The History of the Precordial Early Repolarization and Sudden Death Syndrome, Lately Named Brugada Syndrome

This paper intends to go through the medical history of a new syndrome, beginning from its incidental observation to the nowadays ongoing reports quickly approaching 7000 published papers. This large number makes it difficult for the researcher to correctly quote the previous significant published data, and the usual strategy is to copy and paste the last articles references. This review is mainly detailed historical research of the step-by-step journey mainly of the first three decades, with less attention to the ongoing and late scientific controversies that are indeed quoted. The new syndrome was early named “precordial early repolarization (PER) syndrome” but became popular after being renamed “Brugada syndrome” (BS). Nowadays it is classified as one of the “J wave syndromes” (JWSs). The main characteristic of this new entity was an unusual astonishing precordial coved ST segment elevation that gave rise since its first descriptions to two different pathophysiological theories, one organic and the second functional. The first theory ascribed the ST elevation to an unusual pattern of depolarization at the right ventricular outflow tract (RVOT), while the second favored an abnormal dynamic repolarization pattern. Both phenomena were sometimes linked to an ion channel genetic abnormality. In the following decades, many eminent scientists and also some excellent humble cardiologists made significant observations regarding epidemiology, laboratory, diagnostic techniques, genetic, clinical findings, histology, embryology, therapeutic approaches, and risk stratification. This rush “to be the first who” has created more confusion than certainty, and only in this last decade a more scientific and less emotional approach has led to a common acceptance of an underlying organic background that causes a strange conduction delay mainly at the epicardial level of the RVOT. “Next generation” cardiologists are in charge of further elucidating the genetic, the structural, and electrical pathophysiology, and the correct risk stratification needed to correctly identify the true patients who need a therapy and avoid unusual and dangerous treatments to healthy people with a benign strange ECG.