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21-05-2019 | Rhythm Puzzle - Answer | Uitgave 9/2019 Open Access

Netherlands Heart Journal 9/2019

Funny waves in repolarisation and tachycardia in a patient suspected for Brugada syndrome

Tijdschrift:
Netherlands Heart Journal > Uitgave 9/2019
Auteurs:
L. Jordaens, L. Timmers, P. Goethals

Answer

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.

Conflict of interest

L. Jordaens, L. Timmers and P. Goethals declare that they have no competing interests.
Open Access This article is distributed under the terms of the Creative Commons Attribution 4.0 International License ( http://​creativecommons.​org/​licenses/​by/​4.​0/​), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made.

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Netherlands Heart Journal

Het Netherlands Heart Journal wordt uitgegeven in samenwerking met de Nederlandse Vereniging voor Cardiologie en de Nederlandse Hartstichting. Het tijdschrift is Engelstalig en wordt gratis beschikbaa ...

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