Guideline summary

The 2020 Australian guideline for prevention,

diagnosis and management of acute rheumatic fever
and rheumatic heart disease
Anna P Ralph1 , Sara Noonan2, Vicki Wade2, Bart J Currie1,3

A
cute rheumatic fever (ARF) is an autoimmune disease Abstract
triggered in some children and young adults by infec-
Introduction: Acute rheumatic fever (ARF) and rheumatic heart
tion with group A streptococci.1 Repeated or severe ARF disease (RHD) cause significant morbidity and premature mortality
leads to rheumatic heart disease (RHD), with high morbidity among Australian Aboriginal and Torres Strait Islander peoples.
and mortality. Group A streptococcal infection risk is associated RHD Australia has produced a fully updated clinical guideline in
with socio-­economic factors such as household crowding.2 High response to new knowledge gained since the 2012 edition. The
rates occur in Australian Aboriginal and Torres Strait Islander guideline aligns with major international ARF and RHD practice
populations, especially in rural or remote settings. Prevalence guidelines from the American Heart Association and World Heart
estimates for definite RHD in Australian children range from Federation to ensure best practice. The GRADE system was used to
<  1 per 1000 population in low risk children, to 333 to 504 per assess the quality and strength of evidence where appropriate.
1000 people in high risk populations. High rates of disease also Main recommendations: The 2020 Australian guideline details
occur among Māori and Pacific Islander populations.5 best practice care for people with or at risk of ARF and RHD.
It provides up-­to-­date guidance on primordial, primary and
Given this high burden of disease in Australian subpopulations, secondary prevention, diagnosis and management, preconception
yet rarity in the broader population, clinical practice guidelines and perinatal management of women with RHD, culturally safe
are essential. Australian ARF and RHD guidelines were first practice, provision of a trained and supported Aboriginal and Torres
produced in 20076 and revised in 2012.7 The 2020 guideline, de- Strait Islander workforce, disease burden, RHD screening, control
veloped in accordance with the National Health and Medical programs and new technologies.
Research Council standards for guidelines8 by RHDAustralia Changes in management as a result of the guideline: Key
(the national support unit for ARF and RHD), builds on these and changes include updating of ARF and RHD diagnostic criteria;
incorporates new evidence from trials and other research, new change in secondary prophylaxis duration; improved pain
medication options — such as expanded roles for corticosteroids, management for intramuscular injections; and changes to antibiotic
regimens for primary prevention. Other changes include an
and use of non-­vitamin K antagonist oral anticoagulants — and
emphasis on provision of culturally appropriate care; updated
new expert consensus opinion including revised parameters for burden of disease data using linked register and hospitalisations
ARF and RHD diagnosis. The guideline9 is freely available online, data; primordial prevention strategies to reduce streptococcal
accompanied by a video summary of changes, key information, infection addressing household overcrowding and personal
useful tables and figures, an app for smart devices containing a hygiene; recommendations for population-­based echocardiographic
condensed version, and an interactive ARF diagnosis calculator.10 screening for RHD in select populations; expanded management
All electronic resources align with the 2020 edition and the 2015 guidance for women with RHD or ARF to cover contraception,
American Heart Association (AHA) revised Jones criteria for di- antenatal, delivery and postnatal care, and to stratify pregnancy
agnosis of ARF11 to ensure consistency and best practice. risks according to RHD severity; and a priority classification system
for presence and severity of RHD to align with appropriate timing
of follow-­up.
Methods

