Rheumatic Heart Disease

Rheumatic heart disease (RHD) is heart valve damage from rheumatic fever. Bacterial infections from group A Streptococcus can cause rheumatic fever. An infection like strep throat or scarlet fever triggers your body’s immune response, causing inflammation throughout your body, including in your heart.

Inflammation from rheumatic fever can lead to permanent damage to your heart valves. Damaged valves reduce the amount of blood that can move through your heart. They also may allow some blood to go in the wrong direction.

Rheumatic heart disease is rare in the U.S. It’s more common in parts of the world with limited access to antibiotics. About 306,000 people worldwide die of rheumatic heart disease each year. More than 40 million people in the world have RHD.

Children and teenagers with untreated strep are the most likely to get rheumatic fever, often between ages 5 and 15. Signs of heart damage can develop years after the infection and fever are gone. People often show signs of rheumatic heart disease, like chest pain or shortness of breath, as young adults. Treatment includes medicines, procedures and surgery.

Symptoms of RHD

Some people may have symptoms of heart involvement while they’re sick with rheumatic fever. But in most cases, rheumatic heart disease symptoms may not appear until years after a strep infection or rheumatic fever. People with heart damage may experience these signs and symptoms of RHD:

• Chest pain
• Fatigue
• Heart murmur
• Shortness of breath with exercise, at rest or when lying flat
• Swelling in their stomach, hands or feet
• Palpitations or abnormal heart rhythms like atrial fibrillation (AFib)
• Coughing up blood

Rheumatic heart disease causes

Heart valve inflammation from rheumatic fever causes rheumatic heart disease. The damage may happen right away. Or it can develop over time from repeated strep infections. Continuing inflammation leads to heart valve scarring and narrowing.

RHD isn’t contagious. But strep throat is. It can lead to rheumatic fever, the cause of RHD.

Risk factors

If you’re at risk of rheumatic heart disease, you may need penicillin injections every three to four weeks for five or more years. This can also keep rheumatic heart disease from getting worse by preventing the disease that causes rheumatic fever.

You’re at a higher risk for RHD if you:

• Don’t have easy access to healthcare or antibiotics
• Have repeated strep infections that go untreated
• Live in overcrowded or unsanitary conditions
• Are female

Complications of this condition

Rheumatic fever can affect your heart about 20 or 30 years after you've had an episode of it. If you had repeated episodes or were younger when you had rheumatic fever, you may see the effects of rheumatic heart disease at a younger age.

One or more of your heart valves can grow narrow (valve stenosis) or allow blood to flow backward in the wrong direction (regurgitation). Rheumatic heart disease tends to affect the mitral and aortic heart valves.

Rheumatic heart disease can lead to:

• Abnormal heart rhythms, like atrial fibrillation
• Inflamed heart muscle (myocarditis)
• Heart failure
• Infection in your heart valves (infective endocarditis)
• High blood pressure in your lung arteries (pulmonary hypertension)

Some of these conditions can increase your risk of a stroke or blood clots.

Rheumatic heart disease is especially dangerous if you're pregnant. Pregnancy increases the amount of blood in your body. Your heart has to work harder to pump the extra blood. This means a woman with damaged heart valves can have serious health issues during pregnancy. The fetus's health is also at risk.

If you know that you have a rheumatic heart valve, discuss pregnancy with your healthcare providers.

How doctors diagnose this condition

To diagnose rheumatic heart disease, a healthcare provider will assess your symptoms and perform a physical exam. They’ll also review your health history, especially any history of fevers or bacterial infections.

Your provider may use the following tests to diagnose RHD:

• Blood tests: Check for inflammation, a high immune response or markers of prior strep infection
• Chest X-ray: Looks for signs of heart failure
• Echocardiogram: Uses ultrasound to find leaky or narrowed heart valves
• Electrocardiogram (EKG): Checks your heart’s electrical activity for abnormal rhythms

How is it treated?

Rheumatic heart disease treatments can help you manage symptoms and may delay disease progress. But they can’t cure the condition. Treatments include:

• Medication: Your provider may recommend medication to manage heart failure or an abnormal heartbeat. Anticoagulants (blood thinners) can reduce the risk of a stroke or blood clots if you have AFib in the setting of a rheumatic mitral valve.
• Minimally invasive procedure: If your mitral valve is narrow but you don’t have other issues with it, a provider can perform a valvuloplasty to open the tight valve with a balloon. Providers do this with catheters they place through your groin.
• Heart valve repair: If you have severe rheumatic heart disease, you may need heart valve surgery. A surgeon may be able to repair a damaged heart valve.
• Heart valve replacement: A surgeon can replace the damaged valve with an artificial valve or a tissue valve.

When should I see my healthcare provider?

Visit your provider regularly to monitor your health. They can make sure you get antibiotics to prevent or treat strep throat.

Contact your provider if you experience new or worsened symptoms, including:

• Confusion
• Difficulty breathing
• Chest pain
• Swelling or pain in your lower body
• Coughing up blood

What can I expect if I have this condition?

The right treatments may delay or prevent heart failure in people with rheumatic heart disease. But the disease is permanent and requires long-term care. Without regular checkups, rheumatic heart disease can lead to severe heart failure. Lack of treatment can be fatal.

RHD is highly treatable if providers recognize it and do follow-ups. A valvuloplasty can be curative.

The length of time that you can live with RHD depends on how severe it is at diagnosis. In a study of indigenous people in Australia under 25, those who had severe rheumatic heart disease at diagnosis got worse fairly quickly. Some people with moderate disease improved, while others stayed the same or got worse. Ten years after diagnosis, 6 out of 10 people with mild rheumatic heart disease didn’t get worse.