Heart Health Heart Disease What Is Restrictive Cardiomyopathy? By Richard N. Fogoros, MD facebook linkedin Richard N. Fogoros, MD, is a retired professor of medicine and board-certified internal medicine physician and cardiologist. Learn about our editorial process Richard N. Fogoros, MD Medically reviewed by Medically reviewed by Yasmine S. Ali, MD, MSCI on September 14, 2020 facebook twitter linkedin Yasmine S. Ali, MD, MSCI, is a board-certified preventive cardiologist and lipidologist. Dr. Ali is also an award-winning writer. Learn about our Medical Review Board Yasmine S. Ali, MD, MSCI Updated on November 02, 2020 Print Jochen Tack / Getty Images Table of Contents View All Table of Contents Types of Restrictive Cardiomyopathy Symptoms Causes Diagnosis Treatment Restrictive cardiomyopathy (RCM) is a heart condition in which the walls of the heart become rigid and cannot expand normally to fill with blood. Although it rarely produces noticeable symptoms, RCM can cause heart failure and must be monitored closely once it's diagnosed. Restrictive cardiomyopathy is the rarest form of cardiomyopathy (an umbrella term for any disease of the heart muscle), comprising fewer than 5% of all cardiomyopathies and affecting mostly older people. Treatment for restrictive cardiomyopathy can range from taking medication to receiving a heart transplant. Also Known As Idiopathic restrictive cardiomyopathy Infiltrative cardiomyopathy Types of Restrictive Cardiomyopathy Restrictive cardiomyopathy develops as a result of scar tissue or other abnormal tissue replacing healthy tissue, causing the ventricles of the heart to become stiff and unyielding to blood flow. There are two classifications of restrictive cardiomyopathy: primary and secondary. Primary RCM refers to conditions in which the disease develops independently of underlying factors. Examples of restrictive cardiomyopathy in this category include: Endomyocardial fibrosis Löffler's endocarditis Idiopathic restrictive cardiomyopathy Secondary RCM occurs as a result of another disease that interferes with the normal function of the heart muscle, reducing its elasticity and restricting the ability of the ventricles to fill with blood. There are two categories of such diseases: Infiltrative diseases, meaning inflammatory diseases that spread to organs such as amyloidosis, sarcoidosis, and radiation carditis Storage diseases, such as hemochromatosis, glycogen storage disorders, and Fabry's disease. How Blood Flows Through the Heart and Lungs Symptoms Restrictive cardiomyopathy causes relatively few symptoms, if any. Those that do occur resemble classic symptoms of heart failure: Dyspnea (shortness of breath) Edema (swelling of the legs and feet) Weakness Fatigue Inability to exercise Heart palpitations Weight gain and bloating Nausea Poor appetite Less common symptoms of restrictive cardiomyopathy include: Fainting, often caused by irregular heart rhythms or abnormal blood vessel performance during physical exertion Chest pain or pressure, which mainly occurs with activity, but can also happen at rest or after eating Severe restrictive cardiomyopathy can cause abdominal organ congestion, producing an enlarged liver and spleen, and ascites (fluid accumulation in the abdominal cavity). Causes Restrictive cardiomyopathy occurs when the heart muscle becomes stiff, preventing it from fully relaxing during the diastolic phase of the cardiac cycle—the period between heartbeats when the heart must expand to fill with blood. This makes it difficult for the ventricles of the heart to fill adequately and causes the atria to enlarge because they must work harder than normal. The size and systolic function of the ventricles usually remain normal or near-normal, at least until later stages of the disease. What causes the heart muscle to become stiff is not fully understood. The condition is not related to coronary artery disease (CAD), but a number of conditions are associated with RCM. Sarcoidosis, a rare condition in which groups of immune cells form lumps, called granulomas, in organs in the body. It most often affects the lungs and lymph nodes in the chest, but can directly impact the heart. Hemochromatosis, a disease characterized by the accumulation of excess iron in the body. Unneeded iron is toxic and can cause organ damage. Amyloidosis, in which abnormal proteins build up in the