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Variants of Brugada Syndrome and Early Repolarization Syndrome: An Expanded Concept of J‐Wave Syndrome
Author(s) -
KIM SUNGHWAN,
NAM GIBYOUNG,
YUN SUNGCHEOL,
CHOI HYUNG OH,
CHOI KEEJOON,
JOUNG BOYOUNG,
PAK HUINAM,
LEE MOONHYOUNG,
KIM SUNG SOON,
PARK SEUNGJUNG,
ON YOUNG KEUN,
KIM JUNE SOO,
OH ILYOUNG,
CHOI EUEKEUN,
OH SEIL,
CHOI YUNSHIK,
CHOI JONG IL,
PARK SANG WEON,
KIM YOUNGHOON,
OH YONGSEOG,
LEE MAN YOUNG,
LIM HONG EUY,
LEE YOUNGSOO,
CHO YONGKEUN,
KIM JUN,
RHEE KYOUNGSUK,
LEE DONGIL,
CHO DAE KYOUNG,
KIM YOUHO
Publication year - 2017
Publication title -
pacing and clinical electrophysiology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.686
H-Index - 101
eISSN - 1540-8159
pISSN - 0147-8389
DOI - 10.1111/pace.13000
Subject(s) - brugada syndrome , medicine , j wave , cardiology , repolarization , benign early repolarization , implantable cardioverter defibrillator , ventricular fibrillation , electrocardiography , precordial examination , pathogenesis , st segment , myocardial infarction , electrophysiology
Background The role of J‐waves in the pathogenesis of ventricular fibrillation (VF) occurring in structurally normal hearts is important. Methods We evaluated 127 patients who received an implantable cardioverter‐defibrillator (ICD) for Brugada syndrome (BS, n = 53), early repolarization syndrome (ERS, n = 24), and patients with unknown or deferred diagnosis (n = 50). Electrocardiography (ECG), clinical characteristics, and ICD data were analyzed. Results J‐waves were found in 27/50 patients with VF of unknown/deferred diagnosis. The J‐waves were reminiscent of those seen in BS or ERS, and this subgroup of patients was termed variants of ERS and BS (VEB). In 12 VEB patients, the J/ST/T‐wave morphology was coved, although amplitudes were <0.2 mV. In 15 patients, noncoved‐type J/ST/T‐waves were present in the right precordial leads. In the remaining 23 patients, no J‐waves were identified. VEB patients exhibited clinical characteristics similar to those of BS and ERS patients. Phenotypic transition and overlap were observed among patients with BS, ERS, and VEB. Twelve patients with BS had background inferolateral ER, while five ERS patients showed prominent right precordial J‐waves. Patients with this transient phenotype overlap showed a significantly lower shock‐free survival than the rest of the study patients. Conclusions VEB patients demonstrate ECG phenotype similar to but distinct from those of BS and ERS. The spectral nature of J‐wave morphology/distribution and phenotypic transition/overlap suggest a common pathophysiologic background in patients with VEB, BS, and ERS. Prognostic implication of these ECG variations requires further investigation.