Brugada syndrome is a rare inherited arrhythmia disorder where evolving genetic insights and emerging therapies like substrate-based catheter ablation and subcutaneous ICDs hold significant promise.
This review highlights the evolving understanding of Brugada syndrome genetics and pathophysiology, alongside emerging therapies like substrate-based ablation and subcutaneous ICDs.
Brugada syndrome (BrS) is a rare inherited arrhythmia disorder associated with sudden cardiac death secondary to malignant ventricular arrhythmias. Since its first mention approximately 25 years ago, major strides have been made towards unraveling the condition's genetic and mechanistic underpinnings. Despite considerable progress, however, gaps in the understanding of BrS continue to persist, and clinical management of affected individuals remains challenging. Identification of an underlying genetic culprit continues to be elusive in the majority of patients, while discord regarding the condition's underlying pathophysiology also persists, with strong lines of evidence present for both the "depolarization" and "repolarization" hypotheses. Exciting new therapeutic options hold significant promise, including substrate-based catheter ablation and the subcutaneous implantable cardioverter-defibrillator, although the decision of when to intervene in the cases of asymptomatic patients remains unclear. Provided that the risk of events in BrS is not truly stochastic, distinct sub-phenotypes of the condition, possessing variable levels of arrhythmic risk, may exist, and their identification may lead to the improved care of BrS patients and their families.
Duffett et al. (Thu,) conducted a review in Brugada Syndrome. Brugada syndrome is a rare inherited arrhythmia disorder where evolving genetic insights and emerging therapies like substrate-based catheter ablation and subcutaneous ICDs hold significant promise.
Synapse has enriched 5 closely related papers on similar clinical questions. Consider them for comparative context: