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

Research output: Contribution to journalArticle

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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

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Brugada Syndrome
Electrocardiography
Implantable Defibrillators
Ventricular Fibrillation
Phenotype

All Science Journal Classification (ASJC) codes

  • Cardiology and Cardiovascular Medicine

Cite this

Kim, Sung Hwan ; Nam, Gi Byoung ; Yun, Sung Cheol ; Choi, Hyung Oh ; Choi, Kee Joon ; Joung, Boyoung ; Pak, Hui Nam ; Lee, Moon Hyoung ; Kim, Sung Soon ; Park, Seung Jung ; On, Young Keun ; Kim, June Soo ; Oh, Il Young ; Choi, Eue Keun ; Oh, Seil ; Choi, Yun Shik ; Choi, Jong Il ; Park, Sang Weon ; Kim, Young Hoon ; Oh, Yong Seog ; Lee, Man Young ; Lim, Hong Euy ; Lee, Young Soo ; Cho, Yongkeun ; Kim, Jun ; Rhee, Kyoung Suk ; Lee, Dong Il ; Cho, Dae Kyoung ; Kim, You Ho. / Variants of Brugada Syndrome and Early Repolarization Syndrome : An Expanded Concept of J-Wave Syndrome. In: PACE - Pacing and Clinical Electrophysiology. 2017 ; Vol. 40, No. 2. pp. 162-174.
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title = "Variants of Brugada Syndrome and Early Repolarization Syndrome: An Expanded Concept of J-Wave Syndrome",
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.",
author = "Kim, {Sung Hwan} and Nam, {Gi Byoung} and Yun, {Sung Cheol} and Choi, {Hyung Oh} and Choi, {Kee Joon} and Boyoung Joung and Pak, {Hui Nam} and Lee, {Moon Hyoung} and Kim, {Sung Soon} and Park, {Seung Jung} and On, {Young Keun} and Kim, {June Soo} and Oh, {Il Young} and Choi, {Eue Keun} and Seil Oh and Choi, {Yun Shik} and Choi, {Jong Il} and Park, {Sang Weon} and Kim, {Young Hoon} and Oh, {Yong Seog} and Lee, {Man Young} and Lim, {Hong Euy} and Lee, {Young Soo} and Yongkeun Cho and Jun Kim and Rhee, {Kyoung Suk} and Lee, {Dong Il} and Cho, {Dae Kyoung} and Kim, {You Ho}",
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pages = "162--174",
journal = "PACE - Pacing and Clinical Electrophysiology",
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Kim, SH, Nam, GB, Yun, SC, Choi, HO, Choi, KJ, Joung, B, Pak, HN, Lee, MH, Kim, SS, Park, SJ, On, YK, Kim, JS, Oh, IY, Choi, EK, Oh, S, Choi, YS, Choi, JI, Park, SW, Kim, YH, Oh, YS, Lee, MY, Lim, HE, Lee, YS, Cho, Y, Kim, J, Rhee, KS, Lee, DI, Cho, DK & Kim, YH 2017, 'Variants of Brugada Syndrome and Early Repolarization Syndrome: An Expanded Concept of J-Wave Syndrome', PACE - Pacing and Clinical Electrophysiology, vol. 40, no. 2, pp. 162-174. https://doi.org/10.1111/pace.13000

Variants of Brugada Syndrome and Early Repolarization Syndrome : An Expanded Concept of J-Wave Syndrome. / Kim, Sung Hwan; Nam, Gi Byoung; Yun, Sung Cheol; Choi, Hyung Oh; Choi, Kee Joon; Joung, Boyoung; Pak, Hui Nam; Lee, Moon Hyoung; Kim, Sung Soon; Park, Seung Jung; On, Young Keun; Kim, June Soo; Oh, Il Young; Choi, Eue Keun; Oh, Seil; Choi, Yun Shik; Choi, Jong Il; Park, Sang Weon; Kim, Young Hoon; Oh, Yong Seog; Lee, Man Young; Lim, Hong Euy; Lee, Young Soo; Cho, Yongkeun; Kim, Jun; Rhee, Kyoung Suk; Lee, Dong Il; Cho, Dae Kyoung; Kim, You Ho.

In: PACE - Pacing and Clinical Electrophysiology, Vol. 40, No. 2, 01.02.2017, p. 162-174.

Research output: Contribution to journalArticle

TY - JOUR

T1 - Variants of Brugada Syndrome and Early Repolarization Syndrome

T2 - An Expanded Concept of J-Wave Syndrome

AU - Kim, Sung Hwan

AU - Nam, Gi Byoung

AU - Yun, Sung Cheol

AU - Choi, Hyung Oh

AU - Choi, Kee Joon

AU - Joung, Boyoung

AU - Pak, Hui Nam

AU - Lee, Moon Hyoung

AU - Kim, Sung Soon

AU - Park, Seung Jung

AU - On, Young Keun

AU - Kim, June Soo

AU - Oh, Il Young

AU - Choi, Eue Keun

AU - Oh, Seil

AU - Choi, Yun Shik

AU - Choi, Jong Il

AU - Park, Sang Weon

AU - Kim, Young Hoon

AU - Oh, Yong Seog

AU - Lee, Man Young

AU - Lim, Hong Euy

AU - Lee, Young Soo

AU - Cho, Yongkeun

AU - Kim, Jun

AU - Rhee, Kyoung Suk

AU - Lee, Dong Il

AU - Cho, Dae Kyoung

AU - Kim, You Ho

PY - 2017/2/1

Y1 - 2017/2/1

N2 - 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.

AB - 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.

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