Dilated cardiomyopathy pathophysiology
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor-in-Chief: Sachin Shah, M.D.
Overview
Familial traits and mitochondrial inheritance are thought to play a part in the development of idiopathic dilated cardiomyopathy, and the inheritance occurs in an autosomal dominant pattern. Connective tissue disease, and other diseases or toxins that disrupt the tissue of the heart are also implicated in the development of dilated cardiomyopathy.
Pathophysiology
Genetics
A family history also has a great importance in the diagnosis of dilated cardiomyopathy. It has been suggested that a portion of those patients labeled as "idiopathic" may have a familial form of the disease. The prevalence of this in the population of patients with dilated cardiomyopathy has been estimated as high as 25%.[1] The majority of these are thought to be related to autosomal dominant transmission, the remaining are thought to be transimtted in an autosomal recessive and X-linked fashion.[2] Mitochondrial inheritance of the disease has also been identified.[3]
Associated Conditions
A review of systems is also helpful in regards to connective tissue disease associated dilated cardiomyopathy. Some of the disease that can be associated with dilated cardiomyopathy are:
- SLE (systemic lupus erythematosis)
- Rheumatoid arthritis
- Sarcoidosis
- Scleroderma
- Connective tissue diseases
- Pericardial effusion - It may accompany myocarditis but this finding is not specific.
Gross Pathology
Images shown below are Courtesy of Professor Peter Anderson DVM PhD and published with permission. © PEIR, University of Alabama at Birmingham, Department of Pathology
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Cardiomyopathy: Gross excellent view of mitral valve from left atrium anterior leaflet appears to balloon a bit into the atrium
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Cardiomyopathy: Gross excellent view of mitral and tricuspid valves from atria, appear normal anatomy.
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Dilated Cardiomyopathy: Gross natural color close-up view of heart surgically removed for a transplantation shows aortic valve and anterior leaflet of mitral valve with cholesterol deposits endocardium of left ventricle is diffusely thickened
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Cardiomyopathy: Gross external view of globular heart with patchy fibrosis seen through epicardium
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Cardiomyopathy: Gross dilated left ventricle with marked endocardial thickening this is what has been called adult fibroelastosis
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Dilated Cardiomyopathy: Gross good example huge dilated left ventricle
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Dilated Cardiomyopathy: Gross dilated left ventricle with marked endocardial sclerosis (an excellent example)
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Cardiomyopathy: Gross intact globular shaped heart
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Dilated Cardiomyopathy: Gross opened left ventricle dilated with endocardial thickening good example
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Cardiomyopathy: Gross globular heart external view 10 year old girl with sickle cell anemia
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Cardiomyopathy: Gross horizontal sections of ventricles dilation type 10 year old girl with sickle cell anemia
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Cardiomyopathy: Intermediate between hypertrophic and dilated
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Dilated Cardiomyopathy: Gross opened globular left ventricle natural color (very good example)
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Dilated Cardiomyopathy: Gross natural color external view globular heart 500 gm 24yo female seven pregnancies
References
- ↑ Ross J Jr. Dilated cardiomyopathy: concepts derived from gene deficient and transgenic animal models. Circ J. 2002;66:219-24. PMID 11922267
- ↑ Mestroni L; Rocco C; et al. Familial dilated cardiomyopathy: evidence for genetic and phenotypic heterogeneity. Heart Muscle Disease Study Group. J Am Coll Cardiol 1999 Jul;34(1):181-90.
- ↑ Schonberger J, Seidman CE. Many roads lead to a broken heart: the genetics of dilated cardiomyopathy. Am J Hum Genet. 2001;69:249-60. Epub 2001 Jul 6. PMID 11443548