What Is Rheumatic Heart Disease?
Triggered by an untreated strep infection, rheumatic heart disease is the most common heart disease for those under age 25. Here are the symptoms, treatments, complications, and ways to prevent it.
What is rheumatic heart disease?
Heart disease isn’t usually a young person’s illness.
But unlike more frequently diagnosed types of heart disease, which usually become a problem later in life, rheumatic heart disease typically impacts younger people. In fact, it’s the most common type of heart disease among those 25 and younger, according to the World Health Organization (WHO). Rheumatic heart disease causes more than 290,000 deaths each year.
You may not hear about it much in the U.S. or other wealthy countries since the vast majority of these mortalities occur in lower-income countries. It’s caused by rheumatic fever, an autoimmune reaction brought on by an untreated strep throat infection.
Strep throat is caused by group A strep, a type of bacteria that’s also known as Streptococcus pyogenes or group A Streptococcus. Strep throat symptoms can include a sore throat; fever; swollen glands; red, swollen tonsils; and difficulty swallowing.
Group A strep infections can also be accompanied by scarlet fever, sometimes known as scarlatina, which is a bright red rash over most of the body. (It’s most common in children ages 5 to 15.)
It’s important to see a doctor to get a throat swab to look for signs of strep throat, because it can progress to rheumatic fever.
Rheumatic fever can trigger temporary aches and arthritis. (More on its symptoms later.) If left untreated, it can lead to long-term damage and scarring within the heart. Rheumatic heart disease can result after one or repeated group A strep infections have gone untreated—or haven’t been treated soon enough with an appropriate antibiotic such as penicillin.
Who is most at-risk for rheumatic heart disease?
“Untreated or under-treated strep infections put a person at increased risk,” says Satjit Bhusri, MD, founder of Upper East Side Cardiology in New York City. “Children who get repeated strep throat infections are the most likely to acquire rheumatic fever and rheumatic heart disease.
“A recent history of strep infection or rheumatic fever is key to the diagnosis of rheumatic heart disease,” adds Dr Bhusri. “Symptoms of rheumatic fever vary and typically begin one to six weeks after a bout of strep throat.”
Kids and young adults who reside in highly populated areas with lack of access to adequate health care are most at-risk for rheumatic fever—and later, rheumatic heart disease, according to the WHO.
Currently, it’s most common in the South Pacific, Central and South Asia, sub-Saharan Africa, and the Middle East. Rheumatic heart disease has been eradicated in most of the rest of the world. (In case you missed it, here are 14 things we can learn from the countries with the lowest heart disease rates.)
Causes of rheumatic heart disease
There may be a small genetic component related to susceptibility for rheumatic fever. But more research is needed to understand why. Overall, strep infections and rheumatic fever are both most common in school-aged kids. In fact, it was the leading cause of death among children prior to 1960.
The scientific jury is still out about why group A streptococcal infections can lead to such a severe inflammatory response. But the best guess is that the antibodies that are sent out to attack the strep bacteria might get confused and accidentally go after healthy cells in the heart muscle, heart valves, and connective tissues.
This is similar to what happens with other autoimmune conditions like rheumatoid arthritis, in which the body attacks its own connective tissues that line joints.
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Symptoms of rheumatic heart disease
Besides a sore throat, rheumatic fever starts with a fever, fatigue, painful joints, and sometimes a rash. It can also cause chorea, which are jerky, involuntary movements.
As it progresses, rheumatic fever can cause carditis, or heart inflammation, that may lead to a faster heart rate, chest pain, swelling of the hands or feet, or shortness of breath. About half of rheumatic fever patients develop carditis.
The health risks and impacts of rheumatic heart disease
Mild carditis in young people may not impact them later in life. Severe carditis, however, can cause permanent heart damage, especially to the mitral valve that connects the upper and lower left chambers of the heart.
If this or other heart valves leak, surgery is necessary.
If left untreated, rheumatic heart disease can lead to problems later on, including a higher risk of a bacterial infection within the lining of the heart, pregnancy challenges, maternal mortality, a ruptured heart valve, or heart failure. Heart failure is a chronic condition in which the heart is enlarged and struggles to pump blood, and the result can be fluid accumulation in the lungs and limbs.
How rheumatic heart disease is diagnosed
To diagnose rheumatic heart disease, “a throat culture or blood test may be used to check for strep,” says Dr. Bhusri. “During a routine physical exam, a doctor may hear a patient’s heart murmur, which is caused by the blood leaking around the damaged valve. They may also hear a heart rub, if the inflamed heart tissues move or rub against each other.
“Along with a complete medical history and physical exam, tests used to diagnose rheumatic heart disease may include an echocardiogram, electrocardiogram, chest X-ray, cardiac MRI, and blood tests,” he adds.
Here’s a breakdown of how those tests work,:
- Blood tests: They can help detect infections or inflammation.
- Chest x-ray: An x-ray can help determine if the heart is enlarged.
- Echocardiogram: This is an ultrasound test that checks the heart muscle and valves for proper blood flow.
- Electrocardiogram (ECG or EKG): This records the heart’s electrical activity to help pinpoint heart muscle damage.
- Cardiac MRI: This painless imaging test uses radio waves and a powerful magnetic field to take detailed pictures of the heart.
Rheumatic heart disease treatments
“Treatment depends on how much damage has been done to the heart valves,” Dr. Bhusri says. “In severe cases, treatment may include surgery to replace or repair a badly damaged valve.”
Rheumatic heart disease treatment will vary based on the patient’s health history, how far the infection and rheumatic fever has progressed, and the child’s ability to endure certain therapies.
Anti-inflammatories—like aspirin, steroids or nonsteroidal anti-inflammatory drugs—may limit internal inflammation and reduce the risk of heart damage from rheumatic heart disease. Other medicines may be needed to manage heart failure.
There is no cure for rheumatic heart disease. Surgery can help repair the damage, and medicine may be able to treat symptoms of heart failure or thin the blood to reduce clot risk.
Is rheumatic heart disease preventable?
Yes, rheumatic heart disease can be prevented if rheumatic fever is treated with an antibiotic regimen. (Be sure to ask your doctor these important questions before you or your child begins taking antibiotics.)
“It’s important to take antibiotics as prescribed and to complete them as instructed, even if you feel better after a few days,” Dr. Bhusri says.
Strep throat infections are usually treated with penicillin, amoxicillin, or an injection of benzathine penicillin G (unless someone has an allergy to penicillin, in which case other antibiotics are used).
- Satjit Bhusri, MD, founder of Upper East Side Cardiology in New York City
- Children's Hospital of Philadelphia: "Rheumatic Fever and Rheumatic Heart Disease"
- World Health Organization (WHO): "Rheumatic Heart Disease"
- University of Rochester Medical Center: "Rheumatic Heart Disease"
- Children's Wisconsin: "Rheumatic Heart Disease and Rheumatic Fever"