Brugada Syndrome in a Patient with Vascular Ehlers-Danlos Syndrome: Sudden Death Risk Amplified.

MedStar author(s):
Citation: Cureus. 9(4):e1178, 2017 Apr 19PMID: 28536668Institution: Medstar Franklin Square Medical CenterDepartment: MedicineForm of publication: Journal ArticleMedline article type(s): Journal ArticleSubject headings: PubMed-not-MEDLINE -- Not indexedYear: 2017ISSN:
  • 2168-8184
Name of journal: CureusAbstract: The vast majority of sudden cardiac arrests occur in patients with structural heart disease and in approximately 10% of the cases, it can occur in those with structurally normal hearts. Brugada syndrome is an autosomal dominant sodium channelopathy that has been implicated in sudden deaths. Given their low prevalence, our knowledge about Brugada syndrome is still evolving. Apart from schizophrenia, there have been no reports of associated medical conditions. We recently encountered a patient with vascular Ehlers-Danlos syndrome who was also found to have Brugada syndrome. Both these conditions share some common clinical presentations including a propensity for sudden death.All authors: D'Souza J, Dahagam C, Everett G, Goud A, Malhotra DFiscal year: FY2017Digital Object Identifier: Date added to catalog: 2017-05-26
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Journal Article MedStar Authors Catalog Article 28536668 Available 28536668

The vast majority of sudden cardiac arrests occur in patients with structural heart disease and in approximately 10% of the cases, it can occur in those with structurally normal hearts. Brugada syndrome is an autosomal dominant sodium channelopathy that has been implicated in sudden deaths. Given their low prevalence, our knowledge about Brugada syndrome is still evolving. Apart from schizophrenia, there have been no reports of associated medical conditions. We recently encountered a patient with vascular Ehlers-Danlos syndrome who was also found to have Brugada syndrome. Both these conditions share some common clinical presentations including a propensity for sudden death.

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