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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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Implantable defibrillators and nonischemic cardiomyopathy
From American Family Physician, 4/1/05 by David Slawson

Clinical Question: Do implantable defibrillators reduce mortality in patients with nonischemic cardiomyopathy?

Setting: Various (meta-analysis)

Study Design: Meta-analysis (randomized controlled trials)

Synopsis: Investigators thoroughly searched multiple databases using evidence-based sources including MEDLINE, EMBASE, and the Cochrane Central Register of Controlled Trials. Inclusion criteria identified only prospective randomized controlled trials of implantable defibrillators versus medical therapy in patients with nonischemic cardiomyopathy. The trials reported all-cause mortality. From an initial 675 articles of potential relevance, eight met the inclusion criteria (with a total of 2,146 patients). Of these, five were primary prevention trials enrolling 1,854 patients with nonischemic cardiomyopathy. The authors did not state specifically whether the individual studies were evaluated independently by more than one person.

Overall, mortality was reduced significantly among patients randomized to an implantable defibrillator versus medical therapy (relative risk = 0.69; 95 percent confidence interval, 0.55 to 0.87). The findings from the various studies were similar (homogeneous) and there was no evidence of significant publication bias. Implantable defibrillators did not decrease mortality following a myocardial infarction (Hohnloser SH, et al. Prophylactic use of an implantable cardioverter-defibrillator after acute myocardial infarction. N Engl J Med December 9, 2004;351:2481-8).

Bottom Line: In addition to standard medical therapy, implantable defibrillators further reduce mortality in selected patients with nonischemic cardiomyopathy. (Level of Evidence: 1a-)

Study Reference: Desai AS, et al. Implantable defibrillators for the prevention of mortality in patients with nonischemic cardiomyopathy: a meta-analysis of randomized controlled trials. JAMA December 15, 2004;292:2874-9.

Used with permission from Slawson D. Implantable defibrillators beneficial for nonischemic cardiomyopathy. Accessed online January 25, 2005, at: http://www.InfoPOEMs.com.

COPYRIGHT 2005 American Academy of Family Physicians
COPYRIGHT 2005 Gale Group

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