Dilated cardiomyopathy

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Dilated cardiomyopathy
Dilated Cardiomyopathy: Gross dilated left ventricle with marked endocardial sclerosis
Image courtesy of Professor Peter Anderson DVM PhD and published with permission © PEIR, University of Alabama at Birmingham, Department of Pathology
ICD-10 I42.0
ICD-9 425.4
OMIM 212110
DiseasesDB 3066
MedlinePlus 000168
MeSH D002311

Dilated cardiomyopathy Microchapters

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor-in-Chief: Sachin Shah, M.D.

Synonyms and keywords: DCM; congestive cardiomyopathy

Overview

Causes

Clinical Presentation

Diagnosis

The diagnosis of dilated cardiomyopathy is based on clinical presentation and imaging findings. The most common imaging modality used to diagnose dilated cardiomyopathy is 2D-echocardiography. Echocardiographic findings of dilated cardiomyopathy include dilation of the left ventricle; however, may include dilation of all 4 cardiac chambers, LV (left ventricular) wall thickness usually is normal but given the dilation the LV mass is increased. In addition there is a global reduciton in systolic function. Occasionally there may also be focal wall motion abnormalities even in patients without flow limiting coronary artery disease.[1]

The diagnosis requires a dilated left ventricle and low ejection fraction.

In terms of determining the etiology a careful history is most instrumental. If the patient has CAD (coronary artery disease) risk factors, known CAD, or angina then a workup for CAD should be undertaken with coronary angiography. A viral prodrome such as viral URI or viral gastroenteritis may make viral myocarditis as a more likely cause. If the patient was exposed to chemotherapy such as anthracyclines then this would be the likely cause. Patients at risk for HIV should undergo testing as HIV can cause a dilated cardiomyopathy. Peripartum cardiomyopathy most often occurs 1 month prior to expected delivery or 5 months after delivery, so recent childbirth is important information. Often by 8 months gestational age pregnancy is physically apparent but it is important to rule out pregnancy in women of childbearing age with dilated cardiomyopathy. Screening questions regarding cocaine or alcohol abuse or other toxin exposure (such as cobalt) should be addressed.

A review of systems is also helpful in regards to connective tissue disease associated dilated cardiomyopathy (which can be related to SLE (systemic lupus erythematosis), rheumatoid arthritis, sarcoidosis, scleroderma, as well as other connective tissue diseases).

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%.[2] 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.[3] Mitochondrial inheritance of the disease has also been identified.[4]

Pericardial effusion may accompany myocarditis but this finding is not specific. Cardiac MRI as discussed above may be helpful in diagnosing myocarditis. Endomyocardial biopsy as discussed above has low sensitivy and the findings are also notoriously non-specific. The findings on biopsy usually involve findings of inflammation and specific pathogens are unlikely to be identified. There may be an increased yield to using MRI to target endomyocardial biopsy as described above. Viral titiers (serologies) are often unhelpful and not routinely ordered in clinical practice.

Prognosis

Treatment

Related chapters

Resources

References

  1. Mayo Clinic Cardiology. Concise Textbook. Murphy, Joseph G; Lloyd, Margaret A. Mayo Clinic Scientific Press. 2007.
  2. Ross J Jr. Dilated cardiomyopathy: concepts derived from gene deficient and transgenic animal models. Circ J. 2002;66:219-24. PMID 11922267
  3. 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.
  4. 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


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