Paeds · cardiology

Acute rheumatic fever and rheumatic heart disease

Also known as acute rheumatic fever · ARF · rheumatic heart disease · RHD · Sydenham chorea · rheumatic carditis · post-streptococcal carditis

A fellowship approach to acute rheumatic fever and rheumatic heart disease: the child with a sore throat whose immune response scars a mitral valve for life, the Jones criteria that demand echo, the benzathine penicillin that prevents recurrence, and the disease-of-inequity reality that makes this a scourge of Indigenous Australia and the developing world rather than a closed chapter of Western medicine.

high9 referencesUpdated 13 July 202624 min readVerification in progress

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Red flags

Never diagnose ARF without evidence of preceding GAS — raised or rising ASO titre, positive throat swab, or recent scarlet fever — except in isolated chorea or indolent carditisPolyarthritis is the commonest major criterion but is never diagnostic alone — it must be paired with a second major or two minors plus evidence of GASSubclinical carditis detected only on echocardiography counts as a major criterion in the 2015 revised Jones criteria — a normal child with valve pathology on echo has ARF until proven otherwiseChorea can present months after the streptococcal infection when ASO titres have normalised — diagnose clinically without serology and start prophylaxis immediatelyBenzathine penicillin G must be given every 21 to 28 days — intervals longer than 28 days are the commonest reason for recurrence and the progression to RHDRHD in pregnancy is a leading cause of maternal mortality in endemic regions — assess and intervene before conception, not at the first antenatal visit

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Cardiology - recognise and investigate acquired heart diseaseGeneral and Community Paediatrics - disease prevention and health promotionGrowth and DevelopmentCardiology - diagnose and manage acute rheumatic fever and rheumatic heart diseaseAcquired heart disease - apply the Jones criteria and plan secondary prophylaxisRenewed curriculum for first-year trainees from 2027 - Learning goal 6: Clinical management - essential general paediatricsCardiology 10-12Acute presentations 10-12Clinical ApplicationsLong CasesShort Cases8. Cardiology - acquired heart disease including rheumatic fever11. Child health and immunisation - disease prevention4. Professional skills and knowledge: Patient managementFoundation of Practice (FOP)Theory and Science (TAS)Applied Knowledge in Practice (AKP)ClinicalHistoryCommunicationVideoGeneral Pediatrics Content Outline - D15 Cardiology (5%)General Pediatrics Content Outline - Universal Task 4: Management and TreatmentGeneral Pediatrics Content Outline - Universal Task 2: Health MaintenancePatient Care 4: Clinical ReasoningPatient Care 5: Patient ManagementMedical Knowledge 1: Congenital anomalies and acquired diseaseSystems-Based Practice 2: Population health and advocacyMedical ExpertPediatrics: Foundations EPA #8 - Communicating assessment findings and management plans to patients and familiesPediatrics: Cardiology applied domain

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Target exams

RACP General PaediatricsRACP DWERACP DCEMRCPCH Clinical

Red flags

Life stages

preschoolschool-ageadolescentyoung-adult-transition

Care settings

preventive-medical-homecommunity-schooloutpatientwardrural-remotetelehealth

Clinical exam formats

written-only

Board mappings

The fellowship answer

Acute rheumatic fever is a delayed autoimmune consequence of group A streptococcal pharyngitis, and rheumatic heart disease is the permanent valve damage left behind when recurrent episodes go untreated. The fellowship task is to recognise the Jones criteria, confirm with echocardiography, eradicate the streptococcus, and commit the child to years of secondary prophylaxis so that one episode does not become a lifetime of mitral stenosis. [5] [1]

The clinical thread runs from a school-aged child with a sore throat, through a latent period of two to three weeks, to a migratory polyarthritis or a new murmur of mitral regurgitation, to the echocardiogram that shows valvulitis, and finally to the benzathine penicillin injection every three to four weeks that prevents the next episode and protects the valve. The disease is common where crowding and poverty converge, and it is vanishingly rare where it does not — which is why this is a disease of inequity, not biology. [5] [3]

A nine-year-old Aboriginal boy in remote Northern Territory presents with a swollen, painful knee that migrated to the elbow over two days, a fever, and a new heart murmur; or a twelve-year-old girl of Pacific Islander background is admitted with dyspnoea and a harsh apical pansystolic murmur four weeks after a sore throat; or a teenager develops involuntary grimacing and writhing movements of the hands and face weeks after a febrile illness. The fellowship task in each is the same: apply the Jones criteria, confirm the streptococcal link with serology, request echocardiography, and commit to secondary prophylaxis. [3] [1]

The five moves — Suspect, Score, Scan, Strike, Sustain

Run the pathway as five moves. Suspect ARF in any school-aged child with a sore throat followed two to three weeks later by arthritis, a murmur, or chorea, especially if Indigenous or from an endemic region. Score with the revised Jones criteria — two majors, or one major and two minors, plus evidence of preceding GAS. Scan with echocardiography, because subclinical carditis now counts as a major criterion. Strike with benzathine penicillin G intramuscularly every twenty-one to twenty-eight days. Sustain the prophylaxis for years — until at least age twenty-one, and longer if rheumatic heart disease is already established. [1] [3]

Overview & Definition

Acute rheumatic fever is a delayed, nonsuppurative inflammatory disease that follows untreated group A streptococcal pharyngitis by two to three weeks, affecting the heart, joints, skin, and central nervous system. Rheumatic heart disease is the chronic, progressive, and largely irreversible damage to the cardiac valves — predominantly the mitral — that results from one or more episodes of acute rheumatic fever. The two are one disease viewed at different points in time. [5] [1]

