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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- Mitral anterior leaflet
- Mitral posterior leaflet
- Chordae tendineae
- Left atrium
- Aortic right coronary cusp
- Aortic left coronary cusp
- Aortic non-coronary cusp
- Tricuspid anterior leaflet
- Left ventricle papillary muscles
- Pulmonary veins
Microorganisms that cause it
In immunology
In histology
In embryology
In the clinical exam
- Murmurs: timing, grade, location and radiation
- Valve murmurs: the table to know
- Heart failure syndrome: left- and right-sided signs
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
- Benzathine benzylpenicillin
- Phenoxymethylpenicillin (penicillin V)
- Sulfadiazine
- Benzylpenicillin (penicillin G)
- Warfarin
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.
Related conditions
- Atrial fibrillation
- Heart failure
- Aortic Valve Stenosis
- Infective endocarditis
- Ischaemic stroke
- Streptococcal pharyngitis
- Mitral regurgitation
- Aortic regurgitation
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.