Specialties & Conditions Rheumatic Heart Disease
Rheumatic Heart Disease
What Is Rheumatic Heart Disease?
Rheumatic heart disease refers to heart valve damage that occurs after a child has rheumatic fever. Rheumatic fever can occur when a child doesn’t get proper antibiotic care for group A Streptococcus bacterial infections, such as strep throat and scarlet fever.
Rheumatic heart disease is more common in lower-income countries that don’t have quality medical services. In the U.S., it’s more likely to affect economically disadvantaged children who live in crowded housing and have limited access to healthcare. Globally, more than 55 million people have rheumatic heart disease.
A child with rheumatic heart disease will need lifelong heart care and monitoring. Some children need heart surgery. With proper medical care, children with rheumatic heart disease enjoy long and active lives.
What Parts of the Heart Does Rheumatic Heart Disease Affect?
Rheumatic heart disease can affect any of the four heart valves. These valves open and close to allow blood to flow between heart chambers.
Rheumatic heart disease is more likely to affect these heart valve functions:
- Mitral valve: Almost all of the time, the condition affects the mitral valve. This valve allows blood to flow from the heart’s upper left chamber (atrium) into the lower left chamber (ventricle).
- Aortic valve: In about half of the cases, the rheumatic heart disease affects the aortic valve. The aortic valve allows blood to flow from the heart’s left ventricle to the aorta. The body’s largest blood vessel, the aorta, carries oxygen-rich blood to the rest of the body.
- Aortic valve and mitral valve: Approximately 20 percent of the time, the condition affects both the mitral and aortic valves.
- Tricuspid valve: In 10–20 percent of cases, the condition affects the tricuspid valve along with the aortic valve and/or mitral valve. The tricuspid valve allows blood to flow from the heart’s right atrium into the right ventricle.
- Pulmonary valve: It’s extremely rare for the disease to affect the pulmonary valve. This valve allows oxygen-poor blood to flow from the heart’s right ventricle to the pulmonary trunk (large, short artery) and into the lungs for oxygen.
What Are the Complications of Rheumatic Heart Disease?
Rheumatic heart disease causes heart valve inflammation (valvulitis) and scarring. The disease permanently damages one or more heart valves, affecting blood flow.
Rheumatic heart disease can cause:
- Regurgitation: A valve doesn’t fully close, allowing blood to flow backward or leak into a heart chamber.
- Stenosis: A valve doesn’t fully open, narrowing the passage of blood. Aortic stenosis affects the aortic valve.
- Cardiac arrhythmia: Damage to the heart muscle and valves can affect the heart’s electrical system, which controls the heart’s rhythm. The heart may beat too fast or too slow (arrhythmia).
- Carditis: Inflammation can affect the heart muscle (myocarditis) or the pericardial sac that surrounds the heart (pericarditis). Myocarditis and pericarditis weaken the heart’s ability to pump blood.
- Pulmonary hypertension: Improper blood flow through the heart can cause high blood pressure in the lungs (pulmonary hypertension).
- Stroke: A leaky heart valve increases the risk of blood clots that cause strokes.
- Heart failure: The heart has to work harder when heart valves leak or become too narrow. Over time, the heart can weaken, leading to heart failure.
What Causes Rheumatic Heart Disease in Children?
Rheumatic fever causes rheumatic heart disease. A child can get rheumatic fever when they have untreated or under-treated strep throat or scarlet fever (red rash, high fever and severe sore throat). Group A strep bacteria cause these infections.
Rheumatic fever is an immune response to this bacterial infection. Your child’s immune system mistakenly attacks healthy cells, causing inflammation. In addition to affecting the heart, rheumatic fever can damage a child’s joints, skin and brain.
Symptoms of rheumatic fever typically appear two to three weeks after a strep infection. Your child may have:
- Extreme fatigue
- Fever
- Involuntary hand, arm and facial movements (chorea)
- Painful, tender joints
What Are the Risk Factors for Rheumatic Heart Disease?
Rheumatic fever is more common in children ages 5 to 15. A child is more at risk for rheumatic fever and heart disease if they:
- Currently have or previously had an undiagnosed strep throat or infection
- Don’t get antibiotics for strep infections
- Don’t take all of the antibiotics as prescribed to treat a strep infection
- Have recurrent strep throat infections (even with antibiotic treatment)
- Lack access to healthcare
- Live in crowded areas where bacteria can easily spread
What Are the Signs and Symptoms of Rheumatic Heart Disease?
Signs of rheumatic heart disease may not appear until years after a child has recovered from rheumatic fever or strep infection. Some children have mild strep throat or rheumatic fever symptoms that are mistaken for a cold or flu. These children don’t get antibiotics because they’re unaware they have a strep infection.
