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Dilated cardiomyopathy

Dilated cardiomyopathy or DCM (also known as congestive cardiomyopathy), is a disease of the myocardium (the muscle of the heart) in which a portion of the myocardium is dilated, often without any obvious cause. About one in three cases of congestive heart failure (CHF) is due to dilated cardiomyopathy.1 more...

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A cardiomyopathy is any disease that primarily affects the muscle of the heart. In DCM, left and/or right ventricular systolic pump function of the heart is impaired, leading to progressive cardiac enlargement and hypertrophy, a process called remodeling.1

Dilated cardiomyopathy is the most common form of cardiomyopathy. It occurs more frequently in men than in women, and is most common between the ages of 20 and 60 years.2

Etiology

Although no cause is apparent in many cases, dilated cardiomyopathy is probably the end result of myocardial damage produced by a variety of toxic, metabolic, or infectious agents. It may be the late sequel of acute viral myocarditis, possibly mediated through an immunologic mechanism. Alcohol abuse is also strongly associted with the development of dilated cardiomyopthy in some cases. Autoimmune mechanisms are also suggested as a cause for dilated cardiomyopathy.3

A reversible form of dilated cardiomyopahty may be found with alcohol abuse, pregnancy, thyroid disease, cocaine use, and chronic uncontrolled tachycardia.

Genetics

About 20-40% of patients have familial forms of the disease, with mutations of genes encoding cytoskeletal, contractile, or other proteins present in myocardial cells.4 The disease is genetically heterogenous, but the most common form of its transmission is an autosomal dominant pattern. Autosomal recessive, X-linked, and mitochondrial inheritance of the disease is also found.5

Althought the disease is more common in African-Americans than in whites, it may occur in any patient population.

Associated symptoms

Symptoms of left- and right-sided congestive heart failure develop gradually in most patients. Left ventricualr dilatation may be present for months or even years before the patient becomes symptomatic.

Vague chest pain may be present, but typical angina pectoris is unusual and suggests the presence of concomitant ischemic heart disease. Syncope due to arrhythmias, and systemic embolism may occur.

Physical examination

The patients may present variable degrees of cardiac enlargement, and findings of congestive heart failure. In advance stages of the disease, the pulse pressure is narrowed and the jugular venous pressure is elevated. Third and fourth heart sounds are common. Mitral or tricuspid regurgitation may occur, presented by systolic murmurs upon auscultation (see mitral regurgitation and tricuspid insufficiency for more details about the findings).

Laboratory examinations

Generalized enlargement of the heart is seen upon normal chest X-ray. Pleural effusion may also be noticed, which is due to pulmonary venous hypertension.

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Managing life threatening viral myocarditis with dilated cardiomyopathy by Drotrecogin alfa and circulatory assisted devices
From CHEST, 10/1/05 by Tsung P. Tsai

PURPOSE: Acute fulminant myocarditis with dilated cardiomyopathy caused by Parvovirus B19 may present with cardiogenic shock refractory to the maximum dose of inotropics and intra-aortic balloon pumping (IABP). The benefits of extracorporeal membrane oxygenation (ECMO) support for patients with life-threatening myocarditis has been established. Drotrecogin alfa, recombinant human activated protein C, has antithrombotic, anti-inflammatory and profibrinolytic properties. The effectiveness from the circulatory support (ECOM or IABP) and activated PaurOtein C use in managing acute myocarditis with dilated cardiomyopathy caused by Parvovirus B19 has to be defined.

METHODS: Four patients (2 male, 2 female, mean age 37.2 years) presented with congestive heart failure 3 to 4 days after flu-like symptoms (intermittent fever 38-39 [degrees]C, dyspnea and chest tightness). Chest roentgenograms showed cardiomegaly and bilateral pulmonary infiltrates. EKG revealed non-specific ST wave changes. 2-D echocardiograms demonstrated severe myocardial dysfunction with LVEF, measured between 18.4 to 22% (mean, 19.5%). Coronary angiography was performed in each patient and excluded ischemic heart disease. Acute decompensation with more than 2 organ Failure (heart and lungs) and unresponsive to more than 2 inotropics and acute respiratory therapy were indications for the use of circulatory support by IABP (3pts) and/or ECMO (3pts) as well as activated protein C (3pts). Serological test and myocardial biopsy for Parvovirus B19 was positive in 3 pts and one pt, respectively.

RESULTS: All three pts with ECMO and IABP support were weaned. Follow-up LVEF measured were 53%, 53%, 55% and 60%, respectively. However one pt died one month later because the deterioration of her SLE condition and repeated infection. There were no neurologic sequelae in survivors.

CONCLUSION: Use of circulatory support and activated protein C is an effective alternative for treating life-threatening viral myocarditis with dilated cardiomyopathy, especially caused by Parvovirus B19 virus.

CLINICAL IMPLICATIONS: Parvovirus B19virus can cause severe myocarditis with dilated cardiomyopathy and circulatory collapse. Combined use of Drotrecogin alfa and ECMO and/or IABP is an effective novel therapy for this cohort of patients.

DISCLOSURE: Tsung Tsai, None.

Tsung P. Tsai PhD * Shyh M. Tsao MD Yi L. Wu MD Jung M. Yu MD Kuei C. Chan MD Kwo C. Ueng MD Chung Shan Medical University Hospital, Taichung, Taiwan ROC

COPYRIGHT 2005 American College of Chest Physicians
COPYRIGHT 2005 Gale Group

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