SARCOIDOSIS AND THE HEART

The heart is affected by sarcoidosis in two ways. Firstly, sarcoidosis can occur in the heart itself (cardiac sarcoidosis) and secondly, the heart may be affected as a result of sarcoidosis in the lung (pulmonary hypertension). Both cases are rare but can have serious consequences.

Cardiac Sarcoidosis

Sarcoidosis of the heart is also called cardiac sarcoidosis. The inflammation and accumulation of white blood cells (granulomas) can occur in the heart in different places: the left or right ventricle, the interventricular septum, the papillary heart muscles, and (less often) the left-hand and right-hand atrium. They can also occur in the muscular guidance system (which ensures the heart beats), and the heart muscle itself. Cardiac sarcoidosis occurs in 5 to 15% of all sarcoidosis patients.

Symptoms:

  • irregular heartbeat (pounding and skipping)

  • dyspnea

  • chest pain

  • swelling of the legs (in later stages)

Pulmonary Hypertension

Pulmonary sarcoidosis (sarcoidosis of the lung) can also cause reduced heart function. The lung abnormalities increase the blood pressure in the heart as the pulmonary artery overloads the right ventricle.

This is also called ‘high blood pressure of the lungs’. It occurs in between 5 to 15% of patients with sarcoidosis.

Symptoms:

  • tiredness

  • shortness of breath, particularly with exertion

  • swelling of the ankles (and legs)

  • dizziness and/or fainting

  • chest pain

Techniques to Understand your Condition

ECG (Electro Cardio Gram): This provides information about heart function, heart rate, the size of the heart and the oxygen supply.

Holter monitor: This is a removable band worn for a longer time (usually 24 hours) to record cardiac function.

Echocardiography: This shows the pumping function of the heart as an image with the use of radio waves.

MRI (Magnetic Resonance Imaging): With the aid of a magnetic field and radio waves, this images the heart.
Nuclear Scans: These create images of the hearts metabolism within the body with the help of radioactive substances. Active sarcoidosis is made visible. Thallium scan and PET (Positron Emission Tomography) scans may also be used.

Biopsy of the Heart: Your doctor may take a sample piece of tissue to be investigated under the microscope (endomyocardial biopsy). However, due to granuloma distribution, cardiac sarcoidosis may be present even if biopsy results are negative – further tests may be required.

Electrophysiological Research: This uncovers conduction disturbances and / or ventricular arrhythmias using a catheter through the groin vein.

Lung Function Test: A breathalyser analyses the contents of the lungs and the rate of oxygen uptake.

Bike Test: Examines oxygen flow to the heart and may be used if you are experiencing any of the following:

  • irregular heartbeat

  • heart pounding or skipping

  • pressing, stinging or burning sensation in the chest

If necessary, you will be referred to a cardiologist.

Treatment

Condition Cardiac Sarcoidosis Pulmonary Hypertension
Overview Timely diagnosis and treatment with medication is important for a favourable prognosis. Medications can reduce symptoms but pulmonary hypertension is not curable.
Medications for Sarcoidosis Your doctor may prescribe medication to supress the sarcoidosis. This may be corticosteroids in tablet form; either prednisone or prednisolone. These medications must be used for a number of years, or even permanently. There are also other medicines that suppress the immune system, for example azathioprine, hydroxychloroquine and methotrexate. These drugs suppress the inflammation but do not medicate the heart itself. Your doctor may prescribe medication to supress the sarcoidosis. This may be corticosteroids in tablet form; either prednisone or prednisolone. These medications must be used for a number of years, or even permanently. There are also other medicines that suppress the immune system, for example azathioprine, hydroxychloroquine and methotrexate. These drugs suppress the inflammation but do not medicate the heart itself.
Treatment Your doctor may prescribe medications or implant a pacemaker or defibrillator (ICD). Your doctor may prescribe diuretics, endothelin antagonists or prostacyclins.
Transplant Heart transplant may be considered. This is very rare. Lung transplant may be considered. This is very rare.