Variants of Brugada Syndrome and Early Repolarization Syndrome: An Expanded Concept of J-Wave Syndrome

Sung Hwan Kim, Gi Byoung Nam, Sung Cheol Yun, Hyung Oh Choi, Kee Joon Choi, Boyoung Joung, Hui Nam Pak, Moon Hyoung Lee, Sung Soon Kim, Seung Jung Park, Young Keun On, June Soo Kim, Il Young Oh, Eue Keun Choi, Seil Oh, Yun Shik Choi, Jong Il Choi, Sang Weon Park, Young Hoon Kim, Yong Seog OhMan Young Lee, Hong Euy Lim, Young Soo Lee, Yongkeun Cho, Jun Kim, Kyoung Suk Rhee, Dong Il Lee, Dae Kyoung Cho, You Ho Kim

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Abstract

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.

Original languageEnglish
Pages (from-to)162-174
Number of pages13
JournalPACE - Pacing and Clinical Electrophysiology
Volume40
Issue number2
DOIs
Publication statusPublished - 2017 Feb 1

All Science Journal Classification (ASJC) codes

  • Cardiology and Cardiovascular Medicine

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    Kim, S. H., Nam, G. B., Yun, S. C., Choi, H. O., Choi, K. J., Joung, B., Pak, H. N., Lee, M. H., Kim, S. S., Park, S. J., On, Y. K., Kim, J. S., Oh, I. Y., Choi, E. K., Oh, S., Choi, Y. S., Choi, J. I., Park, S. W., Kim, Y. H., ... Kim, Y. H. (2017). Variants of Brugada Syndrome and Early Repolarization Syndrome: An Expanded Concept of J-Wave Syndrome. PACE - Pacing and Clinical Electrophysiology, 40(2), 162-174. https://doi.org/10.1111/pace.13000