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Rheumatic Fever & Rheumatic Heart Disease

๐Ÿ“‹ Key Information Summary

๐Ÿ“‹
  • Acute rheumatic fever (ARF) is an autoimmune inflammatory condition triggered by Group A Streptococcus (GAS) pharyngitis, primarily affecting children aged 5โ€“14 years.
  • Recurrent episodes of ARF cause cumulative cardiac valve damage, leading to rheumatic heart disease (RHD) โ€” a chronic and often disabling condition.
  • Diagnosis is clinical, based on the revised Jones Criteria (2015): two major manifestations OR one major + two minor, plus evidence of preceding GAS infection.
  • Major Jones manifestations: carditis (including valvulitis), polyarthritis, Sydenham chorea, erythema marginatum, subcutaneous nodules.
  • Minor Jones manifestations: fever, elevated acute-phase reactants (ESR/CRP), prolonged PR interval on ECG, arthralgia (only if arthritis is not a major manifestation).
  • Evidence of preceding GAS infection: positive throat culture, elevated/strengthening ASO titre or anti-DNase B, or positive rapid antigen detection test.
  • Acute treatment: benzathine penicillin G 1.2 MU IM (single dose) to eradicate GAS, plus anti-inflammatory therapy (aspirin for arthritis, corticosteroids for severe carditis).
  • Secondary prophylaxis: benzathine penicillin G 1.2 MU IM every 21 days (every 14 days in high-risk patients) โ€” duration depends on age and presence of RHD.
  • RHD management requires multidisciplinary care, echocardiographic surveillance, anticoagulation for atrial fibrillation or prosthetic valves, and timely surgical valve repair or replacement.
  • Aboriginal and Torres Strait Islander peoples bear the greatest burden of ARF and RHD in Australia, with incidence rates among the highest globally โ€” RHDAustralia clinical guidelines (2020) are the standard of care.
  • The Australian RHD Register and Control Programme support notification, recall, and secondary prophylaxis adherence in endemic regions (NT, QLD, WA).
  • Penicillin allergy: use azithromycin 500 mg PO day 1 then 250 mg daily for 4 days (secondary prophylaxis: erythromycin 250 mg BD or azithromycin if tolerated).
  • ARF is a notifiable disease in the NT, QLD, SA, and WA.

Introduction & Australian Epidemiology

Acute rheumatic fever (ARF) is a systemic autoimmune condition triggered by pharyngeal infection with Group A Streptococcus (GAS; Streptococcus pyogenes). An abnormal immune response leads to inflammation of the heart, joints, brain, and skin. Recurrent episodes cause cumulative valvular damage, culminating in rheumatic heart disease (RHD) โ€” a chronic, progressive condition responsible for significant morbidity and premature mortality.

In Australia, ARF and RHD are diseases of inequity. The burden falls overwhelmingly on Aboriginal and Torres Strait Islander peoples, particularly children and young adults living in remote and very remote communities across the Northern Territory, Queensland, and Western Australia. Incidence rates of ARF among Indigenous Australians in high-risk areas can exceed 150โ€“200 per 100,000 children aged 5โ€“14 years, comparable to the highest rates globally. In contrast, ARF in non-Indigenous Australians in metropolitan areas is exceedingly rare, though clusters of ARF have been reported in non-Indigenous children in New Zealand and parts of the United States.

โš ๏ธ
Key equity issue: ARF has been virtually eliminated from affluent populations through improved housing, reduced overcrowding, and access to timely antibiotics. Its persistence in Australia reflects ongoing social determinants of health disadvantage.

ARF is a notifiable disease in the Northern Territory, Queensland, South Australia, and Western Australia. National notification data consistently show that over 95% of ARF cases occur in Aboriginal and Torres Strait Islander peoples. The National Aboriginal Community Controlled Health Organisation (NACCHO) and RHDAustralia coordinate registries, clinical guidelines, and control programmes across endemic jurisdictions.

