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Clinical Subject Page

Rheumatic Heart Disesae

Also called

Chronic, heart-related Conditions

ICD-10

I09.9

Specialty

Cardiology

Onset

Chronic

Reviewed

June 2026
On This Page

Overview

Rheumatic heart disease (RHD) is a chronic heart condition caused by permanent damage to the heart valves following acute rheumatic fever, which occurs after an untreated group A β-hemolytic streptococcal (strep throat) infection.

Etiology & Risk Factors

  • Untreated or inadequately treated group A β-hemolytic Streptococcus (Streptococcus pyogenes) infection
    • Most commonly strep throat (acute tonsillitis/pharyngitis)
    • Less commonly after group A streptococcal skin infections
  • Acute rheumatic fever usually develops 2–4 weeks after the infection.
  • The resulting inflammation can permanently damage the heart valves, especially the mitral valve, leading to rheumatic heart disease.

Risk Factors

  • Untreated or recurrent group A streptococcal throat infections
  • Previous episode of acute rheumatic fever
  • Age 5–15 years (highest risk for acute rheumatic fever)
  • Living in resource-limited or overcrowded settings, where streptococcal infections are more common
  • Limited access to prompt antibiotic treatment for streptococcal infections

Pathophysiology

  • Untreated group A β-hemolytic streptococcal (GAS) throat infection occurs.
  • The body produces antibodies against the streptococcal M protein.
  • Due to molecular mimicry, these antibodies cross-react with cardiac proteins (especially myosin).
  • This causes a type II hypersensitivity reaction, leading to acute rheumatic fever (ARF) and inflammation of the heart (carditis/valvulitis).
  • Recurrent or severe inflammation causes permanent scarring and fibrosis of the heart valves, resulting in rheumatic heart disease (RHD).
  • The mitral valve is most commonly affected, followed by the aortic valve, leading to valve stenosis, regurgitation, or both.

Simple Flow

Untreated strep throat → Antibodies against streptococcal M protein → Molecular mimicry → Autoimmune attack on heart valves → Carditis/valvulitis → Valve scarring and fibrosis → Chronic rheumatic heart disease

Clinical Presentation

General Symptoms

  • Fever

  • Malaise

  • Fatigue

Cardiac Manifestations

  • Carditis (pancarditis) – inflammation of the endocardium, myocardium, and pericardium

  • Heart murmur due to valve disease

  • Symptoms of heart failure in severe disease

Joint Manifestations

  • Migratory polyarthritis (pain and swelling that moves from one large joint to another)

Neurologic Manifestations

  • Sydenham chorea (involuntary, irregular movements)

Skin Manifestations

  • Subcutaneous nodules

  • Erythema marginatum (nonpruritic rash with central clearing)

Valve Involvement

  • Mitral valve (most common)

    • Early: Mitral regurgitation

    • Late: Mitral stenosis

  • Aortic valve

    • Aortic regurgitation

    • Late aortic stenosis

High-Yield Mnemonic: JONES

    • J – Joints (migratory polyarthritis)

    • O –  (Carditis/Pancarditis)

    • N – Nodules (subcutaneous)

    • E – Erythema marginatum

    • S – Sydenham chorea

Rheumatic Heart Disease Overview
Rheumatic Heart Disease Overview

History Taking

  • “Have you had a sore throat or strep throat in the past few weeks?”
  • “Was it treated with antibiotics?”
  • “Have you had a fever recently?”
  • “Do you have pain or swelling that moves from one joint to another?”
  • “Do you get short of breath during activity or when lying flat?”
  • “Do you have chest pain or palpitations?”
  • “Do you feel unusually tired or weak?”
  • “Have you noticed any skin rash or painless lumps under your skin?”
  • “Have you had any unusual jerky or involuntary movements?”
  • “Have you ever been told you have a heart murmur, rheumatic fever, or a heart valve problem?”
  • “Have you had rheumatic fever or repeated throat infections in the past?”

Physical Examination

General

  • Fever
  • Appears tired or unwell (malaise)

Cardiovascular

  • Heart murmur due to valvular disease
  • Signs of carditis (pancarditis)
  • Signs of heart failure in severe cases

Joints

  • Migratory arthritis involving the large joints (e.g., knees, ankles, elbows, wrists)
  • Swollen and tender joints

Skin

  • Erythema marginatum (nonpruritic rash with central clearing)
  • Subcutaneous nodules over extensor surfaces

Neurological

  • Sydenham chorea (involuntary, irregular movements)

Cardiac Examination Findings

  • Murmur of mitral regurgitation (acute rheumatic valvulitis)
  • Murmur of aortic regurgitation
  • In chronic RHD, findings of mitral stenosis may be present

Investigations

  • Laboratory Tests

    • Complete blood count (CBC) – may show leukocytosis or anemia
    • ESR and CRP – elevated inflammatory markers
    • Antistreptolysin O (ASO) titer – elevated
    • Anti-DNase B titer – elevated
    • Throat swab/culture or rapid streptococcal antigen test to identify recent GAS infection

    Cardiac Investigations

    • Echocardiography (Gold standard) – detects valvular damage (e.g., mitral regurgitation, aortic regurgitation, or chronic mitral stenosis)
    • ECG – commonly shows prolonged PR interval (first-degree AV block); may also show other conduction abnormalities
    • Chest X-ray – may show enlarged left atrium, enlarged left ventricle, or pulmonary edema

Diagnosis

Acute Rheumatic Fever · Jones Criteria (2015 AHA Revision)