The fellowship framing rests on a single immunological truth. Group A streptococcal M proteins share antigenic similarity with human cardiac myosin, valvular endothelium, and the basal ganglia, so the antibodies and T cells raised against the streptococcus cross-react with host tissue. This molecular mimicry is the mechanism that turns a sore throat into a scarred mitral valve, and it is why the disease targets the heart rather than the throat. [5] [7]

The clinical importance comes from what happens if the disease is missed or undertreated. A single episode of acute rheumatic fever may resolve fully, but each recurrent episode driven by a new streptococcal infection cumulatively damages the valve, and established rheumatic heart disease carries a heavy burden of heart failure, atrial fibrillation, stroke, infective endocarditis, and premature death. The stakes are highest in pregnancy, where undiagnosed rheumatic heart disease remains a leading cause of maternal mortality in endemic regions. [8] [4]

Paediatrics Fellowship Pro

You have read the opening of this topic. The complete unit — every section and its primary-source references — is part of the Paediatrics Fellowship fellowship atlas.

References9Show ledger

[1]Gewitz MH, Baltimore RS, Tani LY, et al. Revision of the Jones Criteria for the diagnosis of acute rheumatic fever in the era of Doppler echocardiography: a scientific statement from the American Heart Association. Circulation, 2015.PMID 25908771
[2]Reményi B, Wilson N, Steer A, Ferreira B, Kado J, Kumar K, Lawrenson J, Maguire G, Marijon E, Mirabel M, Mocumbi AO, Mota C, Paar J, Saxena A, Scheel J, Stirling J, Viali S, Balekundri VI, Wheaton G, Zühlke L, Carapetis J. World Heart Federation criteria for echocardiographic diagnosis of rheumatic heart disease--an evidence-based guideline. Nat Rev Cardiol, 2012.PMID 22371105
[3]Ralph AP, Noonan S, Wade V, et al. The 2020 Australian guideline for prevention, diagnosis and management of acute rheumatic fever and rheumatic heart disease. Med J Aust, 2021.PMID 33190309
[4]Watkins DA, Roth G. Global Burden of Rheumatic Heart Disease. N Engl J Med, 2018.PMID 29298146
[5]Carapetis JR, McDonald M, Wilson NJ. Acute rheumatic fever. Lancet, 2005.PMID 16005340
[6]Wyber R, Wade V, Anderson A, et al. Rheumatic heart disease in Indigenous young peoples. Lancet Child Adolesc Health, 2021.PMID 33705693
[7]Carapetis JR, Steer AC, Mulholland EK, Weber M. The global burden of group A streptococcal diseases. Lancet Infect Dis, 2005.PMID 16253886
[8]Liaw J, Walker B, Hall L, et al. Rheumatic heart disease in pregnancy and neonatal outcomes: A systematic review and meta-analysis. PLoS One, 2021.PMID 34185797
[9]Grimaldi A, Ammirati E, Mirabel M, et al. Challenges of using ultrasounds for subclinical rheumatic heart disease screening. Int J Cardiol, 2013.PMID 23245797

Acute rheumatic fever and rheumatic heart disease

Also known as acute rheumatic fever · ARF · rheumatic heart disease · RHD · Sydenham chorea · rheumatic carditis · post-streptococcal carditis

high9 referencesUpdated 13 July 202624 min readVerification in progress

Practise this topic

Overview & Definition

Paediatrics Fellowship Pro

You have read the opening of this topic. The complete unit — every section and its primary-source references — is part of the Paediatrics Fellowship fellowship atlas.

References9Show ledger

[1]Gewitz MH, Baltimore RS, Tani LY, et al. Revision of the Jones Criteria for the diagnosis of acute rheumatic fever in the era of Doppler echocardiography: a scientific statement from the American Heart Association. Circulation, 2015.PMID 25908771
[2]Reményi B, Wilson N, Steer A, Ferreira B, Kado J, Kumar K, Lawrenson J, Maguire G, Marijon E, Mirabel M, Mocumbi AO, Mota C, Paar J, Saxena A, Scheel J, Stirling J, Viali S, Balekundri VI, Wheaton G, Zühlke L, Carapetis J. World Heart Federation criteria for echocardiographic diagnosis of rheumatic heart disease--an evidence-based guideline. Nat Rev Cardiol, 2012.PMID 22371105
[3]Ralph AP, Noonan S, Wade V, et al. The 2020 Australian guideline for prevention, diagnosis and management of acute rheumatic fever and rheumatic heart disease. Med J Aust, 2021.PMID 33190309
[4]Watkins DA, Roth G. Global Burden of Rheumatic Heart Disease. N Engl J Med, 2018.PMID 29298146
[5]Carapetis JR, McDonald M, Wilson NJ. Acute rheumatic fever. Lancet, 2005.PMID 16005340
[6]Wyber R, Wade V, Anderson A, et al. Rheumatic heart disease in Indigenous young peoples. Lancet Child Adolesc Health, 2021.PMID 33705693
[7]Carapetis JR, Steer AC, Mulholland EK, Weber M. The global burden of group A streptococcal diseases. Lancet Infect Dis, 2005.PMID 16253886
[8]Liaw J, Walker B, Hall L, et al. Rheumatic heart disease in pregnancy and neonatal outcomes: A systematic review and meta-analysis. PLoS One, 2021.PMID 34185797
[9]Grimaldi A, Ammirati E, Mirabel M, et al. Challenges of using ultrasounds for subclinical rheumatic heart disease screening. Int J Cardiol, 2013.PMID 23245797