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Brugada syndrome in children - Stepping into unchartered territory


1 Nemours Cardiac Center, Nemours/Alfred I duPont Hospital for Children, Wilmington, DE, USA
2 The Pediatric Specialty Clinic, Overland Park Regional Medical Center, Overland Park, KS 66215, USA

Correspondence Address:
Shashank P Behere
Nemours Cardiac Center, Nemours/Alfred I duPont Hospital for Children, Wilmington, DE 19809
USA
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/apc.APC_49_17

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Year : 2017  |  Volume : 10  |  Issue : 3  |  Page : 248-258

 

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Brugada syndrome (BrS) is an autosomal dominant inherited channelopathy. It is associated with a typical pattern of ST-segment elevation in the precordial leads V1–V3 and potentially lethal ventricular arrhythmias in otherwise healthy patients. It is frequently seen in young Asian males, in whom it has previously been described as sudden unexplained nocturnal death syndrome. Although it typically presents in young adults, it is also known to present in children and infants, especially in the presence of fever. Our understanding of the genetic pathogenesis and management of BrS has grown substantially considering that it has only been 24 years since its first description as a unique clinical entity. However, there remains much to be learned, especially in the pediatric population. This review aims to discuss the epidemiology, genetics, and pathogenesis of BrS. We will also discuss established standards and new innovations in the diagnosis, prognostication, risk stratification, and management of BrS. Literature search was run on the National Center for Biotechnology Information's website, using the Medical Subject Headings (MeSH) database with the search term “Brugada Syndrome” (MeSH), and was run on the PubMed database using the age filter (birth–18 years), yielding 334 results. The abstracts of all these articles were studied, and the articles were categorized and organized. Articles of relevance were read in full. As and where applicable, relevant references and citations from the primary articles were further explored and read in full.






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1 Nemours Cardiac Center, Nemours/Alfred I duPont Hospital for Children, Wilmington, DE, USA
2 The Pediatric Specialty Clinic, Overland Park Regional Medical Center, Overland Park, KS 66215, USA

Correspondence Address:
Shashank P Behere
Nemours Cardiac Center, Nemours/Alfred I duPont Hospital for Children, Wilmington, DE 19809
USA
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/apc.APC_49_17

Rights and Permissions

Brugada syndrome (BrS) is an autosomal dominant inherited channelopathy. It is associated with a typical pattern of ST-segment elevation in the precordial leads V1–V3 and potentially lethal ventricular arrhythmias in otherwise healthy patients. It is frequently seen in young Asian males, in whom it has previously been described as sudden unexplained nocturnal death syndrome. Although it typically presents in young adults, it is also known to present in children and infants, especially in the presence of fever. Our understanding of the genetic pathogenesis and management of BrS has grown substantially considering that it has only been 24 years since its first description as a unique clinical entity. However, there remains much to be learned, especially in the pediatric population. This review aims to discuss the epidemiology, genetics, and pathogenesis of BrS. We will also discuss established standards and new innovations in the diagnosis, prognostication, risk stratification, and management of BrS. Literature search was run on the National Center for Biotechnology Information's website, using the Medical Subject Headings (MeSH) database with the search term “Brugada Syndrome” (MeSH), and was run on the PubMed database using the age filter (birth–18 years), yielding 334 results. The abstracts of all these articles were studied, and the articles were categorized and organized. Articles of relevance were read in full. As and where applicable, relevant references and citations from the primary articles were further explored and read in full.






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