Literature DB >> 28389016

Myotonic dystrophy and Brugada syndrome: A common pathophysiologic pathway?

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.
Copyright © 2017 Elsevier Inc. All rights reserved.

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


  2 in total

1.  Transient high-degree right bundle branch block masking the type 1 Brugada ECG pattern associated with possible transient early repolarization syndrome.

Authors:  Andrés Ricardo Pérez-Riera; Raimundo Barbosa Barros; Rodrigo Daminello-Raimundo; Luiz Carlos de Abreu; Kjell Nikus
Journal:  Ann Noninvasive Electrocardiol       Date:  2019-07-25       Impact factor: 1.468

2.  Brugada syndrome in a young patient with type 1 myotonic dystrophy requiring an implantable cardioverter defibrillator for primary prevention: a case report.

Authors:  Panagiotis Korantzopoulos; Aris Bechlioulis; Lampros Lakkas; Katerina K Naka
Journal:  Eur Heart J Case Rep       Date:  2019-06-01
  2 in total

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