Brugada syndrome and right ventricular conduction delay: What else?

Abstract

In the past decades, we have gained enormous knowledge about the epidemiology and pathophysiologic mechanisms underlying inheritable arrhythmia syndromes such as Brugada syndrome and long QT syndrome. However, this has not made clinical decision-making easy when we are confronted with presymptomatic patients and their family members. The booming number of case reports and (smaller) series on these syndromes often resulted in a fast growing number of presymptomatic patients being uncovered and treated with invasive and complication-sensitive therapy such as implantable cardioverter-defibrillators at a young age. In particular, this scenario is true for Brugada syndrome. Meanwhile, both risk stratification in Brugada yndrome and its underlying pathophysiologic mechanisms ecame heavily disputed among experts, further complicatng clinical decision-making. The latter is fueled by the iscussion on whether Brugada syndrome is a depolarizaion (ie, conduction) disorder or a repolarization disorder. This distinction is important for understanding the pathophysiologic mechanisms underlying Brugada syndrome and recently has resulted in specific treatment options for symptomatic Brugada syndrome patients. All of these issues urge physicians worldwide to keep in mind that (1) strict diagnostic criteria should be followed; (2) patients need either to be discussed with a specialized center, or be referred especially when invasive treatment is considered (excellent example for long QT syndrome); (3) noninvasive therapy, such as advising against the use of certain drugs and treatment of fever when appropriate, should be extensively used; and (4) patients should receive adequate (family) counseling.