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Cardiomyopathy

Cardiomyopathy is the deterioration of the cardiac muscle of the heart wall. Cardiomyopathy can lead to heart failure as the pumping efficiency of the heart is diminished. People with cardiomyopathy are often at risk of arrhythmia and/or sudden cardiac death. more...

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Cardiomyopathies can generally be categorized into two groups: ischemic cardiomyopathy and nonischemic cardiomyopathy.

Ischemic

Ischemic cardiomyopathy is weakness in the muscle of the heart due to coronary artery disease. Individuals with ischemic cardiomyopathy typically have a history of myocardial infarction (heart attack).

Nonischemic

Nonischemic cardiomyopathy is weakness in the muscle of the heart that is not due to coronary artery disease. To make a diagnosis of nonischemic cardiomyopathy, significant coronary artery disease should be ruled out. The term nonischemic cardiomyopathy does not describe the etiology of weakened heart muscle. The nonischemic cardiomyopathies are a mixed-bag of disease states, each with their own causes.

Nonischemic cardiomyopathy has a number of causes including drug and alcohol toxicity, certain infections (including Hepatitis C), and various genetic and idiopathic (i.e. unknown) causes.

Nonischemic subtypes

There are four main types of nonischemic cardiomyopathy:

  • Dilated cardiomyopathy (DCM), the most common form of cardiomyopathy, and one of the leading indications for heart transplantation. In DCM the heart (especially the left ventricle) is enlarged and weakened. Approximately 40% of cases are familial, but the genetics are poorly understood compared with HCM. In some cases it manifests as peripartum cardiomyopathy, and in other cases it may be associated with alcoholism.
  • Hypertrophic cardiomyopathy (HCM or HOCM), a genetic disorder caused by various mutations in genes encoding sarcomeric proteins. In HCM the heart muscle is thickened, which can obstruct blood flow and prevent the heart from functioning properly.
  • Arrhythmogenic right ventricular cardiomyopathy (ARVC) arises from an electrical disturbance of the heart in which heart muscle is replaced by fibrous scar tissue. The right ventricle is generally most affected.
  • Restrictive cardiomyopathy (RCM) is the least common cardiomyopathy. The walls of the ventricles are stiff, but may not be thickened, and resist the normal filling of the heart with blood. A rare form of restrictive cardiomyopathy is the obliterative cardiomyopathy, seen in the hypereosinophilic syndrome. In this type of cardiomyopathy, the myocardium in the apicies of the left and right ventricles become thickened and fibrotic, causing a decrease in the volumes of the ventricles and a type of restrictive cardiomyopathy.

Treatment

Treatment depends on the type of cardiomyopathy, but may include medical therapy and implanted artificial pacemakers. The goal of treatment is often symptom relief, with the underlying condition unaffected. Some patients may eventually require a heart transplant. Treatment of cardiomyopathy (and other heart diseases) using alternative methods such as stem cell therapy is commercially available but is not supported by convincing evidence.

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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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