48,702
edits
(→Hypertrophic cardiomyopathy: fixt) |
|||
(10 intermediate revisions by the same user not shown) | |||
Line 10: | Line 10: | ||
==Dilated cardiomyopathy== | ==Dilated cardiomyopathy== | ||
*Abbreviated ''DCM''. | |||
===General=== | ===General=== | ||
*Most common of the cardiomyopathies. | *Most common of the cardiomyopathies. | ||
Line 16: | Line 17: | ||
*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=== | ||
Line 24: | Line 25: | ||
DDx: | DDx: | ||
*Mitochondrial myopathy. | *[[Mitochondrial myopathy]]. | ||
**Perinuclear clearing on light microscopy due to abundant mitochondria. | **Perinuclear clearing on light microscopy due to abundant mitochondria. | ||
**Atypical mitochondria on [[electron microscopy]]. | **Atypical mitochondria on [[electron microscopy]]. | ||
*Muscular dystrophy. | *[[Muscular dystrophy]]. | ||
*[[Storage disease]]. | *[[Storage disease]]. | ||
Line 79: | Line 80: | ||
===Etiology=== | ===Etiology=== | ||
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]]. | *[[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 ([[Pompe disease]]). | *Storage diseases (e.g. [[Pompe disease]]). | ||
*Eosinophilic endocarditis (Loeffler Endocarditis). | *Eosinophilic endocarditis (Loeffler Endocarditis). | ||
==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. | ||
Line 99: | Line 101: | ||
*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=== | ||
Line 109: | Line 122: | ||
*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== | ||
===Etiology=== | ===Etiology=== | ||
*Genetic.<ref> | *Genetic - ''LVNC1 gene''.<ref name=omim604169>{{OMIM|604169}}</ref> | ||
*May be associated with dilation.<ref> | *May be associated with dilation.<ref name=omim604169>{{OMIM|604169}}</ref> | ||
*Rare. | *Rare. | ||
*Not clear whether it is a unique entity.<ref>{{cite journal |author=Paterick TE, Gerber TC, Pradhan SR, Lindor NM, Tajik AJ |title=Left ventricular noncompaction cardiomyopathy: what do we know? |journal=Rev Cardiovasc Med |volume=11 |issue=2 |pages=92–9 |year=2010 |pmid=20700091 |doi= |url=}}</ref> | *Not clear whether it is a unique entity.<ref name=pmid20700091>{{cite journal |author=Paterick TE, Gerber TC, Pradhan SR, Lindor NM, Tajik AJ |title=Left ventricular noncompaction cardiomyopathy: what do we know? |journal=Rev Cardiovasc Med |volume=11 |issue=2 |pages=92–9 |year=2010 |pmid=20700091 |doi= |url=}}</ref> | ||
===Gross=== | ===Gross=== |
edits