Cardiac sarcoidosis is a rare disease in which your body’s immune response to inflammation makes scars. These harm your heart, causing abnormal heart rhythms. Scars can get in the way of heartbeat signals, keeping them from getting where they need to go. Most people need medication, but others need devices to keep their hearts beating normally.
Sarcoidosis is a rare inflammatory disease. It can harm different parts of your body. When it affects your heart, which happens rarely, it’s called cardiac sarcoidosis.
When your body reacts to inflammation from this condition, scars can form. These damage your heart muscle and other parts of your heart. This can cause abnormal heart rhythms and problems with heartbeat signals getting through your whole heart.
Healthcare providers diagnose cardiac sarcoidosis in 2% to 5% of people who have sarcoidosis elsewhere in their bodies. However, an estimated 20% to 30% of Americans with sarcoidosis may actually have it in their hearts.
Sarcoidosis affects up to 30 people out of 100,000 per year.
Cardiac sarcoidosis is more common in Japan. More than half of the people with sarcoidosis in Japan also have the condition in their hearts.
However, cardiac sarcoidosis is less common than sarcoidosis in other organs.
An estimated one-third of people with cardiac sarcoidosis don’t have it anywhere else in their bodies. Your heart can be the first organ sarcoidosis affects.
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Cardiac sarcoidosis symptoms may include:
The cause of cardiac sarcoidosis is unknown. Researchers think some people may get it because their genes make them more at risk for environmental effects.
To make a cardiac sarcoidosis diagnosis, your healthcare provider will:
They’ll check to see if you have sarcoidosis in other parts of your body and confirm that you have signs of it in your heart.
Tests to make a cardiac sarcoidosis diagnosis include:
Healthcare providers often use biopsies (looking at a tissue sample) to diagnose sarcoidosis in other parts of your body. But they don’t use it much for your heart. This is because it’s hard to get a sample of tissue from where the problem is happening.
Imaging can help healthcare providers decide cardiac sarcoidosis stages. These can go from normal to severe, starting with early and then progressive stages. An active stage leads to progressive myocardial (heart muscle) impairment and then fibrosis (scarring).
Your healthcare provider has several options for cardiac sarcoidosis treatment. These treatments include:
Healthcare providers often prescribe a high dose of prednisone (Sterapred® or Rayos®). You take it for several months and then start taking smaller doses. This can make your symptoms better if you get early treatment.
Prednisone side effects include:
Ventricular tachycardia happens again in 86% of people whose catheter ablation worked at first.
People with cardiac sarcoidosis have a less than 10% chance of getting it again after a heart transplant.
It depends on your situation. You’ll have the best outcome if your healthcare provider diagnoses your cardiac sarcoidosis early and starts treatment right away. Your outlook won’t be as good without prompt treatment.
You’ll likely have a less optimistic prognosis if you have pulmonary hypertension or ventricular aneurysm.
Most people do well with early treatment.
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.
A study that followed people with cardiac sarcoidosis for 10 years found they were at a higher risk of ventricular arrhythmia and sudden cardiac death. Medicines can prevent this, but they’re not 100% effective.
Most people with cardiac sarcoidosis die from ventricular arrhythmias. If you’re at risk for sudden death, your healthcare provider may want to give you an ICD. This device goes under your skin. It can protect you from dangerous heart rhythms.
Studies have found that 83% or 93% of people living with cardiac sarcoidosis were alive 10 years later.
Survival rates were 80% or higher for one and five years after a heart transplant.
Yes, cardiac sarcoidosis can be fatal for some people. Your risk of death is higher if your heart’s left ventricle doesn’t pump blood as well as it should.
Because your condition can worsen in a short amount of time, it’s important to keep taking the medicine your healthcare provider prescribed for you. You could be taking prednisone for a year.
You’ll need to get imaging tests again one to six months after you start taking steroids (prednisone). Your provider will want to see how well the medicine is working and figure out the size of future doses.
You’ll need checkups with your provider for at least three years after you stop taking steroids or start taking smaller doses. Cardiac sarcoidosis can come back. This happens in 40% of people.
If you’re having chest pain with shortness of breath, sustained palpitations, dizziness and/or fainting, call 911. You could be having an abnormal heart rhythm that may be dangerous.
A note from Cleveland Clinic
Cardiac sarcoidosis is a condition that has the best outlook when it’s caught early and treated early. Be sure to take the medicine your healthcare provider prescribed for as long as they tell you that you need it. You’ll probably be taking it for a while. Let your provider know if you’re having side effects that bother you. Keep going to your checkups to make sure your condition is under control.
Last reviewed by a Cleveland Clinic medical professional on 07/12/2022.
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