Rheumatic fever and rheumatic heart disease

01OverviewDefinition, clinical context and the essential points that orientate the chapter.

ARF is rare in the UK, so diagnostic delay is common, particularly in migrants or people with prior disease. Diagnosis is clinical and syndromic: no single ASO titre, ECG change or murmur is definitive.

The major preventable harm is recurrence, because repeated inflammatory episodes compound valve injury. Chronic disease then follows standard lesion-specific valve and anticoagulation pathways, with additional attention to prophylaxis adherence and family/social barriers.

Key points

Acute rheumatic fever (ARF) is an immune-mediated complication usually appearing 1-5 weeks after group A streptococcal throat or skin infection.
Use revised Jones criteria plus evidence of preceding streptococcal infection; polyarthritis, carditis, chorea, erythema marginatum and subcutaneous nodules are the classic major manifestations.
Echocardiography with Doppler is required in suspected or confirmed ARF even when auscultation is normal because carditis may be subclinical.
Sydenham chorea and indolent carditis can present late, when streptococcal tests have normalised; do not reject the diagnosis solely for a negative ASO titre.
Eradicate residual streptococcus and start secondary prophylaxis after ARF; benzathine benzylpenicillin 1.2 million units IM every 3-4 weeks is the licensed adult regimen.
Prophylaxis duration is longer with carditis: at least 5 years/up to age 21 without cardiac involvement, at least 10 years/up to age 21 after transient carditis, and at least 10 years/up to age 40 with persistent involvement; some need lifelong prophylaxis.
Chronic rheumatic heart disease most often produces mitral stenosis/regurgitation, frequently with multivalve disease; assess AF, pulmonary pressure and pregnancy risk.
Antibiotic prophylaxis against infective endocarditis is not routinely offered for dental procedures solely because of rheumatic valve disease under NICE CG64.

02AetiologyUnderlying causes, associations and risk factors, with why each one matters.

Preceding group A streptococcal infection

Acute rheumatic fever follows an immune response to group A streptococcal throat or skin infection after a latent interval, rather than direct bacterial invasion of the heart.

Susceptible and repeatedly exposed groups

Host susceptibility, crowding, repeated streptococcal exposure and barriers to prompt diagnosis or treatment increase risk, including for people arriving from higher-prevalence regions.

Recurrent rheumatic fever

A previous episode creates vulnerability to further immune attacks. Missed secondary prophylaxis permits reinfection, and each recurrence can add cumulative valve inflammation and scarring.

03PathophysiologyThe causal sequence from the underlying abnormality to symptoms and harm.

1
Streptococcal immune priming
The immune system generates antibodies and T-cell responses against streptococcal antigens during the antecedent infection, usually after pharyngeal or cutaneous exposure.
2
Molecular mimicry
Cross-reactive immune responses recognise structurally similar host tissues, producing sterile inflammation in joints, heart, skin and the nervous system after infection has subsided.
3
Acute carditis and valvulitis
Inflammation may involve endocardium, myocardium and pericardium. Mitral or aortic valvulitis causes regurgitation, and severe acute valve dysfunction can precipitate heart failure.
4
Healing with valve scarring
Acute and especially recurrent valvulitis can heal through fibrosis, causing leaflet thickening, commissural fusion and chordal shortening. These changes may produce stenosis, regurgitation or mixed multivalve disease.
5
Chronic haemodynamic burden
Rheumatic valve obstruction or leakage can enlarge affected atria and ventricles, raise pulmonary pressure and promote atrial fibrillation, congestion and heart failure.

04Clinical features and red flagsSymptoms, examination findings, patterns of presentation and time-critical warnings.

Migratory large-joint arthritis

Painful swelling moving between knees, ankles, elbows or wrists after recent streptococcal infection is a typical major manifestation.

CarditisRed flag

Tachycardia, new MR/AR murmur, cardiomegaly, pericarditic features or heart failure indicates pancardial involvement; Doppler-only valvulitis also counts.

Sydenham chorea

Delayed, purposeless movements, emotional lability, hypotonia and deteriorating handwriting may be the sole presentation.

Skin signs

Erythema marginatum is an evanescent non-pruritic serpiginous rash; firm painless subcutaneous nodules are uncommon but specific clues.

Chronic valve disease

Exertional dyspnoea, haemoptysis, AF, opening snap/mid-diastolic murmur or multivalve murmurs years later suggest rheumatic heart disease.

