The Brugada syndrome.

ABSTRACT

PURPOSE OF REVIEW The Brugada syndrome has been an area of intensive investigation since its earliest description in 1992, both on a clinical and on a basic research level. In this review, we will focus on recent achievements in the molecular dissection of the disease pathophysiology and on large multicenter studies dealing with prognostic markers and the natural history of the Brugada syndrome. RECENT FINDINGS: In the past year, two additional genetic pathways have been associated with the disease. Also, an inflammatory or infectious etiology has recently been linked with the Brugada syndrome. The debate on the predictive role of programmed electrical stimulation is still ongoing. Very recently, large follow-up studies questioned the prognostic role of programmed electrical stimulation in this disease. SUMMARY: Knowledge on the genetic determinants of the Brugada syndrome remains limited. Therefore, the management and the risk stratification of patients should be performed on a clinical basis. Sufficient evidence exists to reassure clinicians who feel reluctant to include programmed electrical stimulation in the risk stratification strategy of asymptomatic Brugada syndrome patients.