Difference between revisions of "Cardiomyopathy"
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*Considered to be a variant of HCM. | *Considered to be a variant of HCM. | ||
*Historically known as ''idiopathic hypertrophic subaortic stenosis'' (IHSS). | *Historically known as ''idiopathic hypertrophic subaortic stenosis'' (IHSS). | ||
==Restrictive cardiomyopathy== | |||
*Uncommon form of cardiomyopathy. | |||
===Etiology=== | |||
Multiple causes - an incomplete list:<ref name=Ref_HoFP44-5>{{Ref HoFP|44-5}}</ref> | |||
*[[Hemochromatosis]]. | |||
*[[Amyloidosis]]. | |||
*[[Sarcoidosis]]. | |||
*Storage diseases (Pompe disease). | |||
*Eosinophilic endocarditis (Loeffler Endocarditis). | |||
==Arrhythmogenic right ventricular cardiomyopathy== | ==Arrhythmogenic right ventricular cardiomyopathy== | ||
| Line 71: | Line 82: | ||
**Thus, endomyocardial biopsy is ''not'' reliable. | **Thus, endomyocardial biopsy is ''not'' reliable. | ||
*+/-Aneurysms/dilation. | *+/-Aneurysms/dilation. | ||
==Noncompaction cardiomyopathy== | ==Noncompaction cardiomyopathy== | ||
Revision as of 04:26, 22 September 2010
Cardiomyopathy, abbreviated as CM, is a domain of cardiology and forensic pathology, as many cardiomyopathies can lead to sudden death.
Overview
Types[1]
- Dilated cardiomyopathy - most common ~ 90%
- Hypertrophic cardiomyopathy
- Restrictive cardiomyopathy - least common
Note: The frequency of the CMs is in alphabetic order dilated, hypertrophic, restrictive.
Dilated cardiomyopathy
General
- Classic cause of sudden death in young athletes.[2]
- Most common of the cardiomyopathies.
Causes:
- Myocarditis - leading cause, usually viral.[3]
- Familial ~ 30% - can be AD with variable penetrance, AR, X-linked.
- In the forensic context, usually caused by alcoholism.[4]
Microscopic
Features:
- Epicardial fibrosis.
- Usually non-specific.
Hypertrophic cardiomyopathy
General
- Abbreviated HCM.
Microscopic
Features:[5]
- Myocardial fibres have increased transverse size (40 micrometres) - key feature.
- Normal myocardial fibre width = 15 micrometres.
- Haphazard arrangement of myocardial fibres.[6]
- Interstitial fibrosis.
Hypertrophic obstructive cardiomyopathy
- Considered to be a variant of HCM.
- Historically known as idiopathic hypertrophic subaortic stenosis (IHSS).
Restrictive cardiomyopathy
- Uncommon form of cardiomyopathy.
Etiology
Multiple causes - an incomplete list:[7]
- Hemochromatosis.
- Amyloidosis.
- Sarcoidosis.
- Storage diseases (Pompe disease).
- Eosinophilic endocarditis (Loeffler Endocarditis).
Arrhythmogenic right ventricular cardiomyopathy
General
- Previously known as "arrhythmogenic right ventricular dysplasia".
- Associated with sudden cardiac death in "young people".[8]
- Male > female.
Etiology
- Genetic - mutations in:
- Desmosomal proteins, especially plakoglobin and desmoplakin.
- Usually autosomal dominant.
- Autosomal recessive variant: Naxos syndrome.[9]
- Clinical: wooly hair, palmar & plantar keratoses.
Histology
Features:[10]
- "Moth-eaten" appearance:
- Loss of myocytes, replaced by:
- Fat and/or
- Scar tissue.
- Loss of myocytes, replaced by:
- +/-Inflammation (lymphocytes, macrophages).
- Myocytes have "bubbly" appearance with loss of myofibres and cross-striations.
Image:
Gross features
Gross:[10]
- 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.
Noncompaction cardiomyopathy
Etiology
- Genetic.[11]
- May be associated with dilation.[12]
- Rare.
- Not clear whether it is a unique entity.[13]
Gross
- Prominent "mesh-like" trabeculae carnae.
- Enlarged intertrabecular recesses.[14]
See also
- Heart.
- Cardiac sarcoidosis.
- Amyloidosis - covers cardiac amyloidosis.
References
- ↑ Cotran, Ramzi S.; Kumar, Vinay; Fausto, Nelson; Nelso Fausto; Robbins, Stanley L.; Abbas, Abul K. (2005). Robbins and Cotran pathologic basis of disease (7th ed.). St. Louis, Mo: Elsevier Saunders. pp. 601. ISBN 0-7216-0187-1.
- ↑ Gojanovic B, Feihl F, Gremion G, Waeber B (February 2007). "[Sudden death in young athletes]" (in German). Praxis (Bern 1994) 96 (6): 189-98. PMID 17330410.
- ↑ Luk A, Ahn E, Soor GS, Butany J (March 2009). "Dilated cardiomyopathy: a review". J. Clin. Pathol. 62 (3): 219–25. doi:10.1136/jcp.2008.060731. PMID 19017683.
- ↑ DiMaio, Vincent J.M.; Dana, Suzanna E. (2006). Handbook of Forensic Pathology (2nd ed.). CRC Press. pp. 43. ISBN 978-0849392870.
- ↑ Cotran, Ramzi S.; Kumar, Vinay; Fausto, Nelson; Nelso Fausto; Robbins, Stanley L.; Abbas, Abul K. (2005). Robbins and Cotran pathologic basis of disease (7th ed.). St. Louis, Mo: Elsevier Saunders. pp. 601-3. ISBN 0-7216-0187-1.
- ↑ DiMaio, Vincent J.M.; Dana, Suzanna E. (2006). Handbook of Forensic Pathology (2nd ed.). CRC Press. pp. 44. ISBN 978-0849392870.
- ↑ DiMaio, Vincent J.M.; Dana, Suzanna E. (2006). Handbook of Forensic Pathology (2nd ed.). CRC Press. pp. 44-5. ISBN 978-0849392870.
- ↑ 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. Accessed on: 16 December 2009.
- ↑ http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=601214
- ↑ 10.0 10.1 URL: http://emedicine.medscape.com/article/1612324-overview.
- ↑ URL: http://www.ncbi.nlm.nih.gov/omim/604169. Accessed on: 19 August 2010.
- ↑ URL: http://www.ncbi.nlm.nih.gov/omim/601493. Accessed on: 19 August 2010.
- ↑ Paterick TE, Gerber TC, Pradhan SR, Lindor NM, Tajik AJ (2010). "Left ventricular noncompaction cardiomyopathy: what do we know?". Rev Cardiovasc Med 11 (2): 92–9. PMID 20700091.
- ↑ Chin TK, Perloff JK, Williams RG, Jue K, Mohrmann R (August 1990). "Isolated noncompaction of left ventricular myocardium. A study of eight cases". Circulation 82 (2): 507–13. PMID 2372897.