Cardiomyopathy Treatment

Although cardiomyopathy is often not curable, there are many treatment alternatives that can effectively control the symptoms for an improved quality of life for those with the disease. The treatment choice is largely based on the cause and type of cardiomyopathy as well as the extent of the damage to the heart muscle.

Treatment therapies for cardiomyopathy aim to:

• Stop or slow the progression of the damage to the heart
• Improve the function of the heart
• Reduce or eliminate symptoms
• Prevent sudden death
• Treat common associated conditions that worsen heart function, such as sleep apnea or coronary artery blockages

This section contains more information on:

• Treatment of hypertrophic cardiomyopathy
• Treatment of restrictive cardiomyopathy
• Treatment of ischemic and dilated cardiomyopathy
• Medication
• Mitral valve repair
• Ventricular Remodeling or Partial Left Ventriculectomy
• Cardiac pacemaker
• Implantable cardioverter defibrillator
• Left ventricular assist device
• Heart transplant
• Heart surgery

Treatment of Hypertrophic Cardiomyopathy

Because hypertrophic cardiomyopathy (HCM) affects each person differently, there is no "typical patient" and no standard plan of treatment. Furthermore, the treatment rationale for HCM can actually counter the medical rationale used to treat the same suite of symptoms in a patient who does not have HCM. For instance, many HCM patients have chest pain and shortness of breath--a common symptom of coronary artery disease that is treated with nitroglycerin. If a person with HCM were to take nitroglycerin, the symptoms could worsen and the nitroglycerin could even cause a catastrophic deterioration in the person's condition.

Treatment of Restrictive Cardiomyopathy

Restrictive cardiomyopathy is a difficult disease to treat effectively. Medications used for heart failure are often ineffective and surgery is usually not an option. In rare circumstances, a heart transplant may be an option. A pacemaker is sometimes implanted to assist in the transmission of electrical signals in the heart in order to maintain a stable heartbeat.

For patients whose restrictive cardiomyopathy is caused by another disease, treatment of the underlying disease is usually the most effective course of action for controlling the symptoms and slowing down the progression of this disease.

Treatment of Ischemic or Dilated Cardiomyopathy

Although ischemic cardiomyopathy and the other forms of dilated cardiomyopathy can cause the symptoms of heart failure, each type requires a specific treatment strategy of medications, surgical intervention, and lifestyle choices to effectively control the disease. The patient's involvement in the treatment plan and commitment to follow-up care are crucial to the success of the treatment.

For patients with ischemic cardiomyopathy, the underlying cause of their cardiomyopathy, coronary artery disease, must be treated as well. This usually involves restoring circulation to the heart muscle and controlling CAD in addition to treating the cardiomyopathy.

Dilated cardiomyopathy is treated with a combination of the following treatment options:

• Medications including diuretics, beta blockers, angiotensin-converting enzyme (ACE) inhibitors, angiotensin receptor blockers, aldosterone inhibitors, hydralazine, nitrates, digoxin, anticoagulants, or antiarrhythmic medications may be prescribed
• Heart surgery to repair abnormal heart valves, or bypass coronary blockages if present, to improve the functioning of the heart
• Surgical implantation of devices including cardiac pacemaker, cardioverter defibrillator, or left ventricular assist device
• Heart transplant

Medication

Medications commonly used to treat heart failure associated with dilated cardiomyopathy, ischemic cardiomyopathy and restrictive cardiomyopathy include:

Beta-adrenergic (beta) blockers may be prescribed to slow the heart rate and lower blood pressure by reducing the affect of adrenaline on the heart. By slowing the heart rate, beta blockers decrease the heart's demand for oxygen, which decreases the workload on the heart, lessening the symptoms of heart failure. Beta blockers can prevent further heart enlargement and scarring and decrease the risk of sudden death. Beta blockers have also been shown to increase survival by about 30-35 percent in patients with dilated cardiomyopathy. In most cases, your doctor will start you on a low dose of beta blockers, then slowly increase the dose over time until the desired results are achieved.