The revised Australian guidelines for the management of ARF and RHD were published by RHDAustralia (formerly the RHD Control Programme) in 2020, aligned with the New Zealand guidelines and the 2015 AHA revised Jones Criteria. These guidelines form the basis of this article's recommendations.

Rheumatic Fever & Rheumatic Heart Disease clinical infographic โ€” pathophysiology, clinical clues, diagnosis, imaging, and management
Tap or click image to enlarge โ€” Rheumatic Fever & Rheumatic Heart Disease: pathophysiology, clinical clues, diagnosis, imaging, and management.
Rheumatic Fever & Rheumatic Heart Disease infographic, full size

Pathophysiology & Group A Streptococcus

Molecular Mimicry

The pathogenesis of ARF is based on molecular mimicry between GAS antigens and host tissues. Antibodies produced against GAS M-protein and hyaluronate capsule cross-react with:

  • Cardiac myosin and valve glycoproteins โ€” leading to valvulitis and carditis.
  • Synovial joint antigens โ€” causing migratory polyarthritis.
  • Basal ganglia neurones โ€” producing Sydenham chorea.
  • Dermal collagen โ€” associated with subcutaneous nodules.

Susceptible Host

Not all individuals who contract GAS pharyngitis develop ARF. Host susceptibility is influenced by:

  • Genetic factors โ€” certain HLA class II alleles (DR7, DR5, DQ2) confer increased risk.
  • Streptococcal strain โ€” rheumatogenic strains (M-types 1, 3, 5, 6, 14, 18, 19, 24, 27, 29) are associated with pharyngitis and ARF; pyoderma strains (49, 53, 55, 56, 57, 59) are associated with impetigo and, in some populations, post-cutaneous ARF.
  • Repeated exposure in crowded living conditions increases risk.
  • Age โ€” peak incidence in children 5โ€“14 years; rare before age 3 and after age 40.

The Latent Period

ARF typically manifests 2โ€“4 weeks after GAS pharyngitis. The onset may be insidious. Many patients do not recall a preceding sore throat, especially in populations where subclinical GAS infection is common.

Cardiac Pathology in RHD

Repeated episodes of ARF cause progressive valvular inflammation and scarring. The mitral valve is affected in ~90% of cases, followed by the aortic valve. Pathological features include:

  • Acute: fibrinoid necrosis of valve leaflets, Aschoff bodies (granulomas) in myocardium.
  • Chronic: commissural fusion, chordal shortening and thickening, calcification โ€” leading to mitral stenosis, regurgitation, or mixed disease.
  • Aortic regurgitation occurs in ~30% of RHD cases, often combined with mitral disease.
๐Ÿ’ก
Post-cutaneous ARF: In some tropical populations (including parts of northern Australia), skin infections with GAS (impetigo) rather than pharyngitis may trigger ARF. Throat cultures may be negative, but serological markers (anti-DNase B) are often elevated.

Clinical Presentation & Jones Criteria (Major & Minor)

The diagnosis of ARF is clinical. The 2015 revised Jones Criteria, endorsed by the American Heart Association (AHA) and adopted by RHDAustralia, require evidence of preceding GAS infection plus:

  • Two major manifestations, OR
  • One major + two minor manifestations.

Exceptions: Three categories of clinical presentation are considered diagnostic of ARF even without strict adherence to the above combination:

  • Sydenham chorea (may be the sole manifestation).
  • Insidious-onset carditis (may present as heart failure without other criteria).
  • Recurrent ARF โ€” a known history of RHD or prior ARF, with one major or several minor manifestations plus evidence of recent GAS infection.

