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

J-wave syndromes (JWS)-comprising Brugada syndrome (BrS) and early repolarization syndrome (ERS)-are important causes of malignant ventricular arrhythmias and sudden cardiac death in patients whose hearts appear structurally normal. Since the 2016 consensus, advances in genetics, pathophysiology, and therapy have redefined both understanding and management. BrS, once viewed as a purely electrical disorder, is now recognized along a microstructural-electrical continuum, with sodium-channel dysfunction and subtle epicardial fibrosis of the right ventricular outflow tract as key contributors. Likewise, ERS-historically considered benign-carries significant risk when inferolateral J-waves coexist with arrhythmic events. Genetically, SCN5A remains the sole gene with definitive disease association, while polygenic susceptibility materially modulates risk, underscoring complex inheritance. Risk stratification remains challenging: patients with prior cardiac arrest or arrhythmic syncope are highest risk, whereas asymptomatic individuals warrant multiparametric assessment integrating clinical features, ECG markers, electrophysiologic studies, and genetics. For decades, treatment centered on implantable cardioverter-defibrillators and quinidine, both limited by availability, tolerance, and device complications. More recently, epicardial substrate ablation has emerged as a transformative therapy, with large registries and randomized trials demonstrating durable suppression of ventricular fibrillation and acceptable safety. This APHRS-organized international consensus updates and extends the 2016 Expert Consensus and the 2022 ESC Guidelines, providing contemporary diagnostic frameworks, pragmatic risk-stratification tools, and treatment algorithms for BrS and ERS. It emphasizes JWS as a microstructural-electrical disease spectrum and elevates substrate ablation as a major therapeutic advance, while outlining priorities for genetics, risk-stratification and treatment algorithms.

Authors

Institutions

Publication Details

Journal
Journal of Arrhythmia
Published
2026-02-01
DOI
https://doi.org/10.1002/joa3.70284
Primary Topic
Cardiac electrophysiology and arrhythmias
Type
article
Field-Weighted Citation Impact
0.00

Funders

Controls
|||
ALL TIME
JAN
FEB
MAR
APR
MAY
JUN
JUL
AUG
SEP
OCT
article

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

Arthur A.M. Wilde, Connie R. Bezzina, CARLO PAPPONE, Jyh‐Ming Jimmy Juang et al.
Journal of Arrhythmia
Cardiac electrophysiology and arrhythmias
article

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

Arthur A.M. Wilde, Connie R. Bezzina, CARLO PAPPONE, Jyh‐Ming Jimmy Juang, Raphaël Rosso, N. Makita, Tachapong Ngarmukos, Hiroshi Nakagawa, P S Chen, Apichai Khongphatthanayothin, Gumpanart Veerakul, K. Nademanee, John D. Fisher, R F M Coronel, Sami Viskin, Elijah R Behr, W Shimizu, Akihiko Nogami, Michel Haissaguerre, Hiroshi Morita, Fa Po Chung, Silvia G. Priori
article en

Abstract

J-wave syndromes (JWS)-comprising Brugada syndrome (BrS) and early repolarization syndrome (ERS)-are important causes of malignant ventricular arrhythmias and sudden cardiac death in patients whose hearts appear structurally normal. Since the 2016 consensus, advances in genetics, pathophysiology, and therapy have redefined both understanding and management. BrS, once viewed as a purely electrical disorder, is now recognized along a microstructural-electrical continuum, with sodium-channel dysfunction and subtle epicardial fibrosis of the right ventricular outflow tract as key contributors. Likewise, ERS-historically considered benign-carries significant risk when inferolateral J-waves coexist with arrhythmic events. Genetically, SCN5A remains the sole gene with definitive disease association, while polygenic susceptibility materially modulates risk, underscoring complex inheritance. Risk stratification remains challenging: patients with prior cardiac arrest or arrhythmic syncope are highest risk, whereas asymptomatic individuals warrant multiparametric assessment integrating clinical features, ECG markers, electrophysiologic studies, and genetics. For decades, treatment centered on implantable cardioverter-defibrillators and quinidine, both limited by availability, tolerance, and device complications. More recently, epicardial substrate ablation has emerged as a transformative therapy, with large registries and randomized trials demonstrating durable suppression of ventricular fibrillation and acceptable safety. This APHRS-organized international consensus updates and extends the 2016 Expert Consensus and the 2022 ESC Guidelines, providing contemporary diagnostic frameworks, pragmatic risk-stratification tools, and treatment algorithms for BrS and ERS. It emphasizes JWS as a microstructural-electrical disease spectrum and elevates substrate ablation as a major therapeutic advance, while outlining priorities for genetics, risk-stratification and treatment algorithms.

Journal of ArrhythmiaVol. 42(1)
Cedars-Sinai Medical Center (US), Heart Rhythm Society (US), Cleveland Clinic (US), University of Tsukuba (JP), National Yang Ming Chiao Tung University (TW), Vita-Salute San Raffaele University (IT), Chulalongkorn University (TH), Okayama University (JP), St George's, University of London (GB), National Taiwan University (TW), University of Pavia (IT), Bumrungrad International Hospital (TH), Tel Aviv Sourasky Medical Center (IL), St George’s University Hospitals NHS Foundation Trust (GB), Bangkok Hospital (TH), Sir Run Run Shaw Hospital (CN), Taipei Veterans General Hospital (TW), Electrophysiology and Heart Modeling Institute (FR), Istituti Clinici Scientifici Maugeri (IT), New Tokyo Hospital (JP), IRCCS Policlinico San Donato (IT), Amsterdam Neuroscience (NL), National Cerebral and Cardiovascular Center (JP), Hôpital Cardiologique du Haut-Lévêque (FR), Zhejiang Lab (CN), Keiyukai Sapporo Hospital (JP), National Taiwan University Hospital (TW), Ramathibodi Hospital (TH), Genomics England (GB), Pacific Rim Electrophysiology Research Institute (US), Amsterdam University Medical Centers (NL), Toujinkai Hospital (JP), Amsterdam University of Applied Sciences (NL), Zhejiang University (CN), University of Amsterdam (NL)
Bristol-Myers Squibb, Pfizer, Bayer, BioMarin Pharmaceutical, Boston Scientific Corporation, Abbott Japan, Japan Lifeline, Invitae, Daiichi-Sankyo, National Research Council of Thailand, St. Jude Medical, Medtronic Japan, Daiichi Sankyo Europe
Openalex Percentile: Top 51%
Cardiac electrophysiology and arrhythmias
DOIOpen Access PDFOpenAlex
AI Navigator

Ask Laika to Summarize, Analyze, and Connect papers live on the map.

Summarize Papers & Methodologies

Extract key findings, datasets, and comparative methods across publications.

Benchmark Rankings & Visual Analytics

Rank top research institutions, authors, funders, topics, and journals by Field-Weighted Citation Impact (FWCI) and paper volume with instant charts.

Connect Distant Disciplines

Bridge topological clusters on the map to find hidden collaborative intersections.