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 Inherited arrhythmias: The cardiac channelopathies

Shashank P Behere1, Steven N Weindling2
1 Department of Pediatrics, Dartmouth Hitchcock Medical Center, Lebanon, New Hampshire, USA
2 Department of Pediatric Cardiology, Dartmouth Hitchcock Medical Center, Lebanon, New Hampshire, USA

Correspondence Address:
Shashank P Behere
Department of Pediatrics, Dartmouth Hitchcock Medical Center, Lebanon, New Hampshire 03766
USA
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0974-2069.164695

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Year : 2015  |  Volume : 8  |  Issue : 3  |  Page : 210-220

 
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Ion channels in the myocardial cellular membrane are responsible for allowing the cardiac action potential. Genetic abnormalities in these channels can predispose to life-threatening arrhythmias. We discuss the basic science of the cardiac action potential; outline the different clinical entities, including information regarding overlapping diagnoses, touching upon relevant genetics, new innovations in screening, diagnosis, risk stratification, and management. The special considerations of sudden unexplained death and sudden infant death syndrome are discussed. Scientists and clinicians continue to reconcile the rapidly growing body of knowledge regarding the molecular mechanisms and genetics while continuing to improve our understanding of the various clinical entities and their diagnosis and management in clinical setting. Two separate searches were run on the National Center for Biotechnology Information's website. The first using the term cardiac channelopathies was run on the PubMed database using filters for time (published in past 5 years) and age (birth-18 years), yielding 47 results. The second search using the medical subject headings (MeSH) database with the search terms "Long QT Syndrome" (MeSH) and "Short QT Syndrome" (MeSH) and "Brugada Syndrome" (MeSH) and "Catecholaminergic Polymorphic Ventricular Tachycardia" (MeSH), applying the same filters yielded 467 results. The abstracts of 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 where further explored and read in full.






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 © 2008 Annals of Pediatric Cardiology | Published by Wolters Kluwer - Medknow
 Online since 20th April, 2008

 

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Shashank P Behere1, Steven N Weindling2
1 Department of Pediatrics, Dartmouth Hitchcock Medical Center, Lebanon, New Hampshire, USA
2 Department of Pediatric Cardiology, Dartmouth Hitchcock Medical Center, Lebanon, New Hampshire, USA

Correspondence Address:
Shashank P Behere
Department of Pediatrics, Dartmouth Hitchcock Medical Center, Lebanon, New Hampshire 03766
USA
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0974-2069.164695

Rights and Permissions

Ion channels in the myocardial cellular membrane are responsible for allowing the cardiac action potential. Genetic abnormalities in these channels can predispose to life-threatening arrhythmias. We discuss the basic science of the cardiac action potential; outline the different clinical entities, including information regarding overlapping diagnoses, touching upon relevant genetics, new innovations in screening, diagnosis, risk stratification, and management. The special considerations of sudden unexplained death and sudden infant death syndrome are discussed. Scientists and clinicians continue to reconcile the rapidly growing body of knowledge regarding the molecular mechanisms and genetics while continuing to improve our understanding of the various clinical entities and their diagnosis and management in clinical setting. Two separate searches were run on the National Center for Biotechnology Information's website. The first using the term cardiac channelopathies was run on the PubMed database using filters for time (published in past 5 years) and age (birth-18 years), yielding 47 results. The second search using the medical subject headings (MeSH) database with the search terms "Long QT Syndrome" (MeSH) and "Short QT Syndrome" (MeSH) and "Brugada Syndrome" (MeSH) and "Catecholaminergic Polymorphic Ventricular Tachycardia" (MeSH), applying the same filters yielded 467 results. The abstracts of 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 where further explored and read in full.






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 © 2008 Annals of Pediatric Cardiology | Published by Wolters Kluwer - Medknow
 Online since 20th April, 2008