Major Manifestations

class="guideline-td">5โ€“10%
Manifestation Features Frequency
Carditis (including valvulitis) New murmur of mitral regurgitis (apical pansystolic) or aortic regurgitation (early diastolic). Pericarditis, cardiomegaly, heart failure. Echocardiographic evidence of subclinical valvulitis is now accepted as a major manifestation. 50โ€“70%
Polyarthritis Migratory, affecting large joints (knees, ankles, elbows, wrists). Flitting โ€” one joint improves as another becomes involved. Extremely painful and responsive to salicylates. 60โ€“75%
Sydenham chorea Involuntary, purposeless movements; emotional lability; hypotonia; "milkmaid grip"; "darting tongue". May occur in isolation and late (weeks to months after GAS infection). Also called St Vitus dance. 10โ€“30%
Erythema marginatum Evanescent, non-pruritic, erythematous rash with serpiginous margins and central clearing. Trunk and proximal limbs. Not scaly. Worsened by warmth.
Subcutaneous nodules Small (0.5โ€“2 cm), firm, painless, mobile nodules over extensor surfaces of joints (elbows, knees, wrists, occiput, spine). Usually associated with severe carditis. 2โ€“10%

Minor Manifestations

Manifestation Notes
Fever โ‰ฅ38ยฐC. Non-specific. Common at presentation.
Polyarthralgia Can only be counted as minor if arthritis is NOT a major manifestation. Otherwise excluded to avoid "double counting."
Elevated ESR โ‰ฅ60 mm/hr or CRP โ‰ฅ30 mg/L Acute-phase reactants. ESR may be unreliable if there is concurrent heart failure (hepatic congestion lowers ESR).
Prolonged PR interval on ECG Age-adjusted. First-degree AV block. Does NOT imply progression to complete heart block.
๐Ÿšจ
Lower threshold in high-risk populations: The 2015 Jones Criteria introduced population-specific thresholds. For patients from high-risk populations (including Aboriginal and Torres Strait Islander peoples in endemic areas), a lower threshold applies: one major + one minor is acceptable when supported by evidence of preceding GAS infection and clinical suspicion is high.

Evidence of Preceding GAS Infection

At least one of the following must be present (except in Sydenham chorea and insidious-onset carditis):

  • Positive throat swab culture for GAS.
  • Positive rapid antigen detection test (RADT) for GAS.
  • Elevated or rising anti-streptolysin O (ASO) titre.
  • Elevated anti-DNase B titre (particularly useful when pharyngeal infection is absent and post-cutaneous GAS is suspected).
  • Streptozyme test (where available).

Investigations

Laboratory Investigations

Essential
Anti-streptolysin O (ASO) titre
Elevated in ~80% of ARF cases. Paired sera (2โ€“4 weeks apart) showing a โ‰ฅ2-fold rise is more diagnostic. Peak at 3โ€“6 weeks post-infection. Available in all Australian pathology laboratories. MBS item 66844.
Essential
Anti-DNase B titre
Useful in combination with ASO. Rises later and persists longer. Preferred when post-cutaneous GAS is suspected. MBS item 66844.
Available
Throat swab (culture and/or RADT)
Positive in only ~15โ€“20% at ARF presentation due to the 2โ€“4 week latent period. Still recommended. Rapid antigen tests available at point of care in many rural and remote clinics.
Essential
ESR and CRP
Acute-phase reactants. ESR โ‰ฅ60 mm/hr and/or CRP โ‰ฅ30 mg/L are minor Jones criteria. Useful for monitoring response to treatment. CRP normalises faster than ESR.
Essential
Full blood count
Normochromic normocytic anaemia common. Neutrophil leucocytosis may be present.
Available
ECG
Prolonged PR interval (minor criterion). May show ST-T wave changes. Useful baseline for monitoring. Available at all hospitals and many remote clinics.

Cardiac Imaging

Essential
Transthoracic echocardiography (TTE)
Critical for diagnosis of carditis and valvulitis. Echocardiographic evidence of subclinical valvulitis (isolated mitral or aortic regurgitation with morphological valve changes) is accepted as a major Jones criterion. Establishes baseline valve morphology for long-term RHD surveillance. Available at regional hospitals; portable devices increasingly used in remote communities.
Referral
Chest X-ray
May show cardiomegaly and pulmonary congestion in heart failure. Recommended when carditis is suspected.
๐Ÿ’ก
Echocardiographic screening: The RHDAustralia guidelines recommend screening echocardiography for all Aboriginal and Torres Strait Islander peoples in endemic areas at age 5โ€“12 years, and annually thereafter if at high risk. Portable echocardiography programmes in remote communities have detected significant subclinical RHD.

