Acute Rheumatic Fever and Rheumatic Heart Disease: Symptoms, Diagnosis, Treatment, and Prevention

Assoc. Prof. Sezen Bağlan Uzunget, MD
Cardiologist

What Is Acute Rheumatic Fever?

Acute rheumatic fever (ARF) is an immune-mediated inflammatory disease that may develop after a throat infection caused by group A Streptococcus.

It most commonly affects children and adolescents, although adults can also develop the disease.

Acute rheumatic fever may involve the heart, joints, nervous system, and skin.

Its most important long-term complication is permanent heart valve damage, known as rheumatic heart disease (RHD).

How Can a Throat Infection Affect the Heart?

Acute rheumatic fever does not result from direct bacterial infection of the heart valves.

Instead, the immune response triggered by streptococcal infection may cross-react with the body's own tissues.

Symptoms commonly appear approximately two to four weeks after streptococcal pharyngitis, although certain manifestations, such as Sydenham chorea, may develop later.

Not every sore throat or streptococcal infection leads to acute rheumatic fever.

What Are the Symptoms of Acute Rheumatic Fever?

Migratory Arthritis

Painful inflammation may affect large joints, including the knees, ankles, elbows, and wrists.

Symptoms may improve in one joint while appearing in another.

Carditis

Inflammation may involve the heart, particularly its valves.

Possible symptoms include:

• Palpitations
• Shortness of breath
• Fatigue
• Chest discomfort
• A new heart murmur
• Heart failure in severe cases

Some patients have subclinical carditis detected only by Doppler echocardiography.

Sydenham Chorea

This neurological manifestation causes involuntary, irregular movements.

Patients may experience impaired coordination, changes in handwriting, or emotional symptoms.

Erythema Marginatum

A characteristic, usually non-itchy, ring-shaped rash may occur.

Subcutaneous Nodules

Small, painless nodules may develop near tendons or bony prominences.

They are uncommon and often associated with carditis.

How Does Acute Rheumatic Fever Affect Heart Valves?

The mitral valve is most commonly affected, followed by the aortic valve.

During the acute phase, mitral or aortic regurgitation may develop.

Over time, repeated inflammation and healing can cause valve thickening, fusion, and restricted movement.

Long-term consequences may include:

• Mitral stenosis
• Mitral regurgitation
• Aortic stenosis or regurgitation
• Multivalvular disease

Acute rheumatic fever describes the inflammatory illness, while rheumatic heart disease refers to the chronic structural valve damage that may follow.

How Is Acute Rheumatic Fever Diagnosed?

Diagnosis is based on clinical findings, evidence of preceding group A streptococcal infection, and the revised Jones criteria.

Major Criteria

• Carditis, including subclinical carditis
• Arthritis
• Sydenham chorea
• Erythema marginatum
• Subcutaneous nodules

The interpretation of joint manifestations varies according to whether the population is classified as low risk or moderate-to-high risk.

Minor Criteria

• Fever
• Arthralgia
• Elevated inflammatory markers
• Prolonged PR interval on ECG

Thresholds for certain minor criteria depend on population risk.

For an initial episode, evidence of preceding streptococcal infection together with two major criteria or one major and two minor criteria generally supports the diagnosis.

Important exceptions include isolated Sydenham chorea and certain late presentations of carditis.

Does an Elevated ASO Level Confirm Rheumatic Fever?

No.

Antistreptolysin O (ASO) is an antibody test that may provide evidence of recent streptococcal infection.

An elevated ASO level alone does not establish a diagnosis of acute rheumatic fever.

ASO may remain elevated for weeks or months after infection.

It also does not prove that heart valve damage has occurred.

Which Tests Are Used?

Electrocardiography

ECG may reveal PR prolongation or other rhythm and conduction abnormalities.

Echocardiography

Doppler echocardiography is essential for evaluating cardiac involvement.

It is important in suspected acute rheumatic fever even when no murmur is audible.

Blood Tests

Inflammatory markers such as CRP and ESR may be elevated.

ASO, anti-DNase B, and appropriate microbiological testing may help establish evidence of preceding streptococcal infection.

No single test establishes the diagnosis in every patient.

How Is Acute Rheumatic Fever Treated?

Treatment has several components.

Eradication of Streptococcal Infection

Appropriate antibiotic treatment is given to eradicate group A Streptococcus, even when symptoms of active pharyngitis are no longer present.

