Restrictive cardiomyopathy occurs when the lower chambers of your heart are rigid, stiffened, or scarred. It can cause complications like heart failure and arrhythmia. Treatment aims to ease symptoms and prevent complications.

Cardiomyopathy refers to a group of heart conditions that make it more difficult for your heart to pump blood. Restrictive cardiomyopathy (RCM) is a less common type of cardiomyopathy.

RCM happens when the lower chambers of your heart, called the ventricles, have become rigid, stiff, or scarred. This means that they can’t relax properly between heartbeats.

Because of this, the ventricles can’t fill with enough blood to pump to your body’s organs and tissues. Instead, blood can back up into your lungs or the upper chambers of your heart (atria). The atria typically become enlarged.

RCM can either be inherited or acquired. Inherited RCM happens due to gene mutations passed down from your parents. Acquired RCM develops over the course of your lifetime.

Many conditions can lead to RCM. Potential causes of RCM include:

  • Amyloidosis: You can inherit or acquire amyloidosis, in which abnormal proteins accumulate in your body, including in your heart.
  • Hemochromatosis: Hemochromatosis happens when too much iron builds up in your body’s tissues. It can also be inherited or acquired.
  • Sarcoidosis: Sarcoidosis is an acquired condition that leads to increased inflammation and the formation of cell clusters called granulomas throughout the body.
  • Other rare inherited conditions: People with certain rare inherited conditions, such as Gaucher disease or Hurler disease, can develop RCM.

Cancer treatments, such as radiation therapy or certain chemotherapy drugs, may also lead to RCM.

Types of cardiomyopathy

In addition to RCM, other types of cardiomyopathy include:

  • Dilated cardiomyopathy (DCM): DCM occurs when the ventricles weaken and become enlarged.
  • Hypertrophic cardiomyopathy (HCM): HCM is characterized by a thickening of the heart muscle.
  • Arrhythmogenic cardiomyopathy (AC or ACM): This type leads to irregular heart rhythms or heartbeats.
  • Left ventricular noncompaction (LVNC): LVNC is due to structural problems in the heart muscle, which weakens it.
  • Peripartum cardiomyopathy (PPCM): PPCM happens toward the end of pregnancy or shortly after delivery.
  • Takotsubo cardiomyopathy (TCM): Also known as “broken heart syndrome,” TCM is when extreme stress leads the heart muscle to not function properly.

Many people with RCM won’t have symptoms until their condition has worsened. When symptoms are present, they may include:

After getting your medical history and doing a physical exam, your doctor can use the following tests to help diagnose RCM:

Treatment for RCM focuses on managing symptoms and preventing complications.

A doctor may prescribe medications like:

Depending on the cause of your RCM, you may also receive other treatments. For example, corticosteroids can help treat sarcoidosis, while therapeutic phlebotomy (drawing blood from the body) can help treat hemochromatosis.

Some people with very advanced disease may benefit from an implanted device like a left ventricular assist device. Doctors may also consider a heart transplant for people with severe RCM who haven’t responded to other treatments.

RCM can lead to several complications, including:

Some conditions that cause RCM, such as amyloidosis or sarcoidosis, can also affect other organs and tissues in the body. Depending on the affected area, this may lead to additional symptoms or complications.

There’s currently no cure for RCM. The condition generally gets worse as time passes and eventually results in heart failure or other complications.

Because many people with RCM don’t have symptoms in the early stages, diagnosis often doesn’t occur until the condition is already advanced. When RCM is more advanced, the outlook is not as good.

Nevertheless, RCM is a complex condition with many potential causes. If you’ve recently received an RCM diagnosis, it’s important to have an open conversation with your care team about your specific outlook and treatment options.

Anyone can develop RCM. You may have an increased risk of developing RCM if other family members have received an RCM diagnosis or if you already have a health condition that’s associated with RCM.

While there’s no way to prevent the conditions that lead to RCM, there are steps that you can take to keep your heart as healthy as possible. These include:

It’s also important to manage any underlying health conditions by:

  • taking all medications as directed
  • seeing a doctor for regular check-ins
  • letting a doctor know if your treatment plan isn’t helping your symptoms or if your symptoms are getting worse

What is the most common cause of restrictive cardiomyopathy?

Amyloidosis is the most common cause of RCM in the United States.

What is the life expectancy of a person with restrictive cardiomyopathy?

Reports suggest a survival rate for RCM of 2–5 years. One 2023 study reported a median overall survival time of 29 months (a little over 2 years). Survival can also vary by the type of RCM that you have.

Is restrictive cardiomyopathy fatal?

Many forms of RCM are eventually fatal. RCM gets worse over time, and diagnosis typically occurs at more advanced stages when heart function is severely impaired and complications are present.

How common is restrictive cardiomyopathy?

RCM is a rare type of cardiomyopathy. Experts estimate it makes up only 5% of all cardiomyopathy diagnoses.

RCM is a less common type of cardiomyopathy. It happens when your ventricles become rigid, stiffened, or scarred. This makes it harder for your heart to pump blood to your body.

Some potential causes of RCM are due to inherited conditions. Other causes include conditions that develop during your lifetime.

An RCM diagnosis often occurs at an advanced stage when symptoms of heart failure are present. There’s no cure for RCM. Treatment focuses on managing symptoms and any underlying conditions as well as preventing complications.