ARF Diagnosis requires evidence of preceding Group A Streptococcal (GAS) infection PLUS: 2 Major criteria — OR — 1 Major + 2 Minor criteria. Criteria thresholds differ by population risk.
Diagnostic formula
Evidence of GAS infection
+
2 Major
 OR 
1 Major + 2 Minor
=
ARF Diagnosis
Major Criteria 5 features
1. Carditis
Clinical Pancarditis: endocarditis (new valvular regurgitation — mitral > aortic), myocarditis, pericarditis
Subclinical Echocardiographic mitral or aortic regurgitation meeting Doppler criteria without auscultatory findings — counts as major in high-risk populations
2. Polyarthritis
Low-risk Migratory polyarthritis (≥2 large joints)
High-risk Monoarthritis or polyarthralgia may count as major (see population note)
Joints: knees, ankles, wrists, elbows — migratory, swollen, hot, painful. Responds dramatically to aspirin.
3. Chorea (Sydenham's)
Involuntary, purposeless, rapid movements; emotional lability; muscular weakness. May appear weeks–months after GAS infection. Sufficient alone for ARF diagnosis (monosymptomatic chorea).
4. Erythema Marginatum
Evanescent, non-pruritic, pink/red rash with central clearing and serpiginous margins. Trunk and proximal limbs; spares face. Rare (<5% of cases).
5. Subcutaneous Nodules
Firm, painless nodules over bony prominences and tendons (elbows, wrists, knees, spinous processes, occiput). 0.5–2 cm. Associated with severe carditis. Very rare.
Minor Criteria 5 features
1. Fever
Low-risk ≥38.5°C
High-risk ≥38.0°C
Documented at time of presentation
2. Elevated ESR / CRP
Low-risk ESR ≥60 mm/h and/or CRP ≥3.0 mg/dL
High-risk ESR ≥30 mm/h and/or CRP ≥3.0 mg/dL
3. Prolonged PR interval
Age-adjusted first-degree AV block on ECG. Cannot be used as minor criterion if carditis is already counted as a major criterion.
4. Monoarthralgia
High-risk populations only Joint pain without objective signs of inflammation — only qualifies as minor in high-risk populations after excluding other causes.
5. Monoarthritis
High-risk populations only Inflammation in a single joint — may be downgraded from major to minor in low-risk settings, or used as minor in high-risk populations if monoarthralgia is already counted.
Mandatory: Evidence of preceding GAS infection
Serological
Elevated or rising ASO titre
Elevated or rising anti-DNase B titre
Other streptococcal antibodies (anti-streptokinase, anti-hyaluronidase)
Microbiological
Positive throat culture for GAS
Positive rapid antigen test for GAS
Note: positive swab may reflect chronic carriage; serology preferred
Special exceptions
Chorea alone: GAS evidence not always required (long latency)
Insidious-onset carditis alone: GAS evidence may be absent
Recurrent ARF: requires only 1 major OR 2 minor + GAS evidence
Low-risk populations
ARF incidence <2 per 100,000 school-age children per year, OR RHD prevalence <1 per 1,000 per year

  • Stricter thresholds apply (higher fever cutoff, higher ESR)
  • Monoarthritis/monoarthralgia do not count as major criteria
  • Subclinical carditis on echo counts as major only in high-risk
High-risk populations
ARF incidence ≥2 per 100,000 school-age children, OR RHD prevalence ≥1 per 1,000 per year

  • Lower fever and ESR thresholds apply
  • Monoarthritis counts as major; monoarthralgia counts as minor
  • Subclinical carditis on echo counts as major criterion
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Related Topics

Management

1. Eradicate Streptococcal Infection

  • Treat the group A streptococcal (GAS) infection with antibiotics.

2. Long-Term Antibiotic Prophylaxis

  • Essential for all patients with ARF or RHD to prevent recurrence.

  • First-line: Intramuscular benzathine penicillin G every 4 weeks.

  • Alternatives:

    • Oral penicillin V

    • Sulfadiazine or a macrolide for patients with a confirmed penicillin allergy

3. Manage Cardiac Complications

  • Refer patients with carditis or RHD to cardiology.

  • Treat heart failure according to guideline recommendations.

  • Anticoagulation if atrial fibrillation develops.

  • Consider endocarditis prophylaxis in high-risk patients and treat endocarditis promptly if it occurs.

4. Valve Disease Management

  • Valvuloplasty or heart valve replacement may be required for severe valvular disease.

5. Follow-Up

  • Regular echocardiography for long-term monitoring.

  • Educate patients about:

    • Adhering to antibiotic prophylaxis

    • Seeking prompt treatment for sore throats

    • Maintaining good dental hygiene

Complications

    • Permanent valvular heart disease (especially mitral valve, followed by the aortic valve)
    • Heart failure
    • Atrial fibrillation
    • Infective endocarditis
    • Progressive worsening of valve disease, which may require valvuloplasty or heart valve replacement
    • Recurrent acute rheumatic fever, leading to further valve damage if long-term antibiotic prophylaxis is not maintained

Prognosis

  • Cardiac involvement is the most important prognostic factor.
  • Patients with carditis are at high risk of developing permanent valvular heart disease (rheumatic heart disease), especially with recurrent episodes of acute rheumatic fever.
  • Early death from acute rheumatic fever is usually due to myocarditis, rather than valvular disease.
  • Long-term antibiotic prophylaxis helps prevent recurrent acute rheumatic fever and slows progression of rheumatic heart disease.

Key Points / Clinical Pearls

  • RHD is a chronic complication of acute rheumatic fever.
  • Caused by untreated group A streptococcal (strep throat) infection.
  • Mitral valve is most commonly affected; aortic valve is second.
  • Jones criteria are used to diagnose acute rheumatic fever.
  • Gold standard for assessing valve damage: Echocardiography.
  • Main treatment: Eradicate streptococcal infection, long-term penicillin prophylaxis, manage complications, and perform valve repair/replacement if severe.
  • Preventing recurrent acute rheumatic fever is essential to reduce progression of rheumatic heart disease.
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