Difference between revisions of "Cardiomyopathy"

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'''Cardiomyopathy''', abbreviated as '''CM''', is a domain of cardiology and [[forensic pathology]] -- as it many cardiomyopathies can lead to sudden death.
'''Cardiomyopathy''', abbreviated as '''CM''', is a domain of cardiology ''and'' [[forensic pathology]], as many cardiomyopathies can lead to sudden death.


==Overview==
==Overview==
Types<ref>Robbins p.601.</ref>
Types<ref name=Ref_PBoD601>{{Ref PBoD|601}}</ref>
#Dilated cardiomyopathy - most common ~ 90%
#Dilated cardiomyopathy - most common ~ 90%
#Hypertrophic cardiomyopathy
#Hypertrophic cardiomyopathy
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==Dilated cardiomyopathy==
==Dilated cardiomyopathy==
*Abbreviated ''DCM''.
===General===
===General===
*Classic cause of sudden death in young athletes.<ref>{{cite journal |author=Gojanovic B, Feihl F, Gremion G, Waeber B |title=[Sudden death in young athletes] |language=German |journal=Praxis (Bern 1994) |volume=96 |issue=6 |pages=189-98 |year=2007 |month=February |pmid=17330410 |doi= |url=}}</ref>
*Most common of the cardiomyopathies.
*Most common of the cardiomyopathies.


Causes:
Causes:
*Myocarditis - leading cause, usually viral.<ref name=pmid19017683>Dilated cardiomyopathy: a review. Luk A, Ahn E, Soor GS, Butany J. J Clin Pathol. 2009 Mar;62(3):219-25. Epub 2008 Nov 18. Review. PMID 19017683. http://jcp.bmjjournals.com/cgi/content/full/62/3/219</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>


===Microscopic===
===Microscopic===
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*Epicardial fibrosis.
*Epicardial fibrosis.
*Usually non-specific.
*Usually non-specific.
DDx:
*[[Mitochondrial myopathy]].
**Perinuclear clearing on light microscopy due to abundant mitochondria.
**Atypical mitochondria on [[electron microscopy]].
*[[Muscular dystrophy]].
*[[Storage disease]].
===IHC===
Work-up for muscular dystrophy:
*Dystrophin.
Work-up for mitochondrial disease:
*COX.
*SDH.


==Hypertrophic cardiomyopathy==
==Hypertrophic cardiomyopathy==
*Abbreviated HCM.
===General===
===General===
*Abbreviated HCM.
*Genetic.
*Classic cause of sudden death in young athletes.<ref name=pmid17330410>{{cite journal |author=Gojanovic B, Feihl F, Gremion G, Waeber B |title=[Sudden death in young athletes] |language=German |journal=Praxis (Bern 1994) |volume=96 |issue=6 |pages=189-98 |year=2007 |month=February |pmid=17330410 |doi= |url=}}</ref>
 
===Gross===
*Classic: septum:left ventricular free wall = 1.5:1.0.<Ref>JB. 9 June 2011.</ref>


===Microscopic===
===Microscopic===
Features:<ref>Robbins pp.601-3.</ref>
Features:<ref name=Ref_PBoD601-3>{{Ref PBoD|601-3}}</ref>
*Myocardial fibre has increased transverse size (40 micrometres).
*Myocardial fibres have increased transverse size (~40 micrometres) - '''key feature'''.
**Normal myocardial fibre width = 15 micrometres.  
**Normal myocardial fibre width = 15 micrometres.  
*Haphazard arrangement of myocardial fibres;<ref name=Ref_HoFP44>{{Ref HoFP|44}}</ref> "basket weave" pattern.
*Interstitial fibrosis.
*Interstitial fibrosis.
**NOT diffuse patch/area as in an old myocardial infarction.
*Large hyperchromatic nuclei (~3x fibroblast nucleus).<ref>CK. 14 October 2010.</ref>


==Hypertrophic obstructive cardiomyopathy==
Notes:
*Easiest to identify if sections are perpendicular to the long axis of the myocytes.
 
