Causes and Risk Factors of Rheumatic Heart Disease

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Rheumatic heart disease (RHD) is a serious complication of rheumatic fever. It refers to the chronic heart valve damage that can develop years after a bout of rheumatic fever. It can often lead to heart failure if left untreated.

Rheumatic fever is an inflammatory autoimmune disease that can develop from strep throat or scarlet fever. When this happens, the immune system sets off a chronic inflammation response and calcium crystals gradually deposit in and around the heart valves, which damages the heart.

Rheumatic fever is not contagious, but the underlying infections that cause it are.

echocardiogram and ECG
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Common Causes

Current or recent group A streptococcus bacteria infections, such as strep throat infection, scarlet fever, and rheumatic fever, cause rheumatic heart disease. Children who get repeated strep infections are at the highest risk for rheumatic fever and, consequently, rheumatic heart disease.

Rheumatic fever is an inflammatory disease that is far-reaching. It affects many connective tissues, particularly in the heart, joints, skin, or brain. Rheumatic fever can trigger heart inflammation, either on the surface of the heart (pericarditis), within the heart (endocarditis), or involving the heart muscle itself (myocarditis).

In people with endocarditis (inflammation or infection of the heart valves), the inflammatory response can cause permanent scarring over time to one or more of the ​four heart valves. This can lead to narrowed or leaking valves. Once this type of damage occurs, the heart can't function properly.

Rheumatic fever can happen at any age but typically occurs in children aged 6 years old to 16 years old. Although strep throat is common in the United States, rheumatic fever is now rare in most developed countries thanks to antibiotics.

Rheumatic heart disease affects around half of all people who have had an acute rheumatic fever episode. Most cases are diagnosed 10 years to 20 years after being sick. People are at greatest risk for rheumatic heart disease if they've had rheumatic fever more than once.

Genetics

Genetics may play a role in what makes some people more prone to developing rheumatic heart disease. Scientists report a genetic link that makes some people more susceptible to rheumatic heart disease, but little is known about the genetic factors that increase or decrease the risk of developing RHD. Some studies show that differences in the expression of various genes involved in the immune response may contribute to rheumatic fever susceptibility. A 2020 study showed a genetic connection to RHD susceptibility and immunoglobulin heavy chain (IGH) locus on chromosome 14. This study highlights that your risk of getting rheumatic fever with an RHD family history is nearly fivefold higher than if you have no family history of RHD.

Lifestyle Risk Factors

The best way to avoid rheumatic heart disease is by preventing strep throat and scarlet fever or treating them promptly with antibiotics. Handwashing is one of the most effective ways to prevent infection. Washing your hands after sneezing or coughing and before eating is especially important. If your doctor prescribes an antibiotic to treat strep throat or scarlet fever, it's critical to follow the instructions and finish the entire course even if you start to feel better.

Crowded places are high-risk for contracting group A strep. Daycare centers, schools, and military training facilities are especially risky. 

Once you've had rheumatic fever, you are more likely to get it again if you have another strep infection. Therefore, it is vital to prevent future episodes. If you've had rheumatic fever, you may be put on a continuous antibiotic treatment plan as a form of prophylactic (preventive) therapy. Your doctor may prescribe aspirin, steroids, or non-steroidal inflammatory drugs (NSAIDs) to reduce inflammation.

People who have experienced acute rheumatic fever should undergo an annual physical exam to check for a possible heart murmur or any other heart abnormality.

A Word From Verywell

It's imperative to get regularly monitored with an echocardiogram and other diagnostic tools if you're diagnosed with rheumatic heart disease. These routine exams can help determine if and when you need valve replacement surgery since heart valve problems tend to worsen over time.

The timing of valve replacement surgery is critical. The goal is to operate before the person's quality of life is seriously diminished, but not so early as to risk the person outliving the artificial valve's lifespan. Speak to your doctor to determine what action plan is best for you.

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