ARVD6: Difference between revisions
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{{SK}} Arrhythmogenic right ventricular dysplasia type 6; | {{SK}} Arrhythmogenic right ventricular dysplasia type 6; arrhythmogenic right ventricular cardiomyopathy 6; ARVC6 | ||
==Overview== | ==Overview== | ||
Arrhythmogenic right ventricular dysplasia is a type of nonischemic [[cardiomyopathy]] that involves primarily the [[right ventricle]]. It is characterized by hypokinetic areas involving the free wall of the right ventricle, with fibrofatty replacement of the right ventricular [[myocardium]], with associated [[arrhythmia]]s originating in the right ventricle. | |||
==Pathophysiology== | ==Pathophysiology== | ||
The pathogenesis of ARVD involves [[apoptosis]] with fatty and fibro-fatty infiltration of the right ventricular free wall leading to [[heart failure]] and [[ventricular arrhythmias]]. | |||
===Genetics=== | ===Genetics=== | ||
This variant ([http://omim.org/entry/604401 604401]), is associated with a mutation in the chromosome 10p14-p12 region.<ref>Li D, Ahmad F, Gardner MJ et-al. The locus of a novel gene responsible for arrhythmogenic right-ventricular dysplasia characterized by early onset and high penetrance maps to chromosome 10p12-p14. Am. J. Hum. Genet. 2000;66 (1): 148-56. {{doi|10.1086/302713}} - [http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1288320 Free text at pubmed] - [http://www.ncbi.nlm.nih.gov/pubmed/10631146 Pubmed citation]</ref> | |||
==Epidemiology and Demographics== | ==Epidemiology and Demographics== | ||
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==References== | ==References== | ||
{{Reflist|2}} | {{Reflist|2}} | ||
[[CME Category::Cardiology]] | |||
[[Category:Cardiology]] | [[Category:Cardiology]] | ||
[[Category:Electrophysiology]] | [[Category:Electrophysiology]] | ||
Latest revision as of 00:31, 15 March 2016
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Arrhythmogenic right ventricular dysplasia Microchapters |
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Differentiating Arrhythmogenic right ventricular dysplasia from other Diseases |
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Diagnosis |
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Treatment |
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ARVD6 On the Web |
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American Roentgen Ray Society Images of ARVD6 |
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Directions to Hospitals Treating Arrhythmogenic right ventricular dysplasia |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Synonyms and keywords: Arrhythmogenic right ventricular dysplasia type 6; arrhythmogenic right ventricular cardiomyopathy 6; ARVC6
Overview
Arrhythmogenic right ventricular dysplasia is a type of nonischemic cardiomyopathy that involves primarily the right ventricle. It is characterized by hypokinetic areas involving the free wall of the right ventricle, with fibrofatty replacement of the right ventricular myocardium, with associated arrhythmias originating in the right ventricle.
Pathophysiology
The pathogenesis of ARVD involves apoptosis with fatty and fibro-fatty infiltration of the right ventricular free wall leading to heart failure and ventricular arrhythmias.
Genetics
This variant (604401), is associated with a mutation in the chromosome 10p14-p12 region.[1]
Epidemiology and Demographics
Natural History, Complications, Prognosis
Diagnosis
Symptoms
Electrocardiogram
Echocardiogram
MRI
References
- ↑ Li D, Ahmad F, Gardner MJ et-al. The locus of a novel gene responsible for arrhythmogenic right-ventricular dysplasia characterized by early onset and high penetrance maps to chromosome 10p12-p14. Am. J. Hum. Genet. 2000;66 (1): 148-56. doi:10.1086/302713 - Free text at pubmed - Pubmed citation