Treatment of Acute Rheumatic Fever

Step 1 โ€” Eradicate GAS

All patients with ARF โ€” regardless of throat swab result โ€” should receive antibiotic therapy to eradicate residual GAS from the pharynx.

๐Ÿ’Š
Benzathine penicillin G
Bicillin L-Aยฎ ยท Penicillin G ยท First-line
Adult dose 1.2 million units IM as a single dose
Paediatric dose (<30 kg) 600,000 units IM as a single dose
Route Deep intramuscular injection into the gluteal or deltoid muscle
Renal adjustment Not required
PBS status โœ” PBS General Benefit
๐Ÿ’Š
Phenoxymethylpenicillin (Penicillin V)
Penicillin VKยฎ ยท Oral alternative
Adult dose 500 mg PO BD for 10 days
Paediatric dose 250 mg PO BDโ€“TDS for 10 days (dose by weight)
PBS status โœ” PBS General Benefit
โš ๏ธ
Penicillin allergy: If allergic to penicillin (non-anaphylactic or anaphylactic), use azithromycin 500 mg PO on day 1, then 250 mg PO daily for 4 days. Erythromycin 500 mg PO BD for 10 days is an alternative. Do not use cephalosporins if there is a history of anaphylaxis to penicillin.

Step 2 โ€” Anti-inflammatory Therapy

Anti-inflammatory treatment reduces fever, pain, and joint inflammation, but does NOT alter the natural history of carditis.

๐Ÿ’Š
Aspirin (as the salicylate of choice)
Aspro Clearยฎ ยท Disprinยฎ ยท First-line for arthritis
Adult dose 80โ€“100 mg/kg/day PO in divided doses (max 6 g/day). Reduce to 60โ€“70 mg/kg/day once fever and joint inflammation settle. Target serum salicylate: 150โ€“250 mg/L.
Paediatric dose 80โ€“100 mg/kg/day PO in 4โ€“6 divided doses. Start high, taper after 2 weeks as inflammation resolves. Maintain for 4โ€“6 weeks total.
Duration 4โ€“6 weeks, guided by clinical and laboratory response (CRP normalisation)
Monitoring Serum salicylate levels. Watch for tinnitus, nausea, hepatotoxicity. Check LFTs weekly.
PBS status โœ” PBS General Benefit
๐Ÿ’Š
Prednisolone
Panafcorteloneยฎ ยท Soloneยฎ ยท For severe carditis
Adult dose 1โ€“2 mg/kg/day PO (max 60 mg/day). Taper over 2โ€“3 weeks after clinical improvement. Add aspirin during taper to prevent rebound.
Paediatric dose 1โ€“2 mg/kg/day PO. Same taper strategy.
Indication Moderate to severe carditis (heart failure, significant valvulitis, pericardial effusion)
PBS status โœ” PBS General Benefit
๐Ÿ’ก
Corticosteroid vs aspirin: There is no evidence that corticosteroids prevent long-term valvular damage. They are used for symptomatic relief of severe carditis. Aspirin remains the standard for arthritis and mild carditis. Some centres use corticosteroids for the first 2โ€“3 weeks then switch to aspirin for the remainder.

Step 3 โ€” Management of Heart Failure in Acute Carditis

If heart failure is present:

  • Diuretics (frusemide 0.5โ€“1 mg/kg IV/PO) for fluid overload.
  • ACE inhibitors (ramipril or enalapril) if MR is significant โ€” use cautiously, titrate slowly.
  • Bed rest and fluid restriction.
  • Avoid digoxin in acute carditis (higher risk of toxicity due to myocardial inflammation).
  • Urgent cardiology referral for inpatient management.

