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Restrictive Cardiomyopathy Symptoms, Causes & Treatment

Learn about restrictive cardiomyopathy, including symptoms, causes, treatment options, and when to seek consultation. Or take a quiz to get a second opinion on your restrictive cardiomyopathy from our A.I. health assistant.

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What Is Restrictive Cardiomyopathy?

Summary

Restrictive cardiomyopathy is a type of heart disorder in which the walls of the heart become stiff, preventing it from filling with blood normally. The atria become enlarged and blood flow in the heart is gradually reduced, which may lead to heart failure or arrhythmias [1].

Symptoms include shortness of breath, chest discomfort or fluttering in the chest, swelling in the abdomen or legs, and feelings of being weak or more tired than usual.

Treatments include medications to slow the heart rate, diuretics, blood thinners, the surgical implant of a pacemaker, and alleviation of underlying causes.

Recommended care

You should seek immediate medical care at an ER, where an ultrasound can confirm the diagnosis, blood tests can determine the cause of the problem, and treatment is needed immediately to stabilize you.

How common is restrictive cardiomyopathy?

Rare

Restrictive cardiomyopathy is also known as

  • Idiopathic restrictive cardiomyopathy
  • Infiltrative cardiomyopathy

Restrictive Cardiomyopathy Symptoms

It is possible to lead a normal life with restrictive cardiomyopathy. Some experience minor symptoms while others experience symptoms more severely [2].

Main symptoms

Most people with restrictive cardiomyopathy will experience:

  • Shortness of breath: This usually begins gradually and may be worse when lying flat. You may wake up in the middle of the night gasping for air. Shortness of breath occurs when the heart doesn't function properly, causing fluid to back up into the lungs (pulmonary edema).
  • Chest discomfort or a fluttering sensation in the chest: A heart with restrictive cardiomyopathy is prone to develop an abnormal rhythm such as an abnormally fast heartbeat. You may experience discomfort in the chest or a fluttering sensation (palpitations).
  • Feeling weak or more tired than usual: This occurs because in restrictive cardiomyopathy, the heart is no longer able to pump blood to the rest of the body as effectively. You may be unable to exercise as long or as hard as you did previously.

Swelling

Some people may also develop the following symptoms of swelling, which is caused by fluid backing up from the heart to the rest of the body.

Restrictive Cardiomyopathy Causes

Certain diseases, associated conditions, and genetic mutations cause restrictive cardiomyopathy. When no cause can be found the disorder is known as "idiopathic restrictive cardiomyopathy." Specific causes include diseases that result in deposits in the heart, metabolic diseases, genetic mutations, chemotherapy or radiation, and metastatic cancers.

Diseases involving deposits in the heart

Diseases in which abnormal materials deposit in the walls of the heart can lead to restrictive cardiomyopathy. Examples include:

  • Amyloidosis: Abnormally folded proteins deposit in multiple organs including the heart, liver, and kidney.
  • Sarcoidosis: Inflammation and scarring develops in the heart.
  • Hemochromatosis: Too much iron is absorbed through the gut and the excess iron is deposited in multiple organs including the heart, liver, and pancreas..

Metabolic diseases

Diseases resulting in defects in the body's metabolic processes cancause substances to build up in the heart.

  • Glycogen storage disorders: The body fails to normally store sugars.
  • Lysosomal storage disorders: Various substances are not normally processed in the cells of the body and build up to dangerous levels.

Genetic mutations

Restrictive cardiomyopathy caused by genetic mutations usually runs in families. The mutated genes affect proteins in heart muscle cells that are important for normal heart structure and function.

Chemotherapy or radiation therapy

Certain chemotherapy medications and radiation therapy to the chest can cause a restrictive cardiomyopathy by damaging the cells of the heart and causing structural changes [3].

Metastatic cancers

Cancers that metastasize (spread) to the heart can grow in the walls of the heart and cause restrictive cardiomyopathy.

Restrictive Cardiomyopathy Symptom Checker

Take a quiz to find out if your symptoms point to restrictive cardiomyopathy

Treatment Options and Prevention

Restrictive cardiomyopathy is a chronic disease that cannot be completely cured. Treatment is focused on improving symptoms and slowing the progression of the disease. Specific treatment options include medications to slow the heart rate or prevent long-term changes, diuretics, blood thinners, implanting a pacemaker, treatment of underlying diseases, and, if no other option works, a heart transplant.

