Brugada like syndrome in infancy

Brugada like syndrome in infancy

Brugada like syndrome in infancy: Brugada syndrome is a cardiac channelopathy prone for life threatening cardiac arrhythmias and sudden cardiac death. Cases of Brugada syndrome usually manifest in adult life with an incomplete right bundle branch block like pattern with ST segment elevation which is coved in the right precordial leads. Sometimes the characteristic Brugada pattern is present on routine ECG while it has to be unmasked by drugs like Flecainide in other situations. The first mutation to be described in Brugada syndrome was of the sodium channel gene SCN5A. Kanter RJ and colleagues [Brugada-Like Syndrome in Infancy Presenting With Rapid Ventricular Tachycardia and Intraventricular Conduction Delay. Circulation. 2012; 125: 14-22] have now come up with a retrospective analysis demonstrating a Brugada like syndrome in infancy, which is a rare age group for the manifestation of the typical Brugada syndrome. From an analysis of all clinical material at a single institution over a period of nine years, they could identify twelve infants with ventricular tachycardia/fibrillation and a structurally normal heart. Five of them who had no other pathologies had mutations in sodium channel (SCN5A) or calcium channel (CaCNB2b). Four of these infants had undergone electrophysiology studies and three of them had Brugada pattern after intravenous procainamide. Authors conclude that infants with ventricular tachycardia in the absence of structural or metabolic factors could have primary electrical disorders with mutations in the cardiac depolarizing channels.