Diuretics, commonly called water pills, help relieve excess fluid in the body by promoting urine production in the kidneys. This decreases symptoms of shortness of breath, swelling, and bloating. Diuretics vary in potency and speed, and doctors sometimes prescribe a combination of diuretic agents. Diuretics can improve symptoms, but they also activate hormones in the body such as adrenaline that can worsen heart and kidney function. This is why they are used in conjunction with other medicines such as beta blockers and ACE inhibitors.

Positive inotropic medications can beused to strengthen the contractions of the heart in people with severe dilated cardiomyopathy. Examples of these drugs include digoxin, dopamine, dobutamine, and milrinone. Digoxin has been used for over 200 years to improve heart contractions and seems to work best in people who have severe heart failure complicated by atrial fibrillation because digoxin can slow a rapid heart rate. With the exception of digoxin, these drugs are usually only used for a short period of time, or when there are no other treatment options, due to concern that inotropic agents may actually accelerate the degeneration of the heart by forcing an already weakened organ to work harder. Positive inotropic medications should never be used by people with hypertrophic cardiomyopathy because they can exacerbate this condition.

Vasodilators are commonly used to reduce symptoms of heart failure and can prolong the life of people with dilated cardiomyopathy. Angiotensin-converting enzyme (ACE) inhibitors and angiotensin receptor blockers (ARBs) are examples of vasodilators, which cause blood vessels throughout the body to relax. This decreases the resistance to blood flow, measured as blood pressure, which in turn reduces the workload on the heart. These medicines can also reverse the enlargement and scarring of the heart, decrease the risk of hospitalization and death, and improve symptoms. Other vasodilators include hydralazine and nitrates, which also reduce the workload of the heart and have been shown to improve symptoms and survival in dilated cardiomyopathy.

Aldosterone blockers are used to help balance electrolytes in the body and also to prevent heart and blood vessel scarring, stiffening, and enlargement. Studies have shown that aldosterone blockers can improve survival in patients with severe dilated cardiomyopathy and those who have had a heart attack complicated by cardiomyopathy.

Antiarrhythmic drugs alter the way in which electrical currents are transmitted through the heart muscle. The heart pumps most efficiently when it beats in a regular rhythm; antiarrhythmics can increase the efficiency of the heart by regulating the heart rhythm. These drugs are used only in selected patients because they can have significant side effects.

More detailed information on dosages and precautions for each of these types of drugs can be found on the National Institute of Health Medline website.

Mitral Valve Repair

For people with dilated cardiomyopathy, the functions of the mitral valve, which connects the left atrium to the left ventricle, can be affected by the enlargement of the heart. The dilation of the heart can cause the opening to the mitral valve to expand beyond the size of the valve, keeping the mitral valve from closing properly. When this happens, blood can regurgitate back through the valve into the left atrium. Mitral valve regurgitation decreases pumping efficiency of the heart and can lead to the symptoms of heart failure.

For some people with dilated cardiomyopathy, mitral valve repair improves the functioning of the heart muscle and can alleviate many of the symptoms of heart failure. Mitral valve regurgitation in people with dilated cardiomyopathy is treated during open heart surgery by either modifying the original valve and its opening or, in some cases, replacing the valve with a prosthetic heart valve.

Most surgeons agree that heart valves should be repaired when possible and replaced only when necessary. Valvuloplasty, or surgically modifying the original valve, can effectively eliminate regurgitation in some people. Surgeons can shorten or replace the cords that support the valves so that the cords and muscles are the right length and the valve leaflet edges meet, eliminating the leak. Alternatively, surgeons can make the ring of tissue surrounding the valve opening smaller, which allows the leaflets of the valve to close tightly, preventing regurgitation. Sometimes, surgeons will do this by implanting an annuloplasty ring. Surgeons can also repair the valve by removing excess tissue to help the valve close snugly.

