Detection of pathogenic variants in genes involved in cardiac conduction (GPD1L, RYR2, SCN5A, SCN2B) and structural (CTNNA3) defects indicates a more comprehensive approach in the diagnostic workup and requires additional testing; ajmaline provocation tests (Brugada syndrome), electrophysiology cardiac studies as well as magnetic resonance and echocardiographic strain imaging. Here, CTNNA3 is linked to Brugada syndrome.