High-risk decompensationRed flag

Pulmonary oedema, embolic neurology, pregnancy-related deterioration or fast AF with mitral stenosis needs urgent admission.

05InvestigationsWhat to request, why it matters and how to interpret it.

Investigation order

Read from the initial assessment onwards. Tests may run in parallel in urgent care; first-line, preferred, confirmatory, definitive and gold-standard labels appear only when the chapter explicitly states them.

01
Throat culture/rapid GAS test and streptococcal serologyFirst step
Why
Demonstrate preceding group A streptococcal infection.
Interpretation and limitations
A rising or elevated ASO/anti-DNase B supports prior infection; a single normal result does not exclude late chorea or indolent carditis.
02
FBC, CRP and ESR
Why
Support inflammation and provide Jones minor criteria.
Interpretation and limitations
Non-specific; normal markers make active systemic inflammation less likely but can occur in delayed isolated chorea.
03
12-lead ECG
Why
Look for PR prolongation, rhythm and chamber strain.
Interpretation and limitations
PR prolongation is a minor criterion only when carditis is not already counted as a major criterion; detect AF in chronic mitral disease.
04
TTE with Doppler
Why
Identify clinical/subclinical carditis and quantify chronic valve lesions, chambers and pulmonary pressure.
Interpretation and limitations
Pathological MR/AR can establish subclinical carditis; chronic commissural fusion and multivalve involvement support rheumatic morphology.
05
Blood cultures before antibiotics if febrile with a murmur
Why
Exclude infective endocarditis, an important alternative.
Interpretation and limitations
ARF is sterile; positive cultures or destructive valve/perivalvular findings redirect management.
06
CXR and selected CMR/TOE
Why
Assess heart failure, pericardial involvement or complex valve anatomy.
Interpretation and limitations
Use advanced imaging to answer a defined uncertainty, not to replace Jones clinical assessment.

06Differential diagnosisRealistic alternatives and the features that help distinguish them.

Infective endocarditis

Persistent fever, positive blood cultures, destructive valve lesions or embolic phenomena favour active infection; acute rheumatic carditis is sterile and follows a compatible post-streptococcal syndrome.

Septic arthritis

A single intensely inflamed joint, systemic toxicity and positive synovial culture favour infection; rheumatic arthritis more often migrates between large joints.

Viral myocarditis

Prominent ventricular dysfunction, arrhythmia and myocardial injury without Jones-criteria manifestations or preceding streptococcal evidence support myocarditis rather than rheumatic fever.

Post-streptococcal reactive arthritis

Persistent, additive joint inflammation, often with a less marked response to anti-inflammatory treatment and without other rheumatic-fever features, suggests post-streptococcal reactive arthritis.

Alternative causes of chorea

Medication exposure, autoimmune disease and inherited or metabolic disorders can cause involuntary movements; Sydenham chorea has a compatible delayed post-streptococcal phenotype and neurological examination.

07ManagementImmediate care, first-line treatment, alternatives and escalation.

01AcuteSuspected acute rheumatic feverFirst stepCompatible major/minor manifestations after possible GAS infection.

1Admit if carditis, heart failure, important arrhythmia, neurological risk or diagnostic instability; obtain ECG, inflammatory markers, GAS evidence and TTE.
2Apply revised Jones criteria and actively exclude septic arthritis, endocarditis, viral myocarditis, autoimmune disease and drug/toxin causes of chorea.
3Eradicate streptococcus even if the throat is now asymptomatic; treat arthritis/carditis and heart failure with paediatric/adult infection, cardiology and neurology input as appropriate.
4Before discharge, start secondary prophylaxis, document duration and create an echo/follow-up plan.

02PreventionSecondary prophylaxisConfirmed previous ARF or established rheumatic heart disease where prophylaxis is indicated.

1Use benzathine benzylpenicillin 1.2 million units deep IM every 3-4 weeks in adults unless contraindicated; use oral phenoxymethylpenicillin 250 mg twice daily when IM therapy is not feasible.
2Set duration by residual cardiac involvement and age, with the longer endpoint governing; persistent valve disease may justify prophylaxis to age 40 or lifelong.
3Track every dose and address missed appointments, injection pain, allergy history and access barriers rather than labelling non-adherence.
4If a new sore throat or possible recurrence occurs, assess promptly; prophylaxis reduces but does not eliminate risk.

03ChronicRheumatic heart diseaseEstablished valve damage, symptoms, AF, pregnancy planning or changing echo measurements.

