, voltage-gated, type V, alpha subunit gene and glycerol-3-phosphate dehydrogenase 1-like gene are estimated to acco...

BACKGROUND Brugada syndrome is an inherited arrhythmogenic disease that may cause sudden cardiac death due to ventricular fibrillation in young adults. Brugada syndrome caused by propafenone intoxication has been noted rarely in the literature. We report a rare case, Brugada phenocopy due to propafenone intoxication and its treatment. CASE REPORT A 15-year-old girl having a seizure was brough...

BACKGROUND The characteristic ECG pattern of ST-segment elevation in V1 and V2 in the Brugada syndrome is dynamic; it is often intermittently present in affected individuals and can be unmasked by sodium channel blockers, including antiarrhythmic drugs and tricyclic antidepressants. We report here 2 patients who developed the Brugada ECG pattern after administration of lithium, a commonly used ...