4.4 Editorial Material

Brugada syndrome: A most shocking experience

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HEART RHYTHM
卷 20, 期 11, 页码 1589-1590

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ELSEVIER SCIENCE INC
DOI: 10.1016/j.hrthm.2023.04.054

关键词

Atrial; Brugada syndrome; Genotype; Phenotype; Ventricular

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A 47-year-old man with Brugada syndrome presented with ongoing management following recent presyncope. During an exercise stress test, he developed broad complex tachycardia and received a shock from his defibrillator. ECG analysis revealed typical features of Brugada syndrome and the possibility of atrial flutter with aberrancy as the mechanism of the tachycardia.
Case presentation A 47-year-old man with a personal and familial history of Brugada syndrome presented for ongoing management following recent presyncope. He had a primary prevention single-chamber defibrillator in situ with no appropriate ther-apies required since implantation 7 years ago. During an ex-ercise stress test, he developed a broad complex tachycardia and received a shock from his defibrillator. What is the mech-anism of the tachycardia? Electrocardiogram analysis At baseline, the patient showed alternating sinus arrest with junctional escape, sinus bradycardia, and right ventricular apical pacing at a lower rate of 40 bpm (Figure 1A). With exertion, an irregularly irregular rhythm consistent with atrial fibrillation ensues, which then organizes into atrial flutter with 1:1 atrioventricular conduction (Figure 1B). There is a transition to a broad QRS complex with right bundle branch morphology for which a defibrillator shock is delivered (Figure 1C). Although this was interpreted clinically as ven-tricular tachycardia (VT) at the time, the comparable cycle length is in keeping with ongoing atrial flutter with aber-rancy. Discussion The Brugada syndrome is an inherited channelopathy charac-terized by typical electrocardiographic patterns in the right precordial leads (coved-type ST-segment elevation >2 mm followed by a negative T wave in >1 of the right precordial leads V1-V2).1 Although the association with ventricular ar-rhythmias and sudden cardiac death is best known and feared,

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