Dilated (congestive) cardiomyopathy is a group of heart muscle disorders in which the ventricles enlarge but are not able to pump enough blood for the body's needs, resulting in heart failure.
Dilated cardiomyopathy can develop at any age but is more common among people aged 20 to 60 years. About 10% of people who develop dilated cardiomyopathy are older than 65. The disorder occurs in about 3 times as many men as women and 3 times as many blacks as whites. About 5 to 8 of every 100,000 people develop the disorder each year.
In North America, the most common identifiable cause of dilated cardiomyopathy is extensive coronary artery disease. Such coronary artery disease results in an inadequate blood supply to the heart muscle, which leads to permanent injury and death of heart muscle. As a result, the heart cannot pump as forcefully. The dead heart muscle is replaced by fibrous (scar) tissue. The remaining uninjured heart muscle then stretches and thickens (hypertrophies) to compensate for the lost pumping action. The more the heart muscle is stretched, the more forcefully it contracts or pumps but only up to a point. After that point, the stretching and thickening do not adequately compensate, and dilated cardiomyopathy with heart failure develops.
Dilated cardiomyopathy also may be caused by an acute inflammation of the heart muscle (myocarditis) due to a viral infection. This disorder is called viral cardiomyopathy. In North America, infection with coxsackie B virus is the most common cause of viral cardiomyopathy. In other parts of the world, other viral infections are more common causes. The virus infects and weakens the heart muscle. As in coronary artery disease, the weakened heart stretches in an attempt to compensate, resulting in dilated cardiomyopathy and often heart failure. Occasionally, dilated cardiomyopathy results from a bacterial infection.
Other causes of dilated cardiomyopathy include certain chronic hormonal disorders such as long-standing, poorly controlled diabetes or thyroid disease, high blood pressure, morbid obesity, or a persistently rapid heart rate. Dilated cardiomyopathy also can be caused by use of certain substances, especially alcohol (when intake is heavy and malnutrition is also present), cocaine, antidepressants, newer antipsychotic drugs, and a few chemotherapy drugs. Rare causes of dilated cardiomyopathy include pregnancy, iron overload, and connective tissue disorders such as rheumatoid arthritis. Genetic factors play a role in 20 to 35% of cases. When no specific cause can be identified, the disorder is called an idiopathic dilated cardiomyopathy.
Usually, the first symptoms of dilated cardiomyopathy are becoming short of breath during exertion and tiring easily. They result from a weakening of the heart's pumping action, which is called heart failure (see see Heart Failure). When cardiomyopathy results from an infection, the first symptoms may be a sudden fever and flu-like symptoms. Whatever the cause of dilated cardiomyopathy, if the heart damage is severe enough, the heart rate eventually speeds up, blood pressure is normal or low, fluid is retained in the legs and abdomen, and the lungs fill with fluid.
Because the heart is enlarged, the heart valves may be unable to close normally and often allow blood to leak back into the heart chamber rather than flowing into the next blood vessel or chamber. The valves most often affected are the mitral valve, which is positioned between the left atrium (upper heart chamber) and the left ventricle (lower heart chamber), and the tricuspid valve, which is positioned between the right atrium and the right ventricle. Leakage causes murmurs, which doctors can hear with a stethoscope. Damage to and stretching of the heart muscle may result in abnormal heart rhythms (arrhythmias), which may cause sensation of irregular heartbeats (palpitations) or death. The leakage of the valves and the abnormal heart rhythms may interfere further with the heart's pumping action.
Blood can pool in the enlarged heart, increasing the risk of blood clots forming on heart chamber walls. The clots can break into pieces (becoming emboli), travel from the heart to blood vessels elsewhere in the body, and block them, causing damage to the organ they supply. If the blood supply to the brain is blocked, a stroke can result.
The diagnosis is based on the symptoms, the results of a physical examination, and additional tests. Electrocardiography (ECG) may detect abnormalities in the electrical activity of the heart. However, these abnormalities are usually not sufficient evidence for a diagnosis. Echocardiography, which uses ultrasound waves to produce an image of the heart, is the most useful procedure because it can show the size and pumping action of the heart. Cardiac magnetic resonance imaging, which produces very detailed images of the heart, is more often being used to confirm the diagnosis (and sometimes identify the cause).
If the diagnosis remains in doubt, cardiac catheterization, an invasive procedure in which a catheter is threaded into the heart (see Cardiac catheterization), can provide additional information about the pumping ability of the heart. During cardiac catheterization, doctors can also measure pressures in the heart chambers and determine the extent of coronary artery disease. During catheterization, doctors sometimes remove a tissue sample for examination under a microscope (biopsy). 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. However, usually, the results of a biopsy are not specific enough to help with the diagnosis.
Prognosis varies considerably depending on many factors. In general, the prognosis worsens as the heart becomes more dilated and functions less well. Abnormal heart rhythms also indicate a worse prognosis. Overall, men survive only half as long as women, and blacks survive half as long as whites. About 40 to 50% of deaths are sudden, probably resulting from an abnormal heart rhythm or an embolus that blocks blood flow in a critical area. Additional factors include the cause and severity of the cardiomyopathy, the person's age and ability to follow medical advice, access to specialized treatment, poor patient compliance with medications, salt in the diet, and outpatient visits. However, in recent years overall prognosis has improved with current treatments, especially with the introduction of implantable cardioverter-defibrillators, cardiac resynchronization therapy, and other interventions.
If possible, doctors treat the underlying cause.
General treatment measures include avoiding stress, limiting salt in the diet, and having periods of rest, which help reduce strain on the heart, particularly when the cardiomyopathy is acute or severe.
Drugs, such as angiotensin-converting enzyme (ACE) inhibitors, angiotensin II receptor blockers, beta-blockers, aldosterone antagonists (spironolactone or eplerenone), and low-dose digoxin, improve the heart's pumping function, prolong life, and help to decrease persistent symptoms. Diuretics are used to reduce excess fluid in the lungs and decrease symptoms of swelling due to fluid retention, but they do not prolong life.
Antiarrhythmic drugs may be given to treat abnormal heart rhythms. Most of these drugs are initially prescribed in small doses. Doses are increased in small increments because if the dose is too large, an antiarrhythmic drug may worsen heart rhythm abnormalities or depress pumping function. Some people have an abnormality of the electrical conduction in the heart, which can be helped by a pacemaker that stimulates first the atria and then the ventricles (cardiac resynchronization therapy). This type of pacing, when used in the right person, will help to return the contraction pattern of the heart toward normal and improve its function. Doctors may also consider an implantable cardioverter-defibrillator pacemaker in patients with persistent poor heart function and an increased risk of sudden death (see Restoring normal rhythm).
Regardless of the cause of dilated cardiomyopathy, doctors may give anticoagulants, such as warfarin or aspirin, to prevent blood clots, which may form on the heart chamber walls of very dilated and poorly contracting ventricles.
Unless a specific cause of dilated cardiomyopathy can be treated, heart failure in dilated cardiomyopathy is progressive and ultimately fatal. Because of this poor prognosis, dilated cardiomyopathy is the most common reason for heart transplantation (see see Heart Transplantation) or mechanical heart support with a device. Successful heart transplantation cures the disorder, but it has its own complications and limitations.
Last full review/revision September 2013 by J. Malcolm O. Arnold, MD