Causes and Risk Factors of Rheumatic Heart Disease

Rheumatic heart disease (RHD) is heart valve damage that arises as a complication of rheumatic fever years after the illness has resolved. It develops as a result of chronic inflammation and scarring of the heart valves triggered by rheumatic fever—an inflammatory autoimmune disease that can develop as a result of strep throat or scarlet fever. If not treated, rheumatic heart disease can progress to heart failure.

Causes

Rheumatic heart disease is a direct result of rheumatic fever, an inflammatory disease caused by infection with group A streptococcus bacteria, which are responsible for illnesses such as strep throat and scarlet fever. Rheumatic fever affects connective tissue throughout the body, particularly in the heart, joints, skin, and brain.

When the heart is involved, inflammation can develop on the surface (pericarditis), within the valves (endocarditis), or in the heart muscle itself (myocarditis). Over time, people who develop endocarditis can experience permanent damage to one or more of the ​four heart valves, causing them to become narrow or leaky.

Children between 6 and 16 who get repeated strep infections are at the highest risk for rheumatic fever and, consequently, rheumatic heart disease. Although strep throat is common in the United States, rheumatic fever is rare in most developed countries thanks to antibiotics.

Genetics

Genetics may play a role in rheumatic heart disease, as there appears to be 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 have revealed that differences in the expression of various genes involved in the immune response may contribute to susceptibility to rheumatic fever. A 2020 study showed a genetic connection to RHD susceptibility and immunoglobulin heavy chain (IGH) locus on chromosome 14. This study highlights that the 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 primary risk factor for rheumatic fever is failing to take steps to prevent infection from strep bacteria (as well as other infectious microbes). This means infrequent handwashing, especially after sneezing or coughing or before eating.

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

If your healthcare provider 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.

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 healthcare provider may prescribe aspirin, steroids, or non-steroidal inflammatory drugs (NSAIDs) to reduce inflammation.

A Word From Verywell

If you happen to be among the very few people who develop rheumatic heart disease, it's important that your heart is regularly monitored with an echocardiogram and other tests. These routine exams can determine if and when you might need valve replacement surgery, as 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.