ARVD10: Difference between revisions
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==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/610193 (610193]) is associated with a mutation in the [[DSG2]] gene ([http://omim.org/entry/125671 125671]) on chromosome 18q12.1-q12.<ref>Pilichou K, Nava A, Basso C et-al. Mutations in desmoglein-2 gene are associated with arrhythmogenic right ventricular cardiomyopathy. Circulation. 2006;113 (9): 1171-9. {{doi|10.1161/CIRCULATIONAHA.105.583674}} - [http://www.ncbi.nlm.nih.gov/pubmed/16505173 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 00:27, 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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ARVD10 On the Web |
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American Roentgen Ray Society Images of ARVD10 |
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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 10; arrhythmogenic right ventricular cardiomyopathy 10; ARVC10
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 ((610193) is associated with a mutation in the DSG2 gene (125671) on chromosome 18q12.1-q12.[1]
Epidemiology and Demographics
Natural History, Complications, Prognosis
Diagnosis
Symptoms
Electrocardiogram
Echocardiogram
MRI
References
- ↑ Pilichou K, Nava A, Basso C et-al. Mutations in desmoglein-2 gene are associated with arrhythmogenic right ventricular cardiomyopathy. Circulation. 2006;113 (9): 1171-9. doi:10.1161/CIRCULATIONAHA.105.583674 - Pubmed citation