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KMID : 1011920140150010020
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International Journal of Arrhythmia
2014 Volume.15 No. 1 p.20 ~ p.27
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Single center experience of sudden cardiac death caused by primary electrical disorder: focused on early repolarization syndrome
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Nam Gi-Byoung
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Abstract
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Evaluation and management of sudden cardiac death (SCD) survivors who do not have identifiable structural heart diseases is clinically challenging. This requires a systematic approach of stepwise exclusion for all the possible genetic or ion channel diseases or still poorly defined (or being identified) arrhythmia syndromes. In this paper, we report a method of systematic evaluation for SCD survivors, and describe the electrocardiographic characteristics of the patients who have survived SCD or ventricular fibrillation (VF) and received implantable defibrillator implantation at the Asan Medical Center.
Among the 100 study patients, Brugada syndrome (BS), long QT syndrome (LQTS) and early repolarization syndrome (ERS) were responsible in 55% of the study population, while in the remaining 45% of the patients, diagnosis remained unknown. In 19% of the subjects, J waves did not satisfy the diagnostic criteria of BS or ERS, but were thought to have played an important role in the pathogenesis of VF. J waves observed in this subgroup of patients are classified into three patterns; localized type 1 Brugada pattern, type 1-like coved-type pattern but with low (<2 mm) amplitude, and isolated right precordial J wave. We propose this subgroup of patients be called ‘J wave-related idiopathic VF’, and should be included in a broader definition of ‘J wave syndrome’. The mainstay for treatment of these electrical arrhythmic disorders is implantable cardioverter defibrillator. Quinidine may be effective in some selected patients where implantation of defibrillator is not suitable or frequent defibrillator shocks are troublesome.
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KEYWORD
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sudden cardiac death, ventricular fibrillation, electrocardiography, J waves
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