A child with rheumatic heart disease may have these symptoms:
- Chest pain
- Difficulty breathing or shortness of breath
- Extreme fatigue
- Heart murmur
- Heart palpitations
- Swollen hands, feet or stomach
How Is Rheumatic Heart Disease Diagnosed?
Pediatric cardiologists at Lurie Children’s are experts at diagnosing and managing rheumatic heart disease. The doctor will review your child’s symptoms and medical history (including previous strep infections and fevers). They will listen for a heart murmur. At Lurie Children’s, we offer advanced cardiac imaging and other tests to quickly and accurately diagnose rheumatic heart disease.
Your child may receive:
- Blood test to check for inflammation and antibodies (proteins) that indicate a current or previous strep infection
- Electrocardiogram (EKG/ECG) to check for arrhythmias
- Echocardiogram (echo or heart ultrasound) to view the heart and heart valves
How Is Rheumatic Heart Disease Treated?
A child with rheumatic heart disease needs lifelong heart care and treatments to prevent further heart damage. Your child will see a pediatric cardiologist at Lurie Children’s regularly for heart tests and exams. These checkups help detect signs of progressing heart damage.
Medications
Children with rheumatic heart disease are at risk of having rheumatic fever again. Additional bouts of rheumatic fever, or any type of bacterial infection, can cause more heart damage. To protect your child’s heart and overall health, they will take an antibiotic (usually penicillin) every day or receive a monthly shot at their provider’s office. This prophylactic treatment prevents, rather than treats, a bacterial infection. In most cases, a person with rheumatic heart disease takes antibiotics for life.
Other medications that can help include:
- Angiotensin-converting enzyme (ACE) inhibitors to improve blood flow and ease strain on the heart
- Blood thinners to prevent blood clots and strokes when a child has an arrhythmia
- Diuretics to get rid of excess fluid that affects the heart or lungs
Valvuloplasty
Valvuloplasty is a minimally invasive procedure for stenosis that opens a narrowed heart valve. Skilled pediatric interventional cardiologists at Lurie Children’s perform this treatment through a catheter tube (thin, flexible) threaded through a blood vessel. Your child receives anesthesia to sleep through the procedure.
During balloon valvuloplasty, a doctor:
- Inserts a catheter into a blood vessel through a tiny cut in the groin
- Moves a deflated balloon through the catheter to reach the heart
- Inflates the balloon to open the narrowed valve
- Deflates and removes the balloon
- Closes the small incision with glue or stitches
Heart Surgery
Our care team may recommend heart valve repair surgery if your child has severe regurgitation or stenosis. Pediatric heart surgeons at Lurie Children’s are experts in heart valve repair, which typically takes place during the teen years or young adulthood.
In very rare instances, surgeons may replace the heart valve. More often, this surgery happens in adulthood.
What Is Lurie Children’s Approach to Caring for Children With Rheumatic Heart Disease?
The Heart Center at Lurie Children’s is nationally rated by Newsweek and U.S. News & World Report for its exceptional pediatric cardiology and cardiac surgery programs. We offer:
- Specialized clinic: Children with rheumatic heart disease see specialists at our dedicated Center for Kawasaki Disease. While the diseases differ, they both require deep expertise in managing acquired heart disease that develops after an illness or infection.
- Comprehensive care: We have all the experts your child needs, including rheumatologists who treat joint pain and infectious disease experts who manage chronic bacterial infections.
- Safe heart tests: We use 3D echo technology to get clear images of the heart valves. Unlike some heart tests that use X-ray fluoroscopy, a 3D echo doesn’t expose your child to radiation. Testing takes place in the state’s only pediatric echo lab accredited by the Intersocietal Accreditation Commission (IAC) for Echocardiography.
- Surgical expertise: Children get heart valve surgery at our Level I Children’s Surgery Center. This rating is the highest distinction from the American College of Surgeons (ACS). The Regenstein Cardiac Care Unit (CCU) at Lurie Children’s keeps your child in the same private room from admission to discharge.
- Support into adulthood: While rheumatic heart disease isn’t present at birth like congenital heart disease, the care needs are similar. As your child gets older, we aid their transition to the Lurie Children’s Adult Congenital Heart Disease (ACHD) Program and the Bluhm Cardiovascular Institute at Northwestern Memorial Hospital.
The Heart Center at Lurie Children’s
Specialists at The Heart Center at Lurie Children’s are nationally recognized for their expertise in managing complex heart conditions. Our pediatric cardiologists provide expert care to keep heart valve disease from worsening. Our interventional cardiologists and heart surgeons perform hundreds of procedures every year with exceptional outcomes. We offer compassionate, team-based care that promotes an active childhood and helps your family confidently manage a chronic heart condition.