Cardiomyopathy (patient information)

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Editors-in-Chief: C. Michael Gibson, M.S., M.D. Associate Editor-In-Chief: Ujjwal Rastogi, MBBS [mailto:urastogi@perfuse.org]

Overview
Cardiomyopathy refers to diseases of the heart muscle. These diseases enlarge your heart muscle or make it thicker and more rigid than normal. In rare cases, scar tissue replaces the muscle tissue. There are of three main types
 * Dilated
 * Hypertrophic
 * Restrictive

all of these affect heart muscle.

What are the symptoms of cardiomyopathy?
In early stages of the disease, some patient might be asymptomatic. During the course of the disease it can present with the following symptoms
 * Breathlessness which can be exertional or at rest.
 * Swelling of lower limbs.
 * Fatigue and fainting.
 * Irregular heart beat.

What causes Cardiomyopathy?
Heart attacks, high blood pressure, Nutritional deficiencies, Pregnancy, Alcohol/Cocaine abuse or infections can all cause cardiomyopathy. Some types of cardiomyopathy run in families. In many people, however, the cause is unknown

Who is at highest risk?
People of all ages and races can have cardiomyopathy. However, certain types of the disease are more common in certain groups.

Dilated cardiomyopathy is more common in African Americans than Whites. This type of the disease also is more common in men than women.

Teens and young adults are more likely than older people to have arrhythmogenic right ventricular dysplasia, although it's rare in both groups.

Major Risk Factors
Certain diseases, conditions, or factors can raise your risk for cardiomyopathy. Major risk factors include:
 * A family history of cardiomyopathy, heart failure, or sudden cardiac arrest (SCA)
 * A disease or condition that can lead to cardiomyopathy, such as coronary heart disease, heart attack, or a viral infection that inflames the heart muscle
 * Diabetes or other metabolic diseases, or severe obesity
 * Diseases that can damage the heart, such as hemochromatosis, sarcoidosis, or amyloidosis
 * Long-term alcoholism
 * Long-term high blood pressure

Some people who have cardiomyopathy never have signs or symptoms. Thus, it's important to identify people who may be at high risk for the disease. This can help prevent future problems, such as serious arrhythmias (irregular heartbeats) or SCA.

When to seek urgent medical care
One should seek medical care when suffering from symptoms of cardiomyopathy or a positive family history.

Diagnosis
Your doctor will diagnose cardiomyopathy based on your medical and family histories, a physical exam, and the results from tests and procedures.

Specialists Involved
Often, a cardiologist or pediatric cardiologist diagnoses and treats cardiomyopathy. A cardiologist specializes in diagnosing and treating heart diseases. A pediatric cardiologist is a cardiologist who treats children.

Medical and Family Histories
Your doctor will want to learn about your medical history. He or she will want to know what signs and symptoms you have and how long you've had them. Your doctor also will want to know whether anyone in your family has had cardiomyopathy, heart failure, or sudden cardiac arrest.

Physical Exam
Your doctor will use a stethoscope to listen to your heart and lungs for sounds that may suggest cardiomyopathy. These sounds may even suggest a certain type of the disease. For example, the loudness, timing, and location of a heart murmur may suggest obstructive hypertrophic cardiomyopathy. A "crackling" sound in the lungs may be a sign of heart failure. (Heart failure often develops in the later stages of cardiomyopathy.) Physical signs also help your doctor diagnose cardiomyopathy. Swelling of the ankles, feet, legs, abdomen, or veins in your neck suggests fluid buildup, a sign of heart failure. Your doctor may notice signs and symptoms of cardiomyopathy during a routine exam. For example, he or she may hear a heart murmur, or you may have abnormal test results.

Diagnostic Tests
Your doctor may recommend one or more of the following tests to diagnose cardiomyopathy.

Blood Tests
During a blood test, a small amount of blood is taken from your body. It's often drawn from a vein in your arm using a needle. The procedure usually is quick and easy, although it may cause some short-term discomfort. Blood tests give your doctor information about your heart and help rule out other conditions.

Chest X Ray
A chest x ray takes pictures of the organs and structures inside your chest, such as your heart, lungs, and blood vessels. This test can show whether your heart is enlarged. A chest x ray also can show whether fluid is building up in your lungs.

EKG(Electrocardiogram)
An EKG is a simple test that records the heart's electrical activity. The test shows how fast the heart is beating and its rhythm (steady or irregular). An EKG also records the strength and timing of electrical signals as they pass through each part of the heart. This test is used to detect and study many heart problems, such as heart attacks, arrhythmias (irregular heartbeats), and heart failure. EKG results also can suggest other disorders that affect heart function. A standard EKG only records the heartbeat for a few seconds. It won't detect problems that don't happen during the test. To diagnose heart problems that come and go, your doctor may have you wear a portable EKG monitor. The two most common types of portable EKGs are Holter and event monitors.

Holter and Event Monitors
Holter and event monitors are small, portable devices. They record your heart's electrical activity while you do your normal daily activities. A Holter monitor records the heart's electrical activity for a full 24- or 48-hour period. An event monitor records your heart's electrical activity only at certain times while you're wearing it. For many event monitors, you push a button to start the monitor when you feel symptoms. Other event monitors start automatically when they sense abnormal heart rhythms.