Step 4 โ€” Management of Sydenham Chorea

Sydenham chorea is usually self-limiting (resolving within 6 weeks to 6 months) but may persist. Management includes:

  • Reassurance and supportive care. Reduce environmental stimuli.
  • If functionally disabling: sodium valproate 15โ€“20 mg/kg/day PO (first-line) or carbamazepine.
  • Severe cases: short course of corticosteroids may be considered.
  • Psychological support โ€” anxiety and emotional lability are common.
  • Initiate secondary prophylaxis regardless of GAS serology (chorea may occur without evidence of preceding infection).

Monitoring During Acute Episode

Day 0โ€“3
Confirm diagnosis, administer benzathine penicillin G, commence anti-inflammatory therapy, echocardiography, ECG, baseline bloods (FBC, ESR, CRP, ASO, anti-DNase B, LFTs).
Week 1โ€“2
Monitor ESR/CRP trend, salicylate levels (if on aspirin), clinical assessment of arthritis, cardiac function. Check for signs of drug toxicity.
Week 4โ€“6
Repeat ESR/CRP โ€” should be normalising. Taper anti-inflammatory therapy. Repeat echocardiography. Commence secondary prophylaxis schedule.
Month 3
Follow-up echocardiography. Confirm secondary prophylaxis adherence. Repeat ASO/anti-DNase B to document decline.

Secondary Prophylaxis

Secondary prophylaxis with regular benzathine penicillin G (BPG) injections is the cornerstone of preventing recurrent ARF and progressive RHD. Adherence to prophylaxis is the single most important modifiable factor in RHD outcomes.

๐Ÿšจ
Lifelong prophylaxis may be required: Patients with established RHD (especially moderate or severe valvular disease) should receive secondary prophylaxis for life, or until age 35โ€“40 with no valve disease on echocardiography and at least 10 years since the last ARF episode. This decision must be made in consultation with a specialist.

Benzathine Penicillin G โ€” Secondary Prophylaxis

๐Ÿ’Š
Benzathine penicillin G
Bicillin L-Aยฎ ยท Standard regimen
Adult dose 1.2 million units IM every 21 days
Paediatric dose (<30 kg) 600,000 units IM every 21 days
High-risk interval Every 14 days โ€” for patients with severe RHD, recurrent ARF on 21-day dosing, or in high-incidence regions
Route Deep IM injection. Rotate injection sites. Use a 21-gauge needle (minimum). Consider lignocaine diluent to reduce pain.
PBS status โœ” PBS General Benefit

Penicillin Allergy โ€” Alternative Prophylaxis

๐Ÿ’Š
Erythromycin ethyl succinate
Erypedยฎ ยท EESยฎ
Adult dose 250 mg PO BD
Paediatric dose Dose by weight โ€” 10 mg/kg BD (max 250 mg BD)
Note Oral prophylaxis is less effective than BPG injections. Use only if BPG is truly contraindicated.
PBS status โœ” PBS General Benefit
๐Ÿ’Š
Azithromycin
Zithromaxยฎ
Dose 250 mg PO daily (adults and children >40 kg)
Note Used if erythromycin is not tolerated (GI side effects). Less evidence base for long-term prophylaxis.
PBS status โš  PBS Restricted Benefit

Duration of Secondary Prophylaxis

Clinical Scenario Recommended Duration
ARF without carditis (no valve disease on echo) Until age 21 years OR 5 years after last episode (whichever is longer)
ARF with mild carditis (no residual valve disease at follow-up echo) Until age 25โ€“30 years OR 10 years after last episode (whichever is longer)
ARF with persistent valve disease / established RHD Lifelong or until specialist review (age โ‰ฅ35โ€“40, no recurrence โ‰ฅ10 years, no valve disease on echo)
Recurrent ARF episodes Prolonged / lifelong prophylaxis regardless of valve status
โš ๏ธ
Adherence monitoring: BPG adherence should be documented. In Australia, the RHD Register tracks injection dates and sends recall notices. Community-controlled health services employ RHD coordinators and patient support officers to promote adherence. The RHDAustralia target is โ‰ฅ80% adherence (injections received on time within the prescribed interval).