1Quantify every valve lesion and ventricular/pulmonary consequences; multivalve disease requires integrated Heart Team planning.
2Treat congestion and rate/rhythm problems; use VKA anticoagulation for AF with moderate-to-severe rheumatic mitral stenosis rather than a DOAC.
3Refer severe symptomatic mitral stenosis for percutaneous commissurotomy when anatomy is suitable and there is no LA thrombus or important MR; otherwise assess surgery.
4Use pre-pregnancy valve and anticoagulation review because mitral stenosis and pulmonary hypertension can decompensate as plasma volume rises.

Key medicines and prescribing safety2 treatments · regimens, roles and cautions

Eradication/secondary prevention of recurrent rheumatic fever.

Benzathine benzylpenicillin

1.2 million units by deep IM injection every 3-4 weeks for secondary prophylaxis; the SmPC also licenses 1.2 million units IM as a single general streptococcal treatment dose.

Check immediate beta-lactam allergy; administer strictly IM with aspiration and emergency anaphylaxis capability. Long-term therapy needs periodic renal, hepatic and blood-count review.

Oral secondary-prophylaxis alternative when regular IM benzathine treatment is not feasible.

Phenoxymethylpenicillin

250 mg by mouth twice daily for prevention of recurrent rheumatic fever.

Adherence is less reliable than depot injection; check penicillin allergy and renal function, and do not use an abbreviated course for active GAS eradication.

08ComplicationsImportant consequences, why they occur and why they matter clinically.

Chronic rheumatic valve disease

Cumulative valvulitis produces mitral stenosis or regurgitation and often multivalve disease, creating a lifelong need for surveillance and sometimes catheter or surgical intervention.

Pulmonary hypertension and heart failure

Elevated left-atrial pressure from mitral disease is transmitted to the pulmonary circulation, while valve overload eventually causes ventricular dysfunction and systemic congestion.

Atrial fibrillation

Chronic pressure and volume loading enlarge the left atrium, creating an arrhythmic substrate. Rapid ventricular response may abruptly worsen symptoms or precipitate pulmonary oedema.

Systemic embolism and stroke

Atrial fibrillation and blood stasis within an enlarged left atrium promote thrombus formation, particularly with moderate-to-severe mitral stenosis, allowing embolisation to the brain or other organs.

Pregnancy-related decompensation

Increased circulating volume and cardiac output during pregnancy can expose limited reserve in mitral stenosis or pulmonary hypertension, causing congestion and haemodynamic deterioration.

09Monitoring and follow-upTreatment response, safety checks and longer-term review.

Repeat TTE after acute carditis and then according to residual lesion severity, symptoms and ventricular/pulmonary response.
Maintain a prophylaxis register with due dates, administered doses, adverse reactions and planned stop/review date.
Ask at every review about dyspnoea, orthopnoea, palpitations, syncope, haemoptysis, embolic symptoms and new sore throat.
In chronic mitral disease, monitor rhythm, anticoagulation, pulmonary pressure, LA/LV size and exercise tolerance.
Review pregnancy intentions early and refer significant disease to a Pregnancy Heart Team before conception.
Reassess social and travel barriers to injections; recurrence prevention fails if delivery is not practical.

10Special situationsVariants, exceptions and circumstances that change the usual approach.

Chorea may stand alone

Because it occurs months after infection, inflammatory markers and streptococcal serology can be normal; the neurological phenotype and exclusion of alternatives matter.

Do not double-count

PR prolongation is not added as a minor Jones criterion when carditis is already used as a major criterion.

Echo can reveal silent carditis

Pathological Doppler regurgitation can establish subclinical carditis even without an audible murmur.

Recurrence accumulates damage

Secondary prophylaxis is disease-modifying public-health treatment, not an optional add-on after joint symptoms resolve.

Mitral stenosis changes anticoagulant choice

AF with moderate-to-severe rheumatic mitral stenosis remains a VKA indication; it is not the usual non-valvular AF DOAC pathway.

11Common pitfallsFrequent interpretation and management errors.

01
Diagnosing ARF from an ASO titre alone without a compatible syndrome.
02
Excluding carditis because there is no murmur or excluding chorea because serology is negative.
03
Stopping secondary prophylaxis when arthritis resolves without considering residual cardiac disease and age.
04
Using a DOAC for AF with moderate-to-severe rheumatic mitral stenosis.
05
Offering routine dental antibiotic prophylaxis solely for rheumatic valve disease despite NICE CG64.