Cardiomyopathy is the term for any disease of the heart muscle that interferes with the heart’s ability to pump blood with sufficient force. Cardiomyopathy can be classified as ischemic (caused by coronary artery disease and heart attacks where lack of blood damages the heart muscle, resulting in cardiomyopathy) or nonischemic types that include:
Dilated (or congestive) cardiomyopathy is weakness in the walls of the heart that causes them to balloon out, compromising the heart’s efficiency and increasing the risk of congestive heart failure, arrhythmias and the formation of blood clots (which may cause heart attacks or strokes).
Hypertrophic cardiomyopathy, overgrowth or thickening of heart muscle, may compromise blood flow through the heart.
Restrictive cardiomyopathy involves loss of elasticity of the heart walls that prevents the heart from adequately filling with blood prior to contracting.
Except when caused by viral infections, cardiomyopathy develops slowly and may produce no symptoms until the later stages. The disorder is relatively rare, accounting for only 1 percent of heart disease fatalities in the United States, although it is one of the more common causes of serious heart disease in younger people. Cardiomyopathy due to coronary disease is very common in the elderly. Depending upon the type of cardiomyopathy, treatment may include medications, implantable devices or, in severe cases, a heart transplant.
- If you experience severe breathlessness.
- If you experience crushing chest pain, with or without nausea, vomiting, profuse sweating, breathlessness, weakness or intense feelings of dread. Such symptoms may indicate a heart attack
- If you regularly experience fatigue and shortness of breath after mild physical activity.
- If you experience any of the following during treatment: fever, rapid or irregular heartbeat, wheezing or severe shortness of breath or any worsening of the other symptoms.
Often there are no symptoms. When symptoms do occur, they are most commonly associated with the syndrome called congestive heart failure and may include the following:
- Shortness of breath, especially during exertion.
- Swelling of the feet, ankles or hands (edema).
- Dizziness or fainting.
- Wheezing and a dry cough, or a cough producing foamy, bloody phlegm.
- Chest pain (may be mild).
- Stroke or painful and cold extremity due to a blood clot blocking a blood vessel.
- Inherited disease
- Viral infections of the heart can which cause inflammation of the heart muscle (myocarditis) that may result in permanent damage.
- Excess consumption of alcohol that may be toxic to the heart muscle over time.
- Nutritional deficiencies (such as lack of vitamin B1 or potassium) and hormone imbalances that may damage and weaken the heart muscle.
- Amyloidosis, a disorder in which the walls of the heart are infiltrated by a waxy substance that may cause restrictive cardiomyopathy (see amyloidosis for more information).
- Smoking and obesity which increase the risk for cardiomyophathy.
- Advanced coronary artery disease, which is the most common cause of dilated cardiomyopathy.
To reduce the risk of heart disease in general, eat a well-balanced low-fat diet, exercise regularly, and lose weight if you are overweight. Limit yourself to no more than two alcoholic beverages each day and don’t smoke.
The Johns Hopkins Cardiomyopathy and Heart Failure Practice, directed by Dr. Stuart Russell, uses a multidisciplinary approach to evaluate and manage patients with cardiomyopathy, a condition in which the heart’s left ventricle -- its main pumping chamber -- loses its ability to pump blood.
Our multidisciplinary team includes professionals from social work, dietary counseling, physical rehabilitation, educational programs and support groups. In some instances, cardiomyopathy disturbs heart rhythm, leading to irregular heartbeats, or arrhythmias. Our goal is to empower patients to better care for themselves by improving compliance, patient understanding and family support. We believe that transplantation is the “court of last resort” and will do everything possible to avoid transplantation.
To be evaluated for cardiomyopathy, one of our physicians will first examine you at our Outpatient Center or satellite facility at Green Spring Station. If necessary, we may ask you to undergo one or more of the following tests: electrocardiogram (ECG or EKG), cardiac catheterization, pressure-volume loop analysis, X-ray, echocardiogram or biopsy. Electrocardiograms are done at the Outpatient Center; other tests are performed in the hospital’s Cardiovascular Diagnostic Laboratory. We can also now offer all these tests in a single day at the same place in our Heart Success One-Day Clinic.
- An electrocardiogram, also called an ECG or EKG, records your heart’s electrical activity during rest to determine abnormal heart rhythms.
- To evaluate pressures in the heart, your doctor may perform an outpatient procedure called a cardiac catheterization. This test involves the following: inserting a thin plastic tube through a blood vessel until it reaches the heart; injecting a dye into the blood vessels; and taking X-rays to assess the heart’s structure and function. Simultaneously, your doctor may perform a pressure-volume loop analysis. This test, developed at Johns Hopkins, measures the amount of blood flow put out by the heart during each beat. The results can help your doctor pinpoint what kind of cardiomyopathy you might have.
- Your doctor may take chest X-rays to see if the heart is enlarged.
- Your doctor may perform an echocardiogram, which uses ultrasound waves to image the heart’s structure and movement.
- Your doctor may take a biopsy, or small sample, of the heart muscle. In this technique, a doctor will use local anesthesia to insert a small needle into your heart to remove a small bit of tissue, which is then sent to a laboratory for analysis. This helps determine the extent of disease.
Core components of treatment:
- Clinical care of patients with heart failure and following cardiac transplantation.
- Basic science, hemodynamic, genetic and clinical research pertaining to cardiomyopathy, heart failure and cardiac transplantation.
- Education directed at both patients and professionals regarding cardiomyopathy, heart failure and cardiac transplantation.
- All facets of clinical care of patients with cardiomyopathy, heart failure or cardiac transplantation.
- Left ventricular assist devices.
- Novel research programs for patients with cardiomyopathy or heart failure.
- Endomyocardial biopsy.
Possible courses of treatment:
Because cardiomyopathy is difficult to diagnose early, it is rarely treated in its beginning stage. At Johns Hopkins, our treatment goal is to relieve any complicating factors, control symptoms and stop the disease’s progression. No known cure exists.
- Your doctor may prescribe medication to reduce heart muscle exertion, improve the heart’s pumping ability, regulate the heartbeat and ease symptoms.
- You may be advised to make lifestyle changes that include avoiding strenuous physical activity, abstaining from alcohol, losing weight and reducing the amount of salt in your diet.
- If the heart muscle has been badly damaged and your condition does not improve with medications or lifestyle management, you may need a heart transplant.
Cardiovascular Diagnostic and Interventional Laboratory
The Johns Hopkins Hospital Cardiovascular Diagnostic Laboratory (CVDL) is a state-of-the-art imaging facility performing over 24,000 diagnostic and interventional procedures annually. The CVDL operates 11 procedure rooms.
There are three general areas within the CVDL: Cardiology, Radiology and Electrophysiology. The Cardiology section is involved in treating patients with disorders of the heart and vascular tree including coronary artery disease, congestive heart failure, valve disease, congenital heart defects, cardiomyopathy, and peripheral vascular disease.
Cardiomyopathy services included in the CDVL:
- Diagnosis and evaluation of cardiomyopathic conditions using endomyocardial biopsy.
- Hemodynamic and metabolic assessment of hypertrophic cardiomyopathy using transvenous pacing protocols, percutaneous septal myocardial ablation.