ARVD4: 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. This variant of ARVD is somewhat unusual as some family members were found to have involvement of the left ventricle and [[left bundle branch block]]. | |||
==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]]. | 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]]. This variant of ARVD is somewhat unusual as some family members were found to have involvement of the left ventricle and [[left bundle branch block]]. | ||
===Genetics=== | ===Genetics=== | ||
This variant ([http://omim.org/entry/602087 602087]) is associated with a mutation in the chromosome 2q32.1-q32.3 region.<ref>Rampazzo A, Nava A, Miorin M et-al. ARVD4, a new locus for arrhythmogenic right ventricular cardiomyopathy, maps to chromosome 2 long arm. Genomics. 1997;45 (2): 259-63. {{doi|10.1006/geno.1997.4927}} - [http://www.ncbi.nlm.nih.gov/pubmed/9344647 Pubmed citation]</ref> | |||
==Epidemiology and Demographics== | ==Epidemiology and Demographics== | ||
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===Electrocardiogram=== | ===Electrocardiogram=== | ||
This variant of ARVD is somewhat unusual as some family members were found to have involvement of the left ventricle and [[left bundle branch block]]. | |||
===Echocardiogram=== | ===Echocardiogram=== | ||
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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 00:30, 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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ARVD4 On the Web |
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American Roentgen Ray Society Images of ARVD4 |
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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 4; arrhythmogenic right ventricular cardiomyopathy 4; ARVC4
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. This variant of ARVD is somewhat unusual as some family members were found to have involvement of the left ventricle and left bundle branch block.
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. This variant of ARVD is somewhat unusual as some family members were found to have involvement of the left ventricle and left bundle branch block.
Genetics
This variant (602087) is associated with a mutation in the chromosome 2q32.1-q32.3 region.[1]
Epidemiology and Demographics
Natural History, Complications, Prognosis
Diagnosis
Symptoms
Electrocardiogram
This variant of ARVD is somewhat unusual as some family members were found to have involvement of the left ventricle and left bundle branch block.
Echocardiogram
MRI
References
- ↑ Rampazzo A, Nava A, Miorin M et-al. ARVD4, a new locus for arrhythmogenic right ventricular cardiomyopathy, maps to chromosome 2 long arm. Genomics. 1997;45 (2): 259-63. doi:10.1006/geno.1997.4927 - Pubmed citation