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KMID : 1011920150160020108
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International Journal of Arrhythmia
2015 Volume.16 No. 2 p.108 ~ p.112
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An Atypical Familial Presentation of Brugada Syndrome: Can this be Considered as an Overlap Syndrome of the Long QT and Brugada Syndromes?
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Shin Seung-Yong
Woo Jong-Shin
Uhm Jae-Sun
Lee Moon-Hyoung
Kim Sung-Soon
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Abstract
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A 24-year-old man was referred to the Armed Forces Capital Hospital after experiencing aborted sudden cardiac death. His first electrocardiogram (ECG) demonstrated QT prolongation (QTc, 520 ms) with narrow-based and inverted symmetric T waves in leads V3-6 and the inferior leads. The patient was provided genetic counseling. Meanwhile, his father and brother showed ECG patterns suggestive of Brugada syndrome, which became apparent with a flecainide infusion test. Moreover, Brugada syndrome was confirmed in all 3 persons based on (1) ECG changes during follow-up, (2) provocation test with flecainide, and (3) the presence of SCN5A gene mutation and programmed ventricular study reproducibly induced fast polymorphic ventricular tachycardia degenerating VF. Implantable cardioverter defibrillators were implanted in all patients (the proband, his father, and his younger brother).
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KEYWORD
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Brugada Syndrome, Long QT Syndrome
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