Is the Brugada syndrome a distinct clinical entity?

In 1992, Brugada and Brugada described a syndrome characterized by right bundle branch block pattern with ST elevation in leads V1 through V3 and a history of sudden death due to polymorphic ventricular tachycardia or ventricular fibrillation. Since these patients had no evidence of cardiac disease, these findings were ascribed to a distinct clinical entity. Further experience has shown that this same pattern may be mimicked by patients with right ventricular dysplasia, acute ischemia of the right ventricle, other infiltrative cardiomyopathies, as well as tricyclic drug overdose. The pathogenesis of these changes may be due to loss of the dome configuration in the transmembrane potential of right ventricular epicardial cells, which would result in a voltage gradient producing ST elevation. Other explanations involve delayed conduction in a dysplastic right ventricle. The clinical importance of this syndrome is that it calls attention to patients at risk for sudden cardiac death. In addition, these observations have sparked the interest of basic electrophysiologists relative to the relationship of these ECG waveforms and malignant ventricular arrhythmias. Finally, the clinician must exclude other organic diseases before diagnosing this entity.