In some cases, the mitral valve will need to be surgically removed and replaced with a prosthetic one--either a mechanical prosthetic valve or a bioprosthetic valve. The key to selecting the right prosthetic valve involves weighing the advantages and disadvantages of certain valves with the risk of anticoagulation therapy.

Mechanical prosthetic valves, made of synthetic materials, are more durable than tissue valves but require that a blood-thinning drug such as warfarin be taken for the rest of the person's life. People who take these drugs must have their blood tested regularly to ensure that their blood has not gotten too "thick" or "thin." Blood thinners are not recommended for people who play contact sports and pose risks for a developing fetus.

Bioprosthetic valves are made from animal tissue or human tissue taken from a donated heart and do not require blood thinners; however, they are not are not as durable as mechanical valves and may require replacement in about 10 years. In general, patients younger than 65 receive mechanical valves, while those over 70 receive bioprostheses. For some younger people, a bioprosthetic valve may be used with the understanding that additional surgery will be necessary in the future to replace it with a mechanical valve.

Ventricular Remodeling or Partial Left Ventriculectomy

Surgical ventricular remodeling (SVR) is a procedure that reduces the size of left ventricle to improve the function of the heart. This procedure is an alternative to heart transplant for patients with advanced ischemic or dilated cardiomyopathy.

SVR requires open heart surgery, where the surgeon either removes a section of the heart muscle or makes a tuck in the existing muscle to reduce the overall size of the organ. This procedure effectively reshapes the heart from a basketball shape to a more natural football shape and can improve heart function in some people.

Cardiac Pacemaker Implantation

Cardiac pacemakers are sometimes used in patients with dilated cardiomyopathy to help maintain a stable heart rhythm. These small electronic devices, about the size of a stopwatch, monitor the electrical activity of the heart and synchronize the contractions within the heart. When necessary, the pacemaker will send an electrical impulse, which cannot be felt, to stimulate the heart to contract.

The most commonly used pacemaker for cardiomyopathy is a dual chamber pacemaker, which attaches to the heart in two different places using tiny wires known as leads. This allows doctors to alter the sequence of contraction within the heart muscle. The placement of the leads on the heart and the setting of the time delay between the activation of the two leads are crucial to the success of this treatment.

Cardiac resynchronization therapy (CRT) uses a specialized pacemaker designed to coordinate how the heart beats. In addition to overall weakening of the heart muscle and poor contraction, many patients with dilated cardiomyopathy also have an abnormal pattern of contraction, with half of the heart pumping while the other half does not. CRT "re-times" the heart so that both sides pump synchronously. This has been shown to improve symptoms of heart failure and improve survival in appropriate patients.

Pacemakers are implanted in a hospital room equipped with special X-ray equipment. In adults, local anesthesia and sedation are used to minimize any discomfort felt during the procedure. The procedure begins with cleaning the chest with antibacterial soap and starting an intravenous line in the arm. The pacemaker leads are then inserted into a vein under the collarbone and guided into the heart; X-rays are used to help guide the placement of the pacemaker leads. After the leads are in place, the pacemaker is slipped into a small, surgically created pocket under the skin in the upper part of the chest. The pacemaker will then be fine-tuned to your specific heart rhythm over the next 24 hours. Upon dismissal, you'll receive a card that identifies you as having a pacemaker.

Ask your doctor about any restrictions on your activity before you leave the hospital. In most cases, physical activity can gradually be resumed, but your doctor will probably tell you to limit driving and participation in contact sports. The pacemaker must be monitored regularly, which can often be done by telephone using a special transmitter. Usually, weekly monitoring is recommended for the first four weeks, then once every three months after that.

