Brugada ECG Pattern in Wilson Disease

Abstract

Background

Wilson disease and Brugada syndrome initially appear unrelated. Although cardiac manifestations in Wilson disease are increasingly recognized, it remains controversial whether direct copper infiltration is the sole mechanism underpinning structural and electroanatomical abnormalities.

Case Summary

We present the case of a 40-year-old man with genetically confirmed Wilson disease who exhibited a spontaneous Brugada type 3 electrocardiographic pattern. Subsequent to a flecainide challenge, a Brugada type 1 electrocardiographic pattern was unmasked, although no prior history of arrhythmias was reported. Regular follow-up care was not accompanied by any adverse cardiac events.

Discussion

This is the first documented case of such a co-occurrence, to our knowledge, and it suggests a potential Wilson-induced Brugada phenocopy, possibly attributable to the mechanistic effects of copper's redox properties, which may mimic the microstructural impairment postulated in genetic Brugada syndrome.

Take-Home Message

Systemic metabolic disturbances variously precipitate cardiac electrical abnormalities and should be considered in atypical presentations of arrhythmia syndromes.