body's organs, including the heart. Connective tissue disorders, such as scleroderma Certain cancer treatments, including radiation and chemotherapy Storage diseases, such as Gaucher disease Preventing Heart Failure With Lifestyle Changes Diagnosis Your doctor can detect restrictive cardiomyopathy with a physical exam, medical history, and diagnostic tests. Echocardiogram is the standard test used to confirm RCM. It shows diastolic abnormalities and evidence of blood not filling the ventricles properly. Other tests that may be used to diagnose and evaluate restrictive cardiomyopathy include: Electrocardiogram Chest X-ray Exercise stress test Cardiac catheterization CT scan MRI Nuclear heart scan, such as a multiple gated acquisition scan (MUGA) scan. Doctors use this noninvasive test to evaluate heart function. Blood tests to help determine which type of RCM you have. In rare cases, a doctor will order a heart muscle biopsy, which can help make the diagnosis when an infiltrative disease (such as sarcoidosis) or storage disease is present. Differential Diagnosis Restrictive cardiomyopathy may be confused with constrictive pericarditis, in which the layers of the pericardium (the membrane encasing the heart) become thickened, calcified, and rigid. Your doctor may need to rule this out in order to confirm a restrictive cardiomyopathy diagnosis. Treatment Treatment for restrictive cardiomyopathy involves managing heart failure as well as addressing underlying causes. Unfortunately, there is no specific treatment that directly reverses the RCM itself. There are, however, an array of treatment options that can help. Lifestyle Changes Your doctor will likely recommend diet and exercise as a part of your treatment plan. Exercise can help you lose weight and strengthen your heart. Adopting a low-sodium diet and losing weight help lower blood pressure, which eases the strain on your heart. Diuretics Diuretics, such as Lasix (furosemide), offer the most benefit for treating restrictive cardiomyopathy by reducing edema (swelling) but must be used judiciously and their effect monitored, as they can work too well, depleting the body of more fluid than is healthy. This may further reduce the amount of blood that fills the ventricles during the diastolic phase of each heart beat. Close monitoring of diuretic use involves measuring weight at least daily and periodically checking blood to look for signs of chronic dehydration. The optimal dose of diuretics may change over time. Calcium Channel Blockers Calcium channel blockers can help RCM by directly improving the diastolic function of the heart and slowing the heart rate to allow more time to fill the ventricles between heartbeats. For similar reasons, beta-blockers can also be helpful. If atrial fibrillation is present, it is critical to control the heart rate to allow adequate time to fill the ventricles. The use of calcium channel blockers and beta-blockers can usually accomplish this goal. Ace Inhibitors There is some evidence that ACE inhibitors may benefit at least some people with restrictive cardiomyopathy, possibly by reducing heart muscle stiffness. Heart Transplant Your doctor may recommend a heart transplant as a surgical option if other treatments fail to control restrictive cardiomyopathy symptoms. Coping When You or a Loved One Has A-Fib A Word From Verywell Restrictive cardiomyopathy is rare, but if you develop this condition you likely won't have symptoms and your quality of life will not be affected. If you are diagnosed with restrictive cardiomyopathy, your prognosis should be good: In a 2012 study, the one-, five-, and 10-year survival rates for RCM patients overall was 88%, 66%, and 45%, respectively.What's more, despite being a rare type of cardiomyopathy, there are numerous effective treatment options that your doctor can finetune specifically for you. Was this page helpful? Thanks for your feedback! Did you know the most common forms of heart disease are largely preventable? Our guide will show you what puts you at risk, and how to take control of your heart health. Sign Up You're in! Thank you, {{form.email}}, for signing up. There was an error. Please try again. What are your concerns? Other Inaccurate Hard to Understand Submit Article Sources Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. 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