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. 2020 Jul 24;7:117. doi: 10.3389/fcvm.2020.00117

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Copyright © 2020 Nijak, Labro, De Wilde, Dewals, Peigneur, Tytgat, Snyders, Sieliwonczyk, Simons, Van Craenenbroeck, Schepers, Van Laer, Saenen, Loeys and Alaerts.

This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

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(A) Timeline of diagnostic interventions for the described case. ECG traces of (B) the proband on the day of diagnosis with absent atrial activity with junctional escape, (C) repeated during fever at 39°C which unmasked BrS ECG pattern with elevated ST segment, (D) the father carrying the SCN5A founder mutation with absent BrS ECG type-1 pattern after ajmaline challenge, but (E) present during fever at 39°C (visible in lead V1), and (F) the asymptomatic aunt carrying theSCN5A founder mutation with ECG type-1 pattern after ajmaline challenge. (G) Pedigree of the family showing the segregation of the identified variants. Full symbols indicate the affected individuals. The red plus and minus symbols indicate, respectively, the presence or the absence of the variant indicated on the left side of the pedigree.