Images:
*[http://medicinembbs.blogspot.com/2011/03/hypertrophic-obstructive-cardiomyopathy.html HCM (blogspot.com)].
*[http://medcell.med.yale.edu/histology/muscle/hypertrophic_cardiomyopathy.php HCM (yale.edu)].
 
===Variants===
====Hypertrophic obstructive cardiomyopathy====
*Abbreviated ''HOCM''.
*Considered to be a variant of HCM.
*Considered to be a variant of HCM.
*Historically known as ''idiopathic hypertrophic subaortic stenosis'' (IHSS).
====Apical HCM====
*[[AKA]] ''Japanese variant''.<ref name=pmid17392031>{{Cite journal  | last1 = Reddy | first1 = M. | last2 = Thatai | first2 = D. | last3 = Bernal | first3 = J. | last4 = Pradhan | first4 = J. | last5 = Afonso | first5 = L. | title = Apical hypertrophic cardiomyopathy: potential utility of Strain imaging. | journal = Eur J Echocardiogr | volume = 9 | issue = 4 | pages = 560-2 | month = Jul | year = 2008 | doi = 10.1016/j.euje.2007.02.004 | PMID = 17392031 }}
</ref>
*Mid-ventricular septal thickening ''or'' apical thickening (''NOT'' subaortic hypertrophy).
==Restrictive cardiomyopathy==
*Uncommon form of cardiomyopathy.
===Etiology===
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 more commonly causes a [[DCM]].
*[[amyloidosis#Cardiac_amyloidosis|Amyloidosis]].
**Classically described as "stiff" or "rubbery".
*[[Cardiac sarcoidosis|Sarcoidosis]].
*Storage diseases (e.g. [[Pompe disease]]).
*Eosinophilic endocarditis (Loeffler Endocarditis).


==Arrhythmogenic right ventricular cardiomyopathy==  
==Arrhythmogenic right ventricular cardiomyopathy==  
*Abbreviated ''ARVC''.
*Previously known as ''arrhythmogenic right ventricular dysplasia'', abbreviated ''ARVD''.
===General===
===General===
*Previously known as "arrhythmogenic right ventricular dysplasia".
*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===
Etiology:
*Genetic - mutations in:
*Genetic - mutations in:
**Desmosomal proteins, especially plakoglobin and desmoplakin.
**Desmosomal proteins, especially plakoglobin and desmoplakin.
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**Clinical: wooly hair, palmar & plantar keratoses.
**Clinical: wooly hair, palmar & plantar keratoses.


===Histology===
===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===
Features:<ref name=emedicine1612324/>
Features:<ref name=emedicine1612324/>
*"Moth-eaten" appearance:  
*"Moth-eaten" appearance:  
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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.


Image:  
====Images====
*[http://en.wikipedia.org/wiki/Arrhythmogenic_right_ventricular_dysplasia ARVC micrograph (WP)].
<gallery>
Image: Arrhythmogenic right ventricular cardiomyopathy - histology.jpg | ARVC. (WC)
</gallery>
=====www=====
*[http://path.upmc.edu/cases/case223.html ARVC - several images (upmc.edu)].


===Gross features===
==Noncompaction cardiomyopathy==
Gross:<ref name=emedicine1612324>URL: [http://emedicine.medscape.com/article/1612324-overview http://emedicine.medscape.com/article/1612324-overview]</ref>
===Etiology===
*RV wall thinning/replacement with fat.
*Genetic - ''LVNC1 gene''.<ref name=omim604169>{{OMIM|604169}}</ref>
**Especially fat where fat is ''not'' usually seen - posterior RV wall, RVOT.
*May be associated with dilation.<ref name=omim604169>{{OMIM|604169}}</ref>
*Septum usually has relative sparing
*Rare.
**Thus, endomyocardial biopsy is ''not'' reliable.
*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>
*+/-Aneurysms/dilation.
 
===Gross===
*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>


==See also==
==See also==
Michael
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