General guidelines for people with pacemakers:

• Memorize the name of your device's manufacturer.
• Check with your healthcare provider before starting an exercise program.
• Do not drive until your healthcare provider says you may.
• Stay at least 8 feet away from arc welders.
• Avoid powerful magnets and heavy industrial equipment.
• Do not stand in entryways equipped with retail surveillance/shoplifting devices; walking past at normal speed should not interfere with the pacemaker.
• Keep cellular phones at least 6 inches from the pacemaker and use on the opposite side from the implant.
• Do not lean over a running engine; standing next to it is safe.
• Do not have magnetic resonance imaging (MRI).
• Before you receive any healthcare, be sure to tell the provider that you have a pacemaker.
• Tell healthcare provider about your pacemaker before a lithotripsy (procedure used to remove a kidney stone) or any procedures that involves using heat or electricity to burn tissue (cautery, diathermy, or radiation therapy).
• Whenever you have surgery, the surgeon must contact your cardiologist before the surgery because your pacemaker may need to be reprogrammed before the procedure.

Implantable Cardioverter-Defibrillators (ICD)

Implantable cardioverter defibrillators are used to prevent sudden death in patients with cardiomyopathy who are at increased risk. Treatment for the prevention of sudden death is an active area of investigation in cardiomyopathy. Originally, antiarrhythmic medications were used to prevent sudden death, but recent research indicates that implantable cardioverter-defibrillators may be more effective.

Similar to cardiac pacemakers, ICDs are small electric devices that monitor the heart rhythms and send electrical impulses to the heart. If the heart rate becomes dangerously slow, the ICD responds exactly like a pacemaker, sending an electrical impulse that stimulates the heart to beat but cannot be felt. Unlike a pacemaker, ICDs also treat unstable and potential lethal rhythms such as ventricular tachycardia (VT) and ventricular fibrillation (VF). When these rhythms occur, the ICD transmits a succession of low-energy impulses called pacing, a high-energy shock, or a combination of the two. Usually described as a "kick in the chest," shocks may be painful, but last only a fraction of a second with no lingering pain or damage.

The effectiveness of ICDs in preventing sudden death is difficult to assess. In patients who have previously been resuscitated from cardiac arrest and received an ICD, 80 percent of ICDs subsequently discharged appropriately, interrupting a potentially lethal heart rhythm. For other people who have not had cardiac arrest but who are at increased risk for sudden death due to other factors, the likelihood of their ICD interrupting a potentially fatal heart rhythm is about 4 percent to 5 percent per year. While most of these shocks are appropriate, there are instances where a shock is delivered inappropriately for a more benign heart rhythm.

The implantation procedure is done under general anesthesia and requires one to two days of hospitalization. After the anesthetic is administered, the surgeon will make an incision and place one or two electrical leads inside the heart. After the leads are tested, the ICD is implanted in a surgically created pocket near the collarbone. In some instances, an additional lead may be placed under the skin near the rib cage. Usually there is some lingering pain in the area of the incision that can be managed with medication. When you are discharged, the hospital will provide a card that identifies you as having an ICD.

After the cardioverter defibrillator is implanted, patients can gradually return to an active lifestyle as recommended by their doctor. There may be some restrictions on driving, and a stress test may be necessary to ensure your safety and prevent unintended shocks. ICDs contain remote programming devices that allow doctors to adjust the electrical programming without additional surgery. In addition, you will need to keep a written diary of any electrical therapy that occurs--including when it happened, what you were doing, any symptoms experienced before the shock, and how your body responded.

General precautions for ICDs include:

• Avoid contact sports.
• Avoid medical procedures such as magnetic resonance imaging (MRI), lithotripsy (a procedure to remove kidney stones), cautery (using heat or electricity to burn tissue).
• Tell the healthcare provider you have an ICD before receiving radiation therapy or diathermy (therapy that uses electricity to generate heat).
• Do not allow airport security personnel to use hand-held metal detectors on your body. Ask to be searched by hand instead.
• Avoid magnetic fields and keep strong magnets away from the ICD.
• Stay at least 8 feet away from arc welders.
• Show medical personnel the ICD card when receiving care.

