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Rheumatic heart disease !

Rheumatic heart disease is lasting damage to the heart valves, mainly the mitral, after rheumatic fever that follows a streptococcal sore throat.

Updated 2026-10-30

Structures involved

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Microorganisms that cause it

In immunology

In histology

In embryology

In the clinical exam

In physiology

Why it happens

What it is

Permanent damage to the heart valves, mainly the mitral and aortic, after one or more episodes of acute rheumatic fever triggered by group A streptococcal pharyngitis.

Who gets it

Rare in high-income countries but still present in Latin America and other low- and middle-income settings, where it causes valve disease in children and young adults, especially women. A 2022 systematic review found prevalence in schoolchildren ranging from 0.24 to 48 per 1,000 across studies, with few recent data outside Brazil.

Causes

  • Autoimmune reaction after untreated or poorly treated group A streptococcal pharyngitis
  • Repeated attacks of rheumatic fever worsen valve damage

Risk factors

  • Children and adolescents (about 5–15 years) for acute rheumatic fever
  • Poverty and household crowding
  • Limited access to treatment of sore throat
  • Previous rheumatic fever

Pathophysiology

Antibodies and T cells against streptococcal antigens cross-react with cardiac proteins (molecular mimicry), causing pancarditis. Repeated inflammation leads to fibrosis: leaflets thicken and fuse at the commissures, chordae shorten and fuse. The result is regurgitation early and stenosis, typically mitral, years later.

Classification

  • Acute rheumatic fever with carditis
  • Chronic rheumatic heart disease: mitral regurgitation, mitral stenosis, aortic disease or mixed multivalve disease
  • Subclinical (echocardiographic only) vs clinical disease

Symptoms and signs

  • Breathlessness on exertion, orthopnoea
  • Palpitations (atrial fibrillation)
  • Fatigue
  • Haemoptysis in severe mitral stenosis
  • Murmurs: mid-diastolic rumble with opening snap (mitral stenosis), pansystolic murmur (mitral regurgitation)
  • Symptoms often worsen in pregnancy

Diagnosis

Clinical
Acute rheumatic fever is diagnosed with the revised Jones criteria (major: carditis, arthritis, chorea, erythema marginatum, subcutaneous nodules) plus evidence of recent streptococcal infection. Chronic disease is suspected from murmurs and history.
Tests
ASO or anti-DNase B titres and throat culture or rapid test for recent streptococcal infection; ESR and CRP in the acute phase. ECG: prolonged PR interval, left atrial enlargement or atrial fibrillation.
Imaging
Echocardiography is key: thickened leaflets, commissural fusion, restricted motion, valve area and pulmonary pressures; it also detects subclinical disease. Chest X-ray may show left atrial enlargement and congestion.

Differential diagnosis

  • Degenerative or congenital valve disease
  • Infective endocarditis
  • Mitral valve prolapse
  • Other causes of arthritis with fever (septic arthritis, reactive arthritis, lupus)

Goal of treatment

Prevent new attacks of rheumatic fever, treat heart failure and atrial fibrillation, and repair or replace damaged valves at the right time.

Treatment

Medical
Secondary prophylaxis: long-term antibiotics (usually intramuscular benzathine penicillin) to prevent recurrences; duration depends on age and severity, and criteria vary.
Medical
Heart failure and atrial fibrillation treatment: diuretics, rate control and anticoagulation (vitamin K antagonists with mitral stenosis).
Procedure
Percutaneous balloon mitral commissurotomy for suitable mitral stenosis.
Surgical
Valve repair or replacement for severe disease that is not suitable for balloon treatment.
Follow-up
Regular echo follow-up, dental care and pre-pregnancy assessment.

Drugs for this condition

Complications

  • Heart failure
  • Atrial fibrillation and stroke
  • Pulmonary hypertension
  • Infective endocarditis
  • Complications in pregnancy
  • Complications of prosthetic valves and anticoagulation

Prognosis

Depends on the severity of valve damage and on adherence to secondary prophylaxis, which can halt progression and even allow mild disease to regress. Untreated severe disease shortens life in young adults.

Prevention

  • Prompt antibiotic treatment of streptococcal pharyngitis (primary prevention)
  • Secondary prophylaxis after rheumatic fever
  • Better housing and access to health care
  • Echocardiographic screening in high-risk groups

Test yourself

Think of the answer, then tap the question to see it.

Which infection triggers acute rheumatic fever?

Pharyngitis caused by group A beta-haemolytic Streptococcus (Streptococcus pyogenes).

What are the five major Jones criteria?

Carditis, arthritis, Sydenham chorea, erythema marginatum and subcutaneous nodules.

Which valve is most often affected in chronic rheumatic heart disease, and how?

The mitral valve; over the years it typically becomes stenotic through commissural fusion and leaflet thickening.

What is the mechanism of rheumatic fever?

Molecular mimicry: antibodies and T cells against streptococcal antigens cross-react with heart tissue.

What is secondary prophylaxis in rheumatic fever?

Regular long-term antibiotics after a first attack to prevent new streptococcal infections and further valve damage.

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Educational information, not medical advice. For any symptom, see a health professional.

Sources: Rheumatic Heart Disease (StatPearls (NCBI Bookshelf), 2023); Current Situation of Acute Rheumatic Fever and Rheumatic Heart Disease in Latin America and the Caribbean: A Systematic Review (Global heart, 2022) · adapted, CC BY 4.0.

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Representative model, for educational purposes only.

Mitral anterior leaflet highlighted on the Durgical 3D model