Chronic Rheumatic Fever and Rheumatic Heart Disease

From strep throat to heart damage

Acute rheumatic fever develops 2 to 4 weeks after an untreated group A streptococcal throat infection (strep throat), mainly in children aged 5 to 15. The immune system makes antibodies against strep that also attack similar proteins in the heart, joints, brain and skin. Signs include fever, painful swollen joints that move from one to another, a heart murmur, jerky movements (Sydenham chorea) and skin nodules or rash.

The joint and brain effects resolve. The heart effects may not. Each attack inflames the heart (carditis), and the valves heal with scarring. Repeated attacks cause more damage, which is why preventing recurrences is the foundation of treatment.

How rheumatic heart disease affects the valves

Mitral stenosis is the classic lesion of chronic rheumatic heart disease.

Symptoms

Many people have no symptoms for 10 to 20 years after rheumatic fever. When valve damage becomes significant:

• shortness of breath, first on exertion and later at rest or lying flat
• tiredness and reduced ability to exercise
• palpitations, often from atrial fibrillation
• swelling of the ankles and legs
• coughing, sometimes with blood (in severe mitral stenosis)
• chest pain or fainting with aortic valve disease
• symptoms that appear or worsen suddenly during pregnancy

Complications

• Atrial fibrillation and blood clots causing stroke
• Heart failure
• Infective endocarditis: infection of damaged valves
• Pulmonary hypertension
• Pregnancy complications for both mother and baby
• premature death if untreated

Who is at risk

Rheumatic heart disease affects tens of millions of people worldwide, mostly in sub-Saharan Africa, South Asia, the Pacific and among Indigenous populations in Australia and New Zealand. Overcrowded housing and limited access to antibiotics for sore throats are major risk factors. In the US it is now seen mainly in older adults who had rheumatic fever as children and in people born in high-burden countries.

Diagnosis

• History of rheumatic fever or recurrent sore throats in childhood
• Physical exam: heart murmurs, irregular rhythm, signs of heart failure
• Echocardiogram: the key test; shows thickened, restricted or leaking valves. Screening echocardiograms can detect silent disease in high-risk communities
• ECG for atrial fibrillation and heart strain
• Chest X-ray for heart enlargement and lung congestion
• Blood tests: ASO and anti-DNase B antibodies show recent strep infection during an acute attack

Treatment

Secondary prophylaxis: preventing recurrences

The most important treatment. Long-acting penicillin injections, typically every 3 to 4 weeks, prevent new strep infections and new attacks. Oral penicillin or alternatives are used for people who cannot have injections. How long it continues depends on age and severity: often at least 10 years after the last attack or until age 21, and sometimes until age 40 or for life when valve disease is moderate or severe. Consistent prophylaxis can allow mild valve damage to improve.

Medicines for symptoms and complications

• diuretics for fluid buildup
• beta-blockers or other drugs to control heart rate
• anticoagulants for atrial fibrillation or severe mitral stenosis, to prevent stroke
• treatment of heart failure

Valve procedures

• Balloon mitral valvotomy: a catheter-based procedure to open a narrowed mitral valve, highly effective when the valve is suitable
• Valve repair: often preferred in young people
• Valve replacement: mechanical valves need lifelong blood thinners; tissue valves wear out over years

Other care

• excellent dental hygiene and regular dental checks to reduce endocarditis risk
• pregnancy planning with a cardiologist
• vaccination against flu and pneumococcus

Preventing rheumatic fever

Treating strep throat with a full 10-day course of penicillin or amoxicillin prevents rheumatic fever. That is why sore throats with fever in children, especially in high-risk communities, should be tested and treated.

Frequently asked questions

What is chronic rheumatic fever?

It describes the lasting effects of rheumatic fever, mainly permanent scarring of the heart valves called rheumatic heart disease. Repeated attacks of rheumatic fever after strep infections cause most of the damage, which may not cause symptoms for many years.

What are the long-term effects of rheumatic fever?

The main long-term effect is rheumatic heart disease: narrowing or leaking of the heart valves, especially the mitral valve. It can lead to atrial fibrillation, stroke, heart failure, valve infection and pregnancy complications. Joint and brain symptoms of acute rheumatic fever do not usually last.

How long do you need penicillin after rheumatic fever?

It depends on age and whether the heart was affected. Typical schedules continue for at least 10 years after the last attack or until age 21, and longer, sometimes until 40 or for life, if valve disease is moderate or severe. Your cardiologist sets the duration.

Can rheumatic heart disease be cured?

Valve damage cannot be reversed with medicine, but mild damage can improve with consistent penicillin prophylaxis. Balloon procedures, valve repair or replacement can restore valve function, and medicines control symptoms. Lifelong follow-up is needed.

Is rheumatic fever contagious?

Rheumatic fever itself is not contagious, but the strep throat infection that triggers it is. Treating strep throat promptly with antibiotics prevents rheumatic fever and protects others from infection.