Emotional support and encouragement from family members and friends are very important part of helping patients live with their heart condition and ICDs. Other ways family members can participate in the medical care are:

• Complete a cardiopulmonary resuscitation (CPR) lifesaving course.
• Help maintain a diary and an ID bracelet or necklace with up-to-date medical information.
• Memorize the name of the ICD manufacturer.
• Be prepared to show medical personnel the ICD card when healthcare is being administered.

Left Ventricular Assist Devices (LVAD)

Originally designed for short-term use in people waiting for a heart transplant, left ventricular assist devices are now used to treat patients with advanced heart failure who are ineligible for a heart transplant. A left ventricular assist device is an electronic pump that is surgically implanted inside the abdominal cavity to help the weakened heart circulate blood through the body.

LVADs are evolving rapidly; the newest devices are smaller than an adult's fist and can last more than five years. However, these new LVADs are not yet approved by the FDA; to receive one of the newer designs, you must enroll in a clinical trial at participating transplant/VAD centers in the United States.

Implantation of a left ventricular device requires heart surgery. During the procedure, the LVAD is connected to the left ventricle of the heart and the aorta. Oxygenated blood from the weakened left ventricle is channeled into the LVAD, which then pumps the blood out into the aorta, circulating oxygen and nutrients to the tissues of the body.

After recovery, patients can live a near-normal lifestyle, traveling, hiking, and playing golf. Swimming and bathtubs are out, though, because the devices use external batteries that need to stay dry. Patients carry the batteries in packs or vests and can replace them as needed. Like heart transplant patients, people with LVADs need to visit the doctor regularly for follow-up; however, immunosuppressant therapy is not needed. In addition, LVADs can malfunction, so people using LVADs need ready access to a hospital equipped to handle this type of emergency.

Heart Transplant

Dilated cardiomyopathy (including ischemic cardiomyopathy) is the most common reason for a heart transplant. Each year, roughly 2,000 heart transplants are performed. The exact number is determined by the supply of donor hearts. There are many more people needing heart transplants than there are donor hearts. More information on becoming an organ donor is available at donatelife.net.

Most people who receive a heart transplant feel better immediately. At hospitals experienced in heart transplants in the United States, nearly 90 percent of heart recipients survive one year or more and about 70 percent survive five years or more. Some recipients have survived 10 years or more if organ rejection is successfully controlled.

Organ rejection is a lifelong risk for heart transplant recipients. Organ rejection occurs when the body's immune system perceives the donor organ as a foreign substance and tries to destroy it, just as the body fights the flu. Even though the donor heart and recipient are matched by blood type and in some cases tissue typing, no two people are exact matches (except identical twins). To minimize the risk of the body's immune system attacking the donor heart, immunosuppressive medication must be taken regularly after a heart transplant. However, suppressing the immune system reduces the body's ability to fight infection, the main side effect of these medications. Other potential side effects of immunosuppressive therapy include high blood pressure, diabetes, kidney function problems, weight gain and certain types of cancers. So it's crucial for heart transplant recipients to prevent infection and closely monitor their overall health.

In general, to be eligible for a heart transplant, patients must be less than 70 years old, with a life expectancy of less than one to three years without the heart transplant. Patients must be motivated for transplantation and able to comply with a lifelong commitment to medical therapy, as well as be free of any medical barriers for transplantation. People with pulmonary hypertension (high pressure in the arteries to the lungs), infection, obesity, unresolved drug (including tobacco) or alcohol abuse, or any noncardiac disease that significantly limits life expectancy or will be worsened by using the immunosuppressive medications required after transplant generally are ineligible for a heart transplant.

Patients must undergo extensive evaluation to determine if they are eligible to receive a transplant and place them on the transplant list maintained by United Network for Organ Sharing (UNOS). Once eligibility has been established, it may take several days to years before a matching donor heart is available. Eligibility status may change over time as your health changes.

