Unmasked Brugada Pattern by Ajmaline Challenge in Patients with Myotonic Dystrophy Type 1
Myotonic dystrophy type 1 (DM1) generates missplicing of the SCN5A gene, encoding the cardiac sodium channel (Nav1.5). Brugada syndrome, which partly results from Nav1.5 dysfunction and causes increased VF occurrence, can be unmasked by ajmaline. We aimed to investigate the response to ajmaline challenge in DM1 patients and its potential impact on their sudden cardiac death risk stratification.
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