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The resting electrocardiogram (ECG) is diagnostic for Brugada syndrome [1]. His 2 brothers both had ECG patterns typical for Brugada syndrome, his mother had drug-induced Brugada syndrome. His father was from East Asian descent. The waves in V2 are indeed suggestive of an epsilon wave, which might be a sign for arrhythmogenic right ventricular dysplasia, and hence an overlap situation [2]. However, there were no clinical, nor other signs for this disease, and the cardiac magnetic resonance imaging (MRI) was entirely normal. Depolarising abnormalities are observed in 13% of patients with spontaneous or drug-induced Brugada syndrome, without any evidence of cardiomyopathy [2]. The tachycardia was a left-sided posterior fascicular tachycardia and was easily ablated on a site with a Purkinje potential (Fig. 1 and 2). A dual-chamber implantable cardioverter-defibrillator was implanted, given the bradycardia and conduction disease. With a follow-up of 3 years, no events have been recorded. Fascicular tachycardia has in principle no relation with Brugada syndrome, which mainly affects the right ventricular outflow tract [3]. That some molecular common link may exist cannot be excluded. Genetic analysis showed 2 missense variants in the SCN5A gene, and one TMEM43 variant, all with unclear relation to his disease, given his East Asian roots.
References
Gourraud JB, Barc J, Thollet A, et al. Brugada syndrome: Diagnosis, risk stratification and management. Arch Cardiovasc Dis. 2017;110:188–95.
Letsas KP, Efremidis M, Weber R, et al. Epsilon-like waves and ventricular conduction abnormalities in subjects with type 1 ECG pattern of Brugada syndrome. Heart Rhythm. 2011;8:874–8.
Rodríguez-Mañero M, Sacher F, de Asmundis C, et al. Monomorphic ventricular tachycardia in patients with Brugada syndrome: A multicenter retrospective study. Heart Rhythm. 2016;13:669–82.
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L. Jordaens, L. Timmers and P. Goethals declare that they have no competing interests.
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Jordaens, L., Timmers, L. & Goethals, P. Funny waves in repolarisation and tachycardia in a patient suspected for Brugada syndrome. Neth Heart J 27, 454–455 (2019). https://doi.org/10.1007/s12471-019-1292-8
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DOI: https://doi.org/10.1007/s12471-019-1292-8