Page created: 12 April 2021

Heart Involvement in Systemic Sclerosis (SSc)

The Heart

Heart disease becomes more common as we get older and systemic sclerosis (SSc, also known as scleroderma), usually starts in the 50s and 60s, so it is not surprising that heart disease is commonly found in people living with this condition. Systemic sclerosis can also directly affect the heart, placing some people at a greater risk of heart disease.

What is Heart Involvement in Systemic sclerosis (SSc)?

For most people with systemic sclerosis, any involvement of the heart is so mild that it is very difficult to be sure whether it is present or not. Very minor involvement of the heart causes an inability to reach high levels of exercise, but because of lung disease and musculoskeletal problems, reaching very high levels of exercise is usually not possible in people with systemic sclerosis anyway.

Cardiac fibrosis or scarring of the heart muscle can be found in around one-in-five people with systemic sclerosis. Usually this is just something noticed on scans, but rarely it can become very severe and can make the heart unable to relax and can cause heart failure.

The prevalence of heart involvement in SSc is difficult to determine due to the numerous possible cardiac manifestations, the applied diagnostic tools and diverse patient populations.

Five common types of heart disease in systemic sclerosis include:

  • Coronary heart disease – systemic sclerosis may slightly increase your risk of developing the most common form of heart disease. Coronary heart disease causes angina, heart attacks and heart failure
  • Systemic sclerosis heart disease is the most common type of heart disease directly caused by systemic sclerosis. Around one-in-ten patients with systemic sclerosis have high blood pressure in the lung arteries. This increases the work for the right side of the heart, causing it to thicken and eventually fail
  • Valvular heart disease, especially aortic stenosis (narrowing of the main valve that controls blood flow leaving the heart for the rest of the body), is common in elderly people. It seems to occur just as frequently in people with systemic sclerosis as everyone else. Symptoms are similar to coronary disease (above), although blackouts are more likely to occur
  • Hypertensive heart disease – this happens when the heart has to work harder to deal with high blood pressure. The muscle of the heart is thickened, which can lead to rhythm problems (palpitations) and heart failure. High blood pressure affects nearly 50% of middle-aged people and is more common in systemic sclerosis because of kidney problems
  • Rhythm problems - atrial fibrillation (a completely irregular heart rhythm) is one of the commonest rhythm disorders of elderly people, and tends to develop where there is fibrosis of the heart

Other heart problems that may arise include:

  • Pericardial effusions – this is a collection of fluid in the sac that the heart sits in. It rarely causes any problems. It can be due to inflammation of the lining of the pericardial sac or increased pressure within the heart (for example, when the heart is responding to high blood pressure in the lungs)
  • Cardiac fibrosis or scarring of the heart muscle occurs in around one-in-five people with systemic sclerosis. Usually this is only picked up during scans, but very rarely it can become very severe and cause heart failure
  • Small vessel disease - coronary artery disease affects the large vessels on the surface of the heart. In systemic sclerosis, the tiny vessels within the heart muscle also show abnormalities that are more prone to spasm and the failure to relax properly. This can cause very tiny areas of damage widely spread throughout the heart, which leads to a slightly weaker heart and slowed relaxation
  • Myocarditis – sometimes inflammation occurs within the heart. Patients with myocarditis have symptoms of heart failure and rhythm problems. This can be identified fairly easily with blood tests and scans and treated with immunosuppression drugs

More information and finding support

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