DILATED CARDIOMYOPATHY

 

Dilated cardiomyopathy or DCM, also known as congestive cardiomyopathy, is a condition in which the heart becomes weakened and enlarged, and cannot pump blood efficiently. The decreased heart function can affect the lungs, liver, and other body systems.

In DCM a portion of the myocardium is dilated, often without any obvious cause. Left and/or right ventricular systolic pump function of the heart is impaired, leading to progressive cardiac enlargement and hypertrophy, a process called remodeling.

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. About one in three cases of congestive heart failure (CHF) is due to dilated cardiomyopathy. Dilated cardiomyopathy also occurs in children.

 

Causes

Although no cause (etiology) is apparent in many cases, dilated cardiomyopathy is probably the end result of damage to the myocardium 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. Autoimmune mechanisms are also suggested as a cause for dilated cardiomyopathy. A reversible form of dilated cardiomyopathy may be found with alcohol abuse, pregnancy, thyroid disease, stimulant use, and chronic uncontrolled tachycardia. Many cases of dilated cardiomyopathy are described as idiopathic - meaning that the cause is unknown.

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. The disease is genetically heterogeneous. Relatives of dilated cardiomyopathy patients have been found to show preclinical, asymptomatic heart-muscle changes.

Symptoms

 

Dilated cardiomyopathy can be present for several years without causing significant symptoms. With time, however, the enlarged heart gradually weakens.

Typical signs and symptoms of heart failure include: fatigue; weakness; shortness of breath, sometimes severe and accompanied by a cough, particularly with exertion or when lying down; and swelling of the legs and feet, resulting from fluid accumulation that may also affect the lungs (congestion) and other parts of the body. It also produces abnormal weight gain.

Because the heart is enlarged, the heart valves may be unable to close normally. The mitral valve, and the tricuspid valve, often leak. Damage to and stretching of the heart muscle may result in abnormal heart rhythms (arrhythmias). The leakage of the valves and the abnormal heart rhythms may interfere further with the heart's pumping action.

Diagnosis

The diagnosis is based on the symptoms and the results of a physical examination. Electrocardiography (ECG) may detect abnormalities in the electrical activity of the heart. However, these abnormalities are usually not sufficient evidence for a diagnosis. Echocardiography can show the size and pumping action of the heart. Magnetic resonance imaging (MRI), which produces very detailed images of the heart, may be used to confirm the diagnosis.

If the diagnosis remains in doubt, cardiac catheterization,an invasive procedure, can provide additional information about the pumping ability of the heart or help confirm the diagnosis. A biopsy can sometimes identify the characteristic microscopic changes of some disorders that cause dilated cardiomyopathy (such as a viral infection that has just developed) and thus confirm the diagnosis

Treatment

Patients are given the standard therapy for heart failure, typically including salt restriction, angiotensin-converting enzyme (ACE) inhibitors, diuretics, and digitalis. Anticoagulants may also be used. Alcohol should be avoided. Artificial pacemakers may be used in patients with intraventricular conduction delay, and implantable cardioverter-defibrillators in those at risk of arrhythmia. These forms of treatment have been shown to improve symptoms and reduce hospitalization.

In patients with advanced disease who are refractory to medical therapy, cardiac transplantation may be considered.

 

 

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