Contemporary Perspectives on J-Wave Syndromes: An Expert Consensus Statement

Area of Science:

• Cardiology
• Electrophysiology
• Genetics

Background:

• J-wave syndromes (JWS), encompassing Brugada syndrome (BrS) and early repolarization syndrome (ERS), are significant causes of sudden cardiac death in structurally normal hearts.
• Recent advances have redefined the understanding of BrS as a microstructural-electrical continuum and ERS as a condition with significant risk when associated with specific ECG findings and arrhythmias.

Purpose of the Study:

• To update and extend previous consensus guidelines on the diagnosis, risk stratification, and management of JWS.
• To emphasize the evolving understanding of JWS as a spectrum of microstructural-electrical disease.
• To highlight epicardial substrate ablation as a major therapeutic advancement.

Main Methods:

• International consensus update based on recent advances in genetics, pathophysiology, and therapy.
• Review of large registries and randomized trials on epicardial substrate ablation.
• Integration of clinical features, ECG markers, electrophysiologic studies, and genetic data for risk stratification.

Main Results:

• SCN5A remains the primary gene associated with JWS, but polygenic susceptibility plays a significant role.
• Risk stratification requires multiparametric assessment, with prior cardiac arrest or syncope indicating highest risk.
• Epicardial substrate ablation demonstrates durable suppression of ventricular fibrillation with acceptable safety.

Conclusions:

• J-wave syndromes represent a spectrum of microstructural-electrical disease.
• Epicardial substrate ablation is a transformative therapy for managing ventricular arrhythmias in JWS.
• Contemporary diagnostic frameworks, risk-stratification tools, and treatment algorithms are essential for optimal patient care.