Cardiac Sarcoidosis

Cardiac sarcoidosis is a condition in which your immune system triggers inflammation that harms your heart. Clumps of immune cells called granulomas form in your heart. Over time, these may cause scarring. The scars can damage your heart muscle, interfere with your heart’s electrical activity and cause abnormal heart rhythms.

This condition is a less common form of sarcoidosis that specifically affects your heart. Most people with sarcoidosis have granulomas in their lungs. But it’s possible to have them in your heart, too, and not know until testing reveals them. Experts believe that up to 1 in 4 people with sarcoidosis have heart involvement.

Symptoms of cardiac sarcoidosis

Cardiac sarcoidosis can disrupt normal heart function and cause symptoms like:

• Chest pain or tightness
• Coughing
• Fainting
• Fatigue
• Heart palpitations
• Shortness of breath
• Swelling in your legs

It’s also possible not to feel any symptoms of sarcoidosis in your heart. It depends on where the granulomas form and how much inflammation they cause.

Cardiac sarcoidosis causes

Experts don’t fully understand what causes cardiac sarcoidosis. They believe it’s likely a mix of your genes and your environment. Certain genes may make your immune system more likely to overreact to triggers like mold, insecticides, silica dust or bacteria — causing immune cells to cluster in your heart.

You have a higher risk of cardiac sarcoidosis if someone in your biological family has the condition.

Complications of this condition

Cardiac sarcoidosis can keep your heart from working as it should. For example, inflammation or scars may block electrical signals from getting where they need to be. This can cause your heart to beat out of sync. Or your heart may be unable to pump enough blood to meet your body’s needs. Without treatment, these issues can be life-threatening.

Possible complications include:

• Atrial fibrillation
• Heart block
• Heart failure
• Ventricular fibrillation
• Ventricular tachycardia
• Sudden cardiac death

How healthcare providers diagnose this condition

To diagnose cardiac sarcoidosis, your provider will give you a physical exam and ask about your symptoms. They’ll review your health history and ask about any biological family members who have sarcoidosis. You’ll also need some tests. These allow your provider to check for signs of heart inflammation or scarring. Testing also helps rule out other conditions.

Possible tests include:

• Blood tests
• Chest X-ray
• Electrocardiogram (EKG)
• Echocardiogram
• Heart MRI
• Holter monitor
• Heart PET scan

The exact tests you need can vary. If you’ve already been diagnosed with sarcoidosis elsewhere in your body, your provider may want to “screen” for it in your heart. This may happen even without heart symptoms. If you have symptoms, you’ll need more extensive testing. Providers usually don’t use biopsies to diagnose cardiac sarcoidosis.

Early diagnosis gives you the best chance to manage symptoms and protect your heart.

Stages

There’s no single staging system for cardiac sarcoidosis. But providers may use terms like “early stage” or “advanced stage” to describe how much tissue damage they see.

Imaging can help your provider see how far the condition has progressed. Early on, inflammation in your heart may not cause lasting damage. But over time, scars can form. Ideally, providers diagnose this condition in the early stages, before scars damage your heart.

How is cardiac sarcoidosis treated?

Treatment involves medicines to reduce inflammation, manage your heart rhythm and treat heart failure. You may also need a device or procedure to lower your risk of dangerous heart rhythms. In severe cases, a heart transplant may be a last resort.

Medicines that treat cardiac sarcoidosis include:

• Steroids to reduce inflammation, like prednisone
• Immunosuppressants to calm your immune response, like methotrexate (providers may prescribe these along with prednisone)
• Heart failure medicines to help your heart work better
• Antiarrhythmic drugs to help your heart beat in a normal rhythm

Devices and procedures include:

• Pacemaker to help your heart beat in a normal rhythm
• Implantable cardioverter defibrillator (ICD) to send a shock, when needed, to correct an abnormal rhythm
• Cardiac ablation to block abnormal electrical signals

When should I see my healthcare provider?

Your provider will tell you how often you need follow-up appointments and imaging tests. Follow the schedule they give you. Call your provider anytime you have new symptoms, side effects from medicines or questions about your treatment plan.

If possible, seek care at a hospital that has a sarcoidosis center. There, you can receive care from a team of experts in this condition. You may work with heart doctors, lung doctors and other specialists depending on where the granulomas are building up. 

When should I go to the ER?

Call 911 or your local emergency number if you have any of the following signs of a dangerous arrhythmia:

• Chest pain
• Heart palpitations that aren’t going away
• Dizziness
• Fainting
• Racing or erratic pulse
• Shortness of breath

What’s the prognosis for cardiac sarcoidosis?

You’ll typically have the best outcome if your healthcare provider diagnoses your cardiac sarcoidosis early and starts treatment right away. Your outlook may not be as good without prompt treatment. But each person’s experience is a little different. Your provider can tell you what to expect in your situation.

People who only have sarcoidosis in their hearts have a worse prognosis than those who have sarcoidosis throughout their bodies. This is because they have a higher risk of ventricular arrhythmias. These are abnormal heart rhythms in the lower chambers of your heart.

How long can you live with cardiac sarcoidosis?

Your life expectancy depends on many things, including how early you’re diagnosed and treated. One study showed that 95% of people with cardiac sarcoidosis were alive five years after their diagnosis. And 93% were alive 10 years after their diagnosis. Your healthcare provider can help you understand more.