Rheumatic Heart Disease (RHD) Management

RHD results from cumulative valve damage from one or more episodes of ARF. Management requires lifelong secondary prophylaxis plus medical and surgical management of valve disease.

Severity Classification (Echocardiographic)

Mild
Mild RHD
Mild mitral regurgitation (jet area <20% of LA) with normal valve morphology or minor morphological changes (e.g., thickening of anterior mitral leaflet โ‰ค5 mm).
Management: Secondary prophylaxis, echocardiography every 1โ€“2 years, GP follow-up
Moderate
Moderate RHD
Moderate MR or AR. Valve morphological changes. Mild mitral stenosis (MVA >1.5 cmยฒ). May be asymptomatic.
Management: Cardiology review every 6โ€“12 months. Echocardiography annually. Plan surgical review.
Severe
Severe RHD
Severe MR, AR, or mitral stenosis (MVA โ‰ค1.5 cmยฒ). Heart failure symptoms. Atrial fibrillation. Pulmonary hypertension. Candidate for surgery.
Management: Urgent cardiology and cardiothoracic surgery referral. Consider valve repair vs replacement.

Medical Management of RHD

  • Heart failure: Diuretics (frusemide, spironolactone), ACE inhibitors (ramipril, perindopril), beta-blockers (carvedilol). Avoid beta-blockers in severe symptomatic MS.
  • Atrial fibrillation: Anticoagulation with warfarin (target INR 2.0โ€“3.0). DOACs are generally NOT recommended for RHD-related AF with moderate-to-severe mitral stenosis. Rate control with beta-blockers or diltiazem. Consider rhythm control if symptomatic.
  • Anticoagulation for prosthetic valves: Warfarin is mandatory for mechanical prosthetic valves. Target INR varies by valve position and type (aortic: 2.0โ€“3.0; mitral: 2.5โ€“3.5). Low-dose aspirin (75โ€“100 mg) may be added.
  • Endocarditis prophylaxis: Prophylactic antibiotics are recommended for dental and high-risk procedures in patients with prosthetic valves or prior endocarditis, according to current Australian guidelines.
โš ๏ธ
DOACs in RHD: Direct oral anticoagulants (apixaban, rivarelbaan, edoxaban) are not recommended for AF in the setting of moderate-to-severe rheumatic mitral stenosis or mechanical prosthetic valves. The INVICTUS trial (2022) demonstrated inferior outcomes with rivarelbaan compared to warfarin in RHD-associated AF. Warfarin remains the standard of care.

Surgical and Interventional Management

Surgical referral should be considered when there is:

  • Symptomatic severe valve disease despite optimal medical therapy.
  • Asymptomatic severe valve disease with evidence of LV dysfunction, pulmonary hypertension, or progressive chamber dilatation.
  • Severe mitral stenosis (MVA โ‰ค1.0 cmยฒ) โ€” consider percutaneous balloon mitral valvuloplasty (PBMV) if valve morphology is favourable.
Intervention Indication Considerations
Mitral valve repair Preferred for severe MR with suitable anatomy Avoids lifelong anticoagulation. Better outcomes than replacement. Requires surgical expertise.
Mitral valve replacement When repair is not feasible (heavily calcified, fused leaflets) Mechanical valve: lifelong warfarin. Bioprosthetic: limited durability in young patients.
PBMV (balloon valvuloplasty) Severe MS (MVA โ‰ค1.5 cmยฒ) with favourable morphology Avoid if LA thrombus, significant MR, or heavily calcified/subvalvular disease. Available at major cardiac centres.
Aortic valve replacement Severe AR or AS TAVR is generally not used for rheumatic aortic disease. SAVR remains standard.