Echocardiography
Echocardiography (echo) is a test that uses sound waves to create a moving picture of your heart. The picture shows how well your heart is working and its size and shape. There are several types of echo, including stress echo. This test is done as part of a stress test (see below). Stress echo can show whether you have decreased blood flow to your heart, a sign of coronary heart disease. Another type of echo is transesophageal (tranz-ih-sof-uh-JEE-ul) echo, or TEE. TEE provides a view of the back of the heart. For this test, a sound wave wand is put on the end of a special tube. The tube is gently passed down your throat and into your esophagus (the passage leading from your mouth to your stomach). Because this passage is right behind the heart, TEE can create detailed pictures of the heart's structures. Before TEE, you're given medicine to help you relax, and your throat is sprayed with numbing medicine.

Stress Test
Some heart problems are easier to diagnose when your heart is working hard and beating fast. During stress testing, you exercise (or are given medicine if you're unable to exercise) to make your heart work hard and beat fast while heart tests are done. These tests may include nuclear heart scanning, echo, and positron emission tomography (PET) scanning of the heart.

Diagnostic Procedures
You may have one or more medical procedures to confirm a diagnosis or to prepare for surgery (if surgery is planned). These procedures may include cardiac catheterization (KATH-e-ter-i-ZA-shun), coronary angiography (an-jee-OG-ra-fee), or myocardial (mi-o-KAR-de-al) biopsy.

Cardiac Catheterization
This procedure checks the pressure and blood flow in your heart's chambers. The procedure also allows your doctor to collect blood samples and look at your heart's arteries using x-ray imaging. During cardiac catheterization, a long, thin, flexible tube called a catheter is put into a blood vessel in your arm, groin (upper thigh), or neck and threaded to your heart. This allows your doctor to study the inside of your arteries for blockages.

Coronary Angiography
This procedure often is done with cardiac catheterization. During the procedure, dye that can be seen on an x ray is injected into your coronary arteries. The dye lets your doctor study blood flow through your heart and blood vessels. Dye also may be injected into your heart chambers. This allows your doctor to study the pumping function of your heart.

Myocardial Biopsy
For this procedure, your doctor removes a piece of your heart muscle. This can be done during cardiac catheterization. The heart muscle is studied under a microscope to see whether changes in cells have occurred. These changes may suggest cardiomyopathy. Myocardial biopsy is useful for diagnosing some types of cardiomyopathy.

Genetic Testing
Some types of cardiomyopathy run in families. Thus, your doctor may suggest genetic testing to look for the disease in your parents, brothers and sisters, or other family members.

Genetic testing can show how the disease runs in families. It also can find out the chances of parents passing the genes for the disease on to their children. Genetic testing also may be useful if your doctor thinks you have cardiomyopathy, but you don't yet have signs or symptoms. If the test shows you have the disease, your doctor can start treatment early, when it may work best.

People who have cardiomyopathy but no signs or symptoms may not need treatment. Sometimes, dilated cardiomyopathy that comes on suddenly may even go away on its own. For other people who have cardiomyopathy, treatment is needed. Treatment depends on the type of cardiomyopathy you have, the severity of your symptoms and complications, and your age and overall health. The main goals of treating cardiomyopathy include:
 * Managing any conditions that cause or contribute to the disease
 * Controlling signs and symptoms so that you can live as normally as possible
 * Stopping the disease from getting worse
 * Reducing complications and the risk of sudden cardiac arrest (SCA)

Treatment options
Treatments may include lifestyle changes, medicines, surgery, implanted devices to correct arrhythmias (irregular heartbeats), and/or a nonsurgical procedure.

Lifestyle Changes
Your doctor may suggest lifestyle changes to manage a condition that's causing your cardiomyopathy. These changes can help reduce symptoms. Healthy Diet and Physical Activity A healthy diet and physical activity are part of a healthy lifestyle. A healthy diet includes a variety of fruits, vegetables, and grains; half of your grains should come from whole-grain products. Choose foods that are low in saturated fat, trans fat, and cholesterol. Healthy choices include lean meats, poultry without skin, fish, beans, and fat-free or low-fat milk and milk products. Choose and prepare foods with little sodium (salt). Too much salt can raise your risk of high blood pressure. Recent studies show that following the Dietary Approaches to Stop Hypertension (DASH) eating plan can lower blood pressure. Choose foods and beverages that are low in added sugar. If you drink alcoholic beverages, do so in moderation. Aim for a healthy weight by staying within your daily calorie needs. Balance the calories you take in with the calories you use during physical activity. Be as physically active as you can. Some people should get medical advice before starting or increasing physical activity. For example, talk with your doctor if you have a chronic (ongoing) health problem, are on medicine, or have symptoms such as chest pain, shortness of breath, or dizziness. Your doctor can suggest types and amounts of physical activity that are safe for you. For more information about following a healthy diet, go to the National Heart, Lung, and Blood Institute's (NHLBI's) Aim for a Healthy Weight Web site, "Your Guide to a Healthy Heart," and "Your Guide to Lowering Your Blood Pressure With DASH." All of these resources provide general information about healthy eating. For more information about physical activity, go to Diseases and Conditions Index Physical Activity and Your Heart article and the NHLBI's "Your Guide to Physical Activity and Your Heart."

