Inherited arrhythmias: Brugada syndrome and early repolarisation syndrome

Abstract

In this chapter a detailed overview on Brugada syndrome and the early repolarisation syndrome is presented. These two disease entities are associated with malignant arrhythmias and sudden death in otherwise healthy young adults and even children. We discuss their history, clinical perspectives (including patient characteristics and epidemiology), the pathway to the diagnosis, pathophysiological mechanisms (including genetic associations) and review clinical risk stratification and treatment. With this chapter we aim to provide a thorough insight in the knowledge base that has developed in these entities in the past decades and we specifically included current debates and uncertainties that are relevant to daily practice and influence our judgements. Undoubtedly our understanding of these syndromes will continue to develop and influence the management of patients and their families in the coming years. Relevant topics for future research in these syndromes are likewise provided. We sincerely hope that this chapter is of practical use for al health care professionals and researchers in this field and that it will contribute to a better understanding and care for affected patients and their families.