Contraception and Pregnancy Planning

Women of childbearing age with RHD require:

  • Contraception counselling โ€” warfarin is teratogenic (first trimester exposure causes warfarin embryopathy).
  • Pre-pregnancy planning with cardiology and obstetric teams at a tertiary centre.
  • Warfarin should be switched to heparin (LMWH or UFH) before conception or by 6 weeks' gestation.
  • Severe RHD may need surgical correction before pregnancy.

Special Populations

๐Ÿ‘ถ Paediatric
Peak incidence 5โ€“14 years. Polyarthritis and Sydenham chorea are more common in children; carditis is more likely to cause permanent valve damage in younger children.
BPG dose: <30 kg โ†’ 600,000 units IM; โ‰ฅ30 kg โ†’ 1.2 million units IM.
Aspirin use in children: avoid in those <16 years unless specifically indicated for ARF (Reye syndrome risk). Monitor salicylate levels closely.
๐Ÿคฐ Pregnancy
BPG is safe in pregnancy โ€” continue secondary prophylaxis.
Warfarin is teratogenic โ€” switch to LMWH (enoxaparin 1 mg/kg BD, adjusted to anti-Xa levels) in first trimester and near delivery.
Severe valvular disease in pregnancy carries high risk โ€” multidisciplinary management at a tertiary centre with cardiac surgery capability.
๐Ÿซ˜ Renal Impairment
BPG: no dose adjustment required.
Aspirin: use with caution in CKD; monitor for bleeding.
Warfarin: INR monitoring may be less predictable in CKD. Avoid NSAIDs for arthritis in renal impairment.
๐Ÿ›ก๏ธ Immunocompromised
GAS carriage may persist โ€” ensure eradication is confirmed (repeat throat swab at 1 week post-treatment).
HIV-positive individuals in endemic areas should be assessed for ARF risk. Immunocompromise does not increase ARF risk per se but may complicate treatment.
๐Ÿ‘ด Elderly
ARF is rare in adults over 40 years. "New" ARF in older adults should prompt consideration of alternative diagnoses (endocarditis, viral arthritis, crystal arthropathy).
RHD may present late with heart failure or AF from valve disease diagnosed decades earlier. Secondary prophylaxis duration decisions must be individualised.

Aboriginal and Torres Strait Islander Health Considerations

Aboriginal and Torres Strait Islander Health

ARF and RHD are among the starkest markers of health inequity in Australia. Over 95% of new ARF cases occur in Aboriginal and Torres Strait Islander peoples. Rates of RHD are 20โ€“40 times higher than in non-Indigenous Australians. ARF is almost entirely preventable through improved housing, reduced overcrowding, and access to prompt antibiotic treatment for GAS pharyngitis.

The RHDAustralia Australian guideline for prevention, diagnosis and management of acute rheumatic fever and rheumatic heart disease (3rd edition, 2020) is the definitive clinical standard.

Incidence
ARF incidence in Aboriginal and Torres Strait Islander children (5โ€“14 years) in the NT can exceed 200 per 100,000 โ€” among the highest rates globally. RHD prevalence in Indigenous Australians: ~50 per 1,000 in some remote communities.
Social determinants
Overcrowded housing, inadequate water and sanitation, poor access to primary health care, and high GAS carriage rates drive the ongoing epidemic. Close the Gap initiatives must address housing as a health intervention.
BPG adherence
Adherence to secondary prophylaxis is the most modifiable factor in RHD outcomes. Aboriginal Community Controlled Health Services (ACCHS) employ RHD coordinators, patient support officers, and recall systems. Community-based strategies (home visits, school-based injections, yarns with families) improve uptake.
RHD Register
The Australian RHD Register (managed by RHDAustralia and jurisdictional health departments in NT, QLD, WA, SA) tracks all notified cases, injection schedules, and echocardiographic findings. Clinicians should ensure all ARF cases are notified where required.
Remote and rural access
Portable echocardiography, telehealth cardiology reviews, and RFDS-supported BPG injection delivery are critical in remote communities. Echocardiographic screening programmes using handheld devices and store-and-forward telehealth have been validated in NT and QLD.
Cultural safety
Engagement with Aboriginal health workers and Torres Strait Islander health practitioners is essential. Culturally appropriate education materials, Yarning about ARF, and family-centred care improve health literacy and adherence. Interpreter services should be used when indicated.
Notification
ARF is notifiable in NT, QLD, SA, and WA. Clinicians must notify cases to enable registry tracking, recall, and epidemiological surveillance. Failure to notify denies patients access to coordinated support services.
โœ…
Endgame RHD: The Australian Government's Endgame RHD programme aims to eliminate ARF and RHD as public health problems. This includes improved housing in remote communities (National Partnership on Remote Housing), expanded GAS vaccine research, and universal echocardiographic screening in endemic areas.

