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Difference between revisions of "Cardiomyopathy"
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→Arrhythmogenic right ventricular cardiomyopathy: re-order
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==Arrhythmogenic right ventricular cardiomyopathy== | ==Arrhythmogenic right ventricular cardiomyopathy== | ||
*Abbreviated ''ARVC''. | *Abbreviated ''ARVC''. | ||
*Previously known as ''arrhythmogenic right ventricular dysplasia'', abbreviated ''ARVD''. | |||
===General=== | ===General=== | ||
*Associated with sudden cardiac death in "young people".<ref>Sudden cardiac death due to hypertrophic cardiomyopathy can be reduced by pre-participation cardiovascular screening in young athletes. URL: [http://eurheartj.oxfordjournals.org/cgi/content/full/27/18/2152 http://eurheartj.oxfordjournals.org/cgi/content/full/27/18/2152]. Accessed on: 16 December 2009.</ref> | *Associated with sudden cardiac death in "young people".<ref>Sudden cardiac death due to hypertrophic cardiomyopathy can be reduced by pre-participation cardiovascular screening in young athletes. URL: [http://eurheartj.oxfordjournals.org/cgi/content/full/27/18/2152 http://eurheartj.oxfordjournals.org/cgi/content/full/27/18/2152]. Accessed on: 16 December 2009.</ref> | ||
*Male > female. | *Male > female. | ||
Etiology: | |||
*Genetic - mutations in: | *Genetic - mutations in: | ||
**Desmosomal proteins, especially plakoglobin and desmoplakin. | **Desmosomal proteins, especially plakoglobin and desmoplakin. | ||
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*Autosomal recessive variant: Naxos syndrome.<ref>[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=601214 http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=601214]</ref> | *Autosomal recessive variant: Naxos syndrome.<ref>[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=601214 http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=601214]</ref> | ||
**Clinical: wooly hair, palmar & plantar keratoses. | **Clinical: wooly hair, palmar & plantar keratoses. | ||
===Gross=== | |||
Features:<ref name=emedicine1612324>URL: [http://emedicine.medscape.com/article/1612324-overview http://emedicine.medscape.com/article/1612324-overview].</ref> | |||
*RV wall thinning/replacement with fat. | |||
**Especially fat where fat is ''not'' usually seen - posterior RV wall, RVOT. | |||
*Septum usually has relative sparing | |||
**Thus, endomyocardial biopsy is ''not'' reliable. | |||
*+/-Aneurysms/dilation. | |||
===Microscopic=== | ===Microscopic=== | ||
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*[http://en.wikipedia.org/wiki/Arrhythmogenic_right_ventricular_dysplasia ARVC micrograph (WP)]. | *[http://en.wikipedia.org/wiki/Arrhythmogenic_right_ventricular_dysplasia ARVC micrograph (WP)]. | ||
*[http://path.upmc.edu/cases/case223.html ARVC - several images (upmc.edu)]. | *[http://path.upmc.edu/cases/case223.html ARVC - several images (upmc.edu)]. | ||
==Noncompaction cardiomyopathy== | ==Noncompaction cardiomyopathy== | ||