Once patients are on the waiting list, some transplant centers require them to relocate within two to three hours' travel of the transplant center. Sometimes patients choose to register for a transplant in multiple areas of the country to increase their chances of getting a heart, if the transplant centers allow it. For more information on the national organ waiting list, you can visit the website of the Organ Procurement and Transplantation Network.

When a donor heart becomes available, UNOS will rank potential recipients according to criteria such as blood type, heart and body size, and urgency of the case as well as how long the patient has already been on the waiting list and distance between donor and recipient. UNOS then contacts transplant centers starting with the highest-ranked patient and work down the list until the heart is accepted for transplant. More information on matching is available from OPTN.

Successful transplantation requires a healthcare team of cardiologists, surgeons, anesthesiologists, infectious diseases doctors, nurses, counselors, physical therapists, and others. Prior to the operation, patients need to have blood tests, urine samples, and a chest X-ray to ensure that their condition is satisfactory. The heart transplantation procedure is a straightforward, open heart procedure usually involving three to four hours of surgery, barring complications. During the procedure, the donor heart is connected to the aorta, pulmonary arteries, and atria or vessels leading to the atria. After the procedure, the patient will spend several days in the intensive-care unit.

Heart transplant recipients are typically discharged from the hospital after about one to three weeks. Ongoing care can be demanding and many transplant hospitals require patients to stay near the hospital for at least three months for follow-up healthcare. The levels of immunosuppressive medications are closely monitored and heart tissue biopsies are collected at regular intervals to check for signs of rejection. Also, because the nerves to the heart are severed during transplantation, heart recipients do not feel chest pain when the oxygen supply to the heart is compromised. Therefore, annual coronary angiograms are necessary to look for atherosclerosis. Heart transplant requires a lifelong commitment to daily medications, regular exercise, abstaining from smoking and alcohol, and regular medical monitoring.

Heart Surgery

In most heart operations, the surgeon makes an incision in the center of the chest. Alternatively, a less-invasive incision can be made on the side of the chest when appropriate. During the procedure, a cardiopulmonary bypass machine is usually needed to take over the functions of the heart and lungs. Once the procedure is completed and the surgical team is satisfied that the heart is beating strongly, the cardiopulmonary bypass machine is disconnected and the chest incisions are sutured closed. After heart surgery, all patients are moved to a surgical intensive-care unit (ICU) to begin the recovery process.

Complete recovery from heart surgery, to the point where you can return to full activity levels, usually takes about six to eight weeks. Patients usually regain a daily routine in about two to three weeks. The first five to seven days of recovery are spent in the hospital, starting in the ICU.

In the ICU, the patient's condition is constantly monitored using surgically inserted tubes and drains that record fluid uptake and loss and assist with breathing. Sleeping in the ICU can be difficult for patients, and visitors may find the environment distracting. It's not uncommon for patients to appear pale or swollen and even become temporarily confused during this stage of recovery. Once the patient's condition has improved, most of the tubes and drains will be removed and the patient will be moved out of the ICU to complete their hospital stay in a bed equipped for continuous cardiac monitoring.

Before the patient leaves the hospital, members of the healthcare team will discuss the at-home recovery plan with the patient. The staff should provide written instructions for diet, pain management, incision care, physical therapy, medications, and follow-up appointments. Restrictions on activities such as driving, heavy lifting, and showering will also be covered. Pain management is an important aspect of recovery, allowing patients to perform simple functions such as coughing, breathing deeply, and walking that are important steps to recovery. Toughing it out can actually delay recovery.

Emotional responses to heart surgery vary widely due to the stress of operation and medication. These events may trigger feelings of denial, distress, fear, anger, and even depression. Most of the time these feelings are temporary; however, if they persist, discuss them with a member of the healthcare team.