The Brugada Syndrome　― From Gene to Therapy ―

Abstract

It is almost a quarter of century that a pioneering work of 2 researchers named Brugada brought the entire scientific community to understanding the molecular, clinical, and electrophysiological aspects of a distinctive syndrome. It affects mainly young adults with syncope and/or sudden cardiac death caused by polymorphic ventricular tachycardia or ventricular fibrillation in the absence of any sign of cardiac degeneration or alteration. Although the involvement of the epicardial layer of the right ventricular outflow tract, and the requirement of pharmacologic challenge for unveiling concealed forms, have been fully characterized, many areas of uncertainties remain to be elucidated, such as the unpredictable usefulness of programmed ventricular stimulation, the role of radiofrequency catheter ablation for reducing ST-segment elevation, and the value of risk stratification in patients diagnosed with upper displacement of right precordial leads. How much Brugada syndrome is an intense field of research is witnessed by 4 different consensus committees that took place in a relatively short period of time considering the recent discovery of this intricate arrhythmogenic disease. The main focus of this review is to describe the milestones in Brugada syndrome from its first phenotypic and genotypic appraisals to recent achievements in electrical therapies proposed for the management of this fascinating rhythm disturbance that, despite new diagnostic and therapeutic learnings, still predisposes to sudden cardiac death.

Hypnosis and Transversus Abdominis Plane Block for Brugada Syndrome Anesthesia: A Case Report

“…Pathophysiology included a sodium channelopathies which result in a reduction of sodium inflow current, thereby reducing phase 0 depolarization of the action potential and repolarization heterogeneity [1,4]. The association with ventricular arrhythmias and cardiac dysfunction is of concern to the anesthetist because medications routinely used in anesthetic practice may precipitate these events [2,5,6].…”

“…Because he has a Brugada syndrome type 2 with no history of ventricular fibrillation or syncope and no family history of sudden cardiac death, cardioverter defibrillator was not implanted [1][2][3].…”

“…Brugada syndrome is a primary electrical disorder affecting ionic channels of the heart and its anesthetic management is essential work because there are a lot of medications prohibited [1,2].…”

“…His investigations were within normal limit except surface electrocardiogram that found ST segments < 1 mm with saddle-type pattern (Brugada type 2). A provocative drug test with intravenous administration of ajmaline induced type 1 ECG morphology with ST-segment elevation > 2 mm).Because he has a Brugada syndrome type 2 with no history of ventricular fibrillation or syncope and no family history of sudden cardiac death, cardioverter defibrillator was not implanted [1][2][3].Monitoring consisted of ECG with continuous ST segment analysis, a pulse oximeter and non-invasive blood pressure measurement. He was connected to Oxygen 2 L/min with nasal cannula and an external defibrillator pads with an external defibrillator was placed.…”

Exercise stress test reveals ineligibility for subcutaneous implantable cardioverter defibrillator in patients with Brugada syndrome

“…The syndrome is characterized by an electrocardiographic pattern of ST segment elevation with type 1 morphology in the right precordial leads V1 to V3. Most cardiac events occur in middle‐aged men …”