Cardiac Sarcoidosis

What is cardiac sarcoidosis?

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.

How common is cardiac sarcoidosis?

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.

What are the symptoms?

Cardiac sarcoidosis symptoms may include:

• Chest pain.
• Shortness of breath.
• Fainting.
• Tiredness.
• Coughing.
• Swelling in your legs.
• A tight feeling in your chest.
• Abnormal heartbeats that give you heart palpitations.

What causes cardiac sarcoidosis?

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.

How is cardiac sarcoidosis diagnosed?

To make a cardiac sarcoidosis diagnosis, your healthcare provider will:

• Give you a physical exam.
• Talk with you about your medical history.
• Order tests to check for abnormal heart rhythms.

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.

What tests will be done to diagnose cardiac sarcoidosis?

Tests to make a cardiac sarcoidosis diagnosis include:

• Electrocardiogram (EKG).
• Echocardiogram (sometimes, the transthoracic type).
• Holter monitor.
• Positron emission tomography (PET) scan.
• Heart MRI.

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).

How is cardiac sarcoidosis treated?

Your healthcare provider has several options for cardiac sarcoidosis treatment. These treatments include:

• Corticosteroid medication for inflammation.
• Other drugs that keep your immune system from overreacting.
• Medicine to treat irregular heartbeats.
• Pacemaker.
• Implantable cardioverter defibrillator (ICD).
• Catheter ablation for abnormal heart rhythms.

If you have a bad case, you can end up with heart failure. In that situation, you may need a heart transplant.

What medications/treatments are used?

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.

Complications/side effects of the treatment

Prednisone side effects include:

• Osteoporosis.
• Infections.
• High blood pressure.
• Elevated blood sugar.
• Mood swings.
• Insomnia.

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.

How serious is cardiac sarcoidosis?

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.

Outlook for cardiac sarcoidosis

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.

How long can you live with cardiac sarcoidosis?

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.

Is cardiac sarcoidosis fatal?

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.

How do I take care of myself?

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.

When should I see my healthcare provider?

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.

When should I go to the ER?

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.

What questions should I ask my doctor?

• Can I manage my condition with medicines?
• What’s the outlook for me specifically?
• How well is my heart’s left ventricle working?