| Literature DB >> 28389016 |
Andrés Ricardo Pérez-Riera1, Adrian Baranchuk2, Li Zhang3, Raimundo Barbosa-Barros4, Luiz Carlos de Abreu5, Pedro Brugada6.
Abstract
Type 1 myotonic dystrophy (DM1) is a hereditary neuromuscular disease affecting multiple organs in human adults. Here we report a 42-year-old man diagnosed with DM1. Having a history of progressive muscular weakness and gradual loss of visual acuity, he was referred to us by his ophthalmologist for risk assessment of undergoing cataract surgery. Cardiology workup revealed type 1 Brugada ECG pattern, positive late potentials and inducible ventricular fibrillation in an electrophysiology study. Literature review revealed that those ECG changes may be observed in DM1, suggesting that DM1 and Brugada syndrome may share a common pathophysiologic pathway.Entities:
Keywords: Brugada phenocopy; Brugada syndrome; Intraventricular conduction system; Steinert's disease; Type 1 Brugada ECG pattern
Mesh:
Year: 2017 PMID: 28389016 DOI: 10.1016/j.jelectrocard.2017.03.008
Source DB: PubMed Journal: J Electrocardiol ISSN: 0022-0736 Impact factor: 1.438