Quick Reference โ€” Acute ARF Management

Eradicate GAS (all cases)
Benzathine penicillin G 1.2 MU IM ร— 1 (or 600,000 U if <30 kg)
Single dose
Penicillin allergy โ†’ azithromycin 500/250 mg PO ร— 5 days
Arthritis / mild carditis
Aspirin 80โ€“100 mg/kg/day PO (max 6 g/day)
4โ€“6 weeks
Monitor salicylate levels. Taper as CRP normalises.
Moderateโ€“severe carditis
Prednisolone 1โ€“2 mg/kg/day PO (max 60 mg/day)
2โ€“3 weeks, then taper to aspirin
Heart failure: diuretics, ACEi. Avoid digoxin.
Sydenham chorea
Supportive. Severe: sodium valproate 15โ€“20 mg/kg/day PO
Variable (6 weeks to 6 months)
Commence secondary prophylaxis even without GAS evidence.
Secondary prophylaxis
Benzathine penicillin G 1.2 MU IM every 21 days
5 years to lifelong (depends on valve status)
High-risk: every 14 days. Allergy โ†’ erythromycin 250 mg PO BD.

๐Ÿ“š References

  1. 1. RHDAustralia (ARF/RHD writing group). Australian guideline for prevention, diagnosis and management of acute rheumatic fever and rheumatic heart disease. 3rd edition. Darwin: Menzies School of Health Research; 2020.
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  3. 3. Australian Institute of Health and Welfare (AIHW). Rheumatic heart disease in Australia. Cat. no. CVD 87. Canberra: AIHW; 2023.
  4. 4. Parnaby MG, Carapetis JR. Rheumatic fever in Indigenous Australian children. J Paediatr Child Health. 2010;46(9):527โ€“533.
  5. 5. Zรผhlke LJ, Steer AC. Estimates of the global burden of rheumatic heart disease. Glob Heart. 2013;8(3):185โ€“192.
  6. 6. Karthikeyan G, Connolly SJ, Ntsekhe M, et al. The INVICTUS rheumatic heart disease research programme: rationale, design and baseline characteristics of a randomized trial of rivaroxaban compared with warfarin in patients with rheumatic heart disease and atrial fibrillation. Am Heart J. 2020;227:81โ€“89.
  7. 7. Engelman D, Kado JH, Remenyi B, et al. Screening for rheumatic heart disease: quality and agreement of focused cardiac ultrasound by briefly trained health workers. BMC Cardiovasc Disord. 2016;16:30.
  8. 8. Wyber R, Taubert K, Marko S, Carapetis JR. Benzathine penicillin G for the management of RHD: concerns about feasibility and delivery, and opportunities for improvement. Int J Cardiol. 2013;166(2):279โ€“286.
  9. 9. Francis JR, Gargan C, Remenyi B, et al. High rates of Group A streptococcal pharyngitis and acute rheumatic fever in Aboriginal children in the Northern Territory. Med J Aust. 2023;218(5):229โ€“234.
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