Penicillin is a principal treatment option in suitable patients.

Treatment of Arthritis

Anti-inflammatory medications such as aspirin or naproxen may be used according to clinical circumstances.

Management of Carditis

Patients with carditis require assessment of cardiac involvement.

Heart failure treatment may be necessary in severe cases.

Corticosteroids may be considered in selected patients with severe carditis, although their ability to prevent permanent valve damage has not been established.

Management of Sydenham Chorea

Mild cases may improve with supportive care.

More severe cases may require neurological assessment and symptomatic or immunomodulatory treatment.

Why Is Secondary Penicillin Prophylaxis Important?

Repeated group A streptococcal infections can trigger recurrent rheumatic fever and increase the risk of progressive valve damage.

Long-term antibiotic prophylaxis is therefore a key part of management.

Intramuscular benzathine penicillin G is commonly used at regular intervals.

A four-week schedule is widely used, although shorter intervals may be appropriate in higher-risk circumstances.

The regimen should follow individual risk assessment and relevant local recommendations.

How Long Should Penicillin Prophylaxis Continue?

Commonly used recommendations include:

Clinical condition General duration
ARF without carditis Five years after the last episode or until age 21, whichever is longer
Carditis without residual valve disease Ten years after the last episode or until age 21, whichever is longer
Carditis with residual valve disease At least ten years after the last episode or until age 40, whichever is longer

Some patients with severe residual disease or ongoing high exposure risk may require longer or lifelong prophylaxis.

Duration should be individualized.

When Is Valve Intervention Necessary?

Patients with chronic rheumatic heart disease may require intervention if significant valve dysfunction develops.

Mitral Stenosis

Percutaneous balloon mitral commissurotomy may be appropriate for selected patients with severe rheumatic mitral stenosis and favorable valve anatomy.

Left atrial thrombus and more than mild mitral regurgitation are important contraindications.

Mitral or Aortic Regurgitation

Severe regurgitation may require surgical treatment according to symptoms, ventricular function, and cardiac chamber dimensions.

Valve Replacement

Surgical valve replacement may be necessary when valve repair or catheter-based treatment is unsuitable.

Rheumatic Mitral Stenosis and Atrial Fibrillation

Atrial fibrillation is an important complication of rheumatic mitral disease.

It increases the risk of left atrial thrombus formation and stroke.

Patients with atrial fibrillation and moderate-to-severe rheumatic mitral stenosis generally require anticoagulation with a vitamin K antagonist such as warfarin.

Direct oral anticoagulants should not replace warfarin in this specific patient group.

Can Acute Rheumatic Fever Be Prevented?

Timely diagnosis and appropriate antibiotic treatment of group A streptococcal pharyngitis can reduce the risk of a first episode.

However, not every sore throat requires antibiotics.

Most viral throat infections do not benefit from antibiotic treatment.

Appropriate clinical assessment and testing are important.

What Follow-Up Is Needed?

Long-term care depends on the severity of residual valve disease.

It may include:

• Regular cardiology assessment
• Periodic echocardiography
• Monitoring for atrial fibrillation
• Assessment for heart failure symptoms
• Adherence to secondary antibiotic prophylaxis
• Pre-pregnancy cardiac evaluation when appropriate

Pregnancy planning is particularly important in women with significant rheumatic mitral stenosis.

Frequently Asked Questions

Does every throat infection cause rheumatic fever?

No. Acute rheumatic fever occurs in susceptible individuals after certain group A streptococcal infections.

Does a high ASO level mean I have rheumatic heart disease?

No. Elevated ASO alone does not establish either acute rheumatic fever or heart valve damage.

Can acute rheumatic fever resolve completely?

The acute inflammatory illness may resolve, but some patients develop permanent valve damage.

Can rheumatic heart disease become symptomatic years later?

Yes. Rheumatic mitral stenosis may become apparent many years after the original illness.

Why are long-term penicillin injections necessary?

They help prevent recurrent streptococcal infections from triggering additional rheumatic fever episodes and worsening valve damage.

Does everyone with rheumatic heart disease need surgery?

No. Intervention is reserved for patients with significant valve disease and appropriate clinical indications.

This article is intended for general educational purposes. Diagnosis, antibiotic prophylaxis, and treatment of acute rheumatic fever and rheumatic heart disease should be individualized according to age, clinical findings, and the severity of cardiac involvement.

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