Your doctor can help you decide what kind of eating plan and physical activity are right for you.

Other Lifestyle Changes Your doctor also may recommend other lifestyle changes, such as:
 * Quitting smoking
 * Losing excess weight
 * Avoiding the use of alcohol and illegal drugs
 * Getting enough sleep and rest
 * Reducing stress
 * Treating underlying conditions, such as diabetes and high blood pressure

Medicines
Many medicines are used to treat cardiomyopathy. Your doctor may prescribe medicines to:
 * Lower your blood pressure. ACE inhibitors, angiotensin II receptor blockers, beta blockers, and calcium channel blockers are examples of medicines that lower blood pressure.
 * Slow your heart rate. Beta blockers, calcium channel blockers, and digoxin are examples of medicines that slow the heart rate. Beta blockers and calcium channel blockers also are used to lower blood pressure.
 * Keep your heart beating with a normal rhythm. These medicines, called antiarrhythmics, help prevent arrhythmias.
 * Balance electrolytes in your body. Electrolytes are minerals that help maintain fluid levels and acid-base balance in the body. They also help muscle and nerve tissues work properly. Abnormal electrolyte levels may be a sign of dehydration (lack of fluid in your body), heart failure, high blood pressure, or other disorders. Aldosterone blockers are an example of a medicine used to balance electrolytes.
 * Remove excess fluid and sodium from your body. Diuretics, or "water pills," are an example of a medicine that helps remove excess fluid and sodium from the body.
 * Prevent blood clots from forming. Anticoagulants, or "blood thinners," are an example of a medicine that prevents blood clots. Blood thinners often are used to prevent blood clots from forming in people who have dilated cardiomyopathy.
 * Reduce inflammation. Corticosteroids are an example of a medicine used to reduce inflammation.

Surgery
Doctors use several types of surgery to treat cardiomyopathy. They include septal myectomy (mi-EK-toe-me), implanted devices to help the heart work better, and heart transplant.

Septal Myectomy
Septal myectomy is open-heart surgery. It's used for people who have obstructive hypertrophic cardiomyopathy and severe symptoms. This surgery generally is used for younger patients and for people whose medicines aren't working well. During the surgery, a surgeon removes part of the thickened septum that's bulging into the left ventricle. This improves blood flow through the heart and out to the body. The removed tissue doesn't grow back. The surgeon also can repair or replace the mitral valve at the same time (if needed). Septal myectomy often is successful and allows you to return to a normal life with no symptoms.

Surgically Implanted Devices
Surgeons can place several types of devices in the heart to help it work better. One example is a pacemaker. This is a small device that's placed under the skin of your chest or abdomen to help control arrhythmias. The device uses electrical pulses to prompt the heart to beat at a normal rate. Sometimes doctors choose to use a cardiac resynchronization therapy (CRT) device. A CRT device coordinates contractions between the heart's left and right ventricles.

A left ventricular assist device (LVAD) helps the heart pump blood to the body. An LVAD can be used as a long-term therapy or as a short-term treatment for people who are waiting for a heart transplant.

An implantable cardioverter defibrillator (ICD) helps control life-threatening arrhythmias that may lead to SCA. This small device is implanted in the chest or abdomen and connected to the heart with wires.

If an ICD senses a dangerous change in heart rhythm, it will send an electric shock to the heart to restore a normal heartbeat. Heart Transplant

For this surgery, a surgeon replaces a person's diseased heart with a healthy heart from a deceased donor. A heart transplant is a last resort treatment for people who have end-stage heart failure. "End-stage" means the condition has become so severe that all treatments, other than heart transplant, have failed. For more information about this treatment, go to the Diseases and Conditions Index Heart Failure article.

Nonsurgical Procedure
Doctors may use a nonsurgical procedure called alcohol septal ablation to treat cardiomyopathy. For this procedure, your doctor injects ethanol (a type of alcohol) through a tube into the small artery that supplies blood to the thickened area of heart muscle. The alcohol kills cells, and the thickened tissue shrinks to a more normal size.

This procedure allows blood to flow freely through the ventricle, which improves symptoms.

Where to find medical care for Cardiomyopathy
Directions to Hospitals Treating Cardiomyopathy

What to expect (Outlook/Prognosis)
Some people live long, healthy lives with cardiomyopathy. Some people don't even realize they have it. In others, however, it can make the heart less able to pump blood through the body

Some people who have cardiomyopathy—especially those who have hypertrophic cardiomyopathy (HCM)—may live a healthy life with few problems or symptoms. Others may have serious symptoms and complications.

Possible complications
Cardiomyopathy gets worse unless treated. The most likely complications are
 * Heart Failure.
 * Arrythmia i.e. irregular heart beat.
 * Blood Clots.
 * Sudden cardiac death