Medications to slow the heart rate

Slowing the heart rate can help people with restrictive cardiomyopathy by giving the heart more time to properly fill before pumping. Medications that slow the heart rate include:

  • Carvedilol (Coreg)
  • Metoprolol (Lopressor)
  • Verapamil (Calan)
  • Diltiazem (Cardizem)

Medications to prevent long-term heart changes

Other medications can help by inhibiting changes that can lead to long-term remodeling of the heart, thus preventing it from getting weaker. Two common medications include:

  • Lisinopril (Prinivil)
  • Valsartan (Diovan)

Diuretics

People with restrictive cardiomyopathy who experience symptoms such as shortness of breath or swelling may benefit from taking medications that remove fluid from the body (diuretics). These medications will remove fluid by increasing the volume of urine produced [4].

These medications include:

  • Furosemide (Lasix)
  • Bumetanide (Bumex)
  • Torsemide (Demadex)

Blood thinners

Some people with restrictive cardiomyopathy may develop abnormal heart rhythms such as atrial fibrillation. Some of these medications may require regular laboratory testing to make sure you are receiving the right amount of blood thinning. Your physician may recommend starting a blood thinner such as:

  • Warfarin (Coumadin)
  • Dabigatran (Pradaxa)
  • Apixaban (Eliquis)

Implanting a pacemaker

Some people with restrictive cardiomyopathy may develop a problem in heart conduction in which different parts of the heart do not pump in a coordinated manner. In this happens to you, your physician may recommend a surgery to implant a device known as a dual-chamber permanent pacemaker. This device will help the heart beat in a coordinated manner, which can improve its function.

Treatment of underlying diseases

In cases of restrictive cardiomyopathy in which there is a known cause, you should receive treatment for the underlying disease. The specific treatment will vary depending on the specific cause. For example, sarcoidosis, which is a disease of inflammation, is treated with steroids, which reduce inflammation [5].

Heart transplant

If you have restrictive cardiomyopathy that does not respond to other treatments, you may benefit from receiving a heart transplant.

  • Details: The diseased heart will be removed and replaced with a healthy donor heart. As this plan is most likely a lot to absorb, there are support groups available [6].
  • Prognosis: If the underlying disease is not treated, the new donor heart may also eventually develop restrictive cardiomyopathy. Success of transplants varies among children [7] and adults [8].

When to Seek Further Consultation

You should seek medical attention if you believe you have developed restrictive cardiomyopathy, or you have been diagnosed with a disease associated with this condition.

If you develop any symptoms of restrictive cardiomyopathy

You should go see your physician if you experience symptoms such as shortness of breath, swelling, or chest discomfort. He or she can order tests and imaging to determine if you have restrictive cardiomyopathy and then offer the appropriate treatments.

If you have been diagnosed with a disease that can cause restrictive cardiomyopathy

You should regularly follow up with your physician if you experience symptoms such as amyloidosis, sarcoidosis, or hemochromatosis. He or she can monitor you for the development of new symptoms that may suggest the development of restrictive cardiomyopathy and treat you sooner rather than later.

Questions Your Doctor May Ask to Diagnose

To diagnose this condition, your doctor would likely ask about the following symptoms and risk factors.

  • Any fever today or during the last week?
  • Do you have a cough?
  • Are you sick enough to consider going to the emergency room right now?
  • Have you been feeling more tired than usual, lethargic or fatigued despite sleeping a normal amount?
  • Do you notice your heart beating hard, rapidly, or irregularly (also called palpitations)?

The above questions are also covered by our A.I. Health Assistant.

Restrictive Cardiomyopathy Symptom Checker

Take a quiz to find out if your symptoms point to restrictive cardiomyopathy

References

  1. Restrictive Cardiomyopathy. American Heart Association. Published March 31, 2016. AHA Link
  2. Restrictive Cardiomyopathy. Cleveland Clinic. Cleveland Clinic Link
  3. Armanious MA, Mohammadi H, Khodor S, Oliver DE, Johnstone PA, Fradley MG. Cardiovascular Effects of Radiation Therapy. Current Problems in Cancer. 2018. PubMed Link
  4. Guglin M. Diuretics as Pathogenetic Treatment for Heart Failure. International Journal of General Medicine. 2011;4:91-98. DovePress Link
  5. Rammos A, Meladinis V, Vovas G, Patsouras D. Restrictive Cardiomyopathies: The Importance of Noninvasive Cardiac Imaging Modalities in Diagnosis and Treatment - A Systematic Review. Radiology Research and Practice. 2017;2017:2874902. Radiology Research and Practice
  6. Support Groups. United Network for Organ Sharing. NOS Link
  7. Singh TP, Almond CS, Piercey G, Gauvreau K. Current Outcomes in US Children with Cardiomyopathy Listed for Heart Transplantation. Circulation: Heart Failure. 2012;5(5):594-601. AHA Link
  8. DePasquale EC, Nasir K, Jacoby DL. Outcomes of Adults with Restrictive Cardiomyopathy After Heart Transplantation. The Journal of Heart and Lung Transplantation. 2012;31(12):1269-1275. Pubmed Link