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Difference between revisions of "Cardiomyopathy"
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==Dilated cardiomyopathy== | ==Dilated cardiomyopathy== | ||
*Abbreviated ''DCM''. | |||
===General=== | ===General=== | ||
*Most common of the cardiomyopathies. | *Most common of the cardiomyopathies. | ||
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*Myocarditis - leading cause, usually viral.<ref name=pmid19017683>{{cite journal |author=Luk A, Ahn E, Soor GS, Butany J |title=Dilated cardiomyopathy: a review |journal=J. Clin. Pathol. |volume=62 |issue=3 |pages=219–25 |year=2009 |month=March |pmid=19017683 |doi=10.1136/jcp.2008.060731 |url=}}</ref> | *Myocarditis - leading cause, usually viral.<ref name=pmid19017683>{{cite journal |author=Luk A, Ahn E, Soor GS, Butany J |title=Dilated cardiomyopathy: a review |journal=J. Clin. Pathol. |volume=62 |issue=3 |pages=219–25 |year=2009 |month=March |pmid=19017683 |doi=10.1136/jcp.2008.060731 |url=}}</ref> | ||
*Familial ~ 30% - can be AD with variable penetrance, AR, X-linked. | *Familial ~ 30% - can be AD with variable penetrance, AR, X-linked. | ||
*In the [[forensic pathology|forensic]] context, usually caused by alcoholism.<ref name=Ref_HoFP43>{{Ref HoFP|43}}</ref> | *In the [[forensic pathology|forensic]] context, usually caused by [[alcoholism]].<ref name=Ref_HoFP43>{{Ref HoFP|43}}</ref> | ||
===Microscopic=== | ===Microscopic=== | ||
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Multiple causes - an incomplete list:<ref name=Ref_HoFP44-5>{{Ref HoFP|44-5}}</ref> | Multiple causes - an incomplete list:<ref name=Ref_HoFP44-5>{{Ref HoFP|44-5}}</ref> | ||
*[[Hemochromatosis]] - rare.<ref name=pmid7446557>{{Cite journal | last1 = Cutler | first1 = DJ. | last2 = Isner | first2 = JM. | last3 = Bracey | first3 = AW. | last4 = Hufnagel | first4 = CA. | last5 = Conrad | first5 = PW. | last6 = Roberts | first6 = WC. | last7 = Kerwin | first7 = DM. | last8 = Weintraub | first8 = AM. | title = Hemochromatosis heart disease: an unemphasized cause of potentially reversible restrictive cardiomyopathy. | journal = Am J Med | volume = 69 | issue = 6 | pages = 923-8 | month = Dec | year = 1980 | doi = | PMID = 7446557 }}</ref> | *[[Hemochromatosis]] - rare.<ref name=pmid7446557>{{Cite journal | last1 = Cutler | first1 = DJ. | last2 = Isner | first2 = JM. | last3 = Bracey | first3 = AW. | last4 = Hufnagel | first4 = CA. | last5 = Conrad | first5 = PW. | last6 = Roberts | first6 = WC. | last7 = Kerwin | first7 = DM. | last8 = Weintraub | first8 = AM. | title = Hemochromatosis heart disease: an unemphasized cause of potentially reversible restrictive cardiomyopathy. | journal = Am J Med | volume = 69 | issue = 6 | pages = 923-8 | month = Dec | year = 1980 | doi = | PMID = 7446557 }}</ref> | ||
**Hemochromatosis more commonly causes a [[DCM]]. | |||
*[[amyloidosis#Cardiac_amyloidosis|Amyloidosis]]. | *[[amyloidosis#Cardiac_amyloidosis|Amyloidosis]]. | ||
**Classically described as "stiff" or "rubbery". | **Classically described as "stiff" or "rubbery". | ||
*[[Sarcoidosis]]. | *[[Cardiac sarcoidosis|Sarcoidosis]]. | ||
*Storage diseases (e.g. [[Pompe disease]]). | *Storage diseases (e.g. [[Pompe disease]]). | ||
*Eosinophilic endocarditis (Loeffler Endocarditis). | *Eosinophilic endocarditis (Loeffler Endocarditis). | ||
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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> | |||
*Right ventricular 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. | |||
Note: | |||
*† May involve the left ventricle.<ref name=pmid23761986>{{cite journal |author=Romero J, Mejia-Lopez E, Manrique C, Lucariello R |title=Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC/D): A Systematic Literature Review |journal=Clin Med Insights Cardiol |volume=7 |issue= |pages=97–114 |year=2013 |pmid=23761986 |pmc=3667685 |doi=10.4137/CMC.S10940 |url=}}</ref> | |||
===Microscopic=== | ===Microscopic=== | ||
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*Myocytes have "bubbly" appearance with loss of myofibres and cross-striations. | *Myocytes have "bubbly" appearance with loss of myofibres and cross-striations. | ||
Images | ====Images==== | ||
<gallery> | |||
Image: Arrhythmogenic right ventricular cardiomyopathy - histology.jpg | ARVC. (WC) | |||
</gallery> | |||
=====www===== | |||
*[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== | ||
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*Prominent "mesh-like" [[trabeculae]] carnae. | *Prominent "mesh-like" [[trabeculae]] carnae. | ||
**Enlarged intertrabecular recesses.<ref name=pmid2372897>{{cite journal |author=Chin TK, Perloff JK, Williams RG, Jue K, Mohrmann R |title=Isolated noncompaction of left ventricular myocardium. A study of eight cases |journal=Circulation |volume=82 |issue=2 |pages=507–13 |year=1990 |month=August |pmid=2372897 |doi= |url=}}</ref> | **Enlarged intertrabecular recesses.<ref name=pmid2372897>{{cite journal |author=Chin TK, Perloff JK, Williams RG, Jue K, Mohrmann R |title=Isolated noncompaction of left ventricular myocardium. A study of eight cases |journal=Circulation |volume=82 |issue=2 |pages=507–13 |year=1990 |month=August |pmid=2372897 |doi= |url=}}</ref> | ||
==Takotsubo cardiomyopathy== | |||
{{Main|Takotsubo cardiomyopathy}} | |||
*Also known as ''broken heart syndrome''. | |||
==See also== | ==See also== | ||