A guideline steering committee was formed comprising (53% urban, 18% rural, 29% remote) indicated that a freely avail-
RHDAustralia members and partners, ARF and RHD experts, able digital version as well as print copies was desired, with a
and Aboriginal and Torres Strait Islander advisors. The steering quick guide format as additional detail. Each chapter structure
committee provided high level strategic direction and advice, therefore comprises a key information section followed by an
content support and endorsement of the final version. evidence-­based discussion and, where relevant, case studies.
RHDAustralia's Senior Aboriginal Cultural Advisor led a re- The steering committee developed chapter headings and invited
view of all content. A sociocultural framework highlighted multidisciplinary experts (Indigenous and non-­Indigenous med-
social and emotional wellbeing, and ensured that recommen- ical, nursing, research and allied health specialties) from among
dations adhered to culturally safe practice. An Aboriginal and Australian and New Zealand topic authorities (Supporting
Torres Strait Islander advisory group provided expert cul- Information, Table 1). Authors reviewed relevant chapters from
tural advice, with consumer input from community members the 2012 edition (unless developing a new chapter), conducted
(Champions4Change12). Insights from the Champions introduce literature reviews using MEDLINE and PubMed Central, and
each chapter, such as: “You need to understand the community considered in-­process citations, research underway and grey lit-
and the problems that they are facing and then, and only then, erature. The lived experience of ARF and RHD was represented
you can help them to get rid of RHD”.9 through patient stories and case studies.
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A targeted health workforce survey was conducted to inform The Grading of Recommendations Assessment, Development
the format and scope of the new edition. The 196 respondents and Evaluation (GRADE) system13 was applied by writing

1
Menzies School of Health Research, Darwin, NT. 2 RHDAustralia, Darwin, NT. 3 Royal Darwin Hospital, Darwin, NT. anna.ralph@menzies.edu.au ▪ 1
doi: 10.5694/mja2.50851
 Guideline summary
groups where appropriate. Quality of evidence was classified for ARF and RHD control in such cases; examples include defini-
from A (high) to D (very low) and the strength of the recommen- tion of ARF risk groups, duration of secondary prophylaxis, and
dations graded as 1 (strong) or 2 (weak). For example, GRADE recommendations for community echocardiogram screening for
1A indicates that the recommendation should be applied to most active case finding in high risk communities. Changes from the
patients without reservation, while GRADE 2D indicates that previous edition are summarised in Box 1.
evidence is lacking but expert consensus weakly supports the
recommendation. New recommendations not firmly supported Feedback was invited from multidisciplinary content experts
by evidence or where evidence was contentious were discussed in Australia and New Zealand, and recommendations were
until consensus was reached, or until an acceptable majority po- incorporated where appropriate. Endorsement was sought
sition was obtained considering the available evidence. The aim from peak health policy, advocacy and training organisations
was to present feasible rather than highly aspirational guidance (Supporting Information, Table 2). The editors reviewed the

1  Summary of changes from the 2012 edition

Chapter Changes

Primary prevention • Updated definition of high risk groups for empirical treatment of throat and skin infections.
• New recommendations for management of group A streptococcal skin infections to prevent ARF.
• BPG dosing streamlined to three dose bands for simplicity compared with the previously recommended five dose bands.
• Option for tablet dosing of cotrimoxazole to treat group A streptococcal impetigo if syrup is in short supply.

Diagnosis of ARF • In low risk populations, subclinical carditis is now a major criterion.
• In low risk populations, ESR as a minor criterion is now ≥ 60 mm rather than ≥ 30 mm.
• In low risk populations, fever as a minor criterion is now ≥ 38.5°C rather than ≥ 38.0°C.
• For all populations, a definite recurrent episode of ARF after documented history of ARF or RHD now requires two major, or one
major and two minor, or three minor criteria (plus evidence of preceding group A streptococcal infection) rather than two major, or
one major and one minor, or three minor criteria.

Management of ARF • Probable ARF – “highly suspected” renamed “probable” ARF.

• Probable ARF – “uncertain” renamed “possible” ARF.
• Suspected ARF refers to presentations not yet allocated to a category of definite, probable, possible ARF or no ARF.
• Non-steroidal anti-inflammatory drugs naproxen and ibuprofen are now recommended first line in children for joint pain, ahead of
aspirin.
• A revised hierarchy of therapeutic approaches for Sydenham chorea is provided.
Diagnosis of RHD • World Health Federation guidelines for the diagnosis of RHD, validated in high and low prevalence populations, are endorsed and
described.
• Echocardiographic features of severity are aligned with updated international guidelines for valvular heart disease (European
Society of Cardiology 2017 14 and American Heart Association/American College of Cardiology 201415).
• Exercise stress testing has been included as an additional testing modality in determining severity of RHD and planning for
intervention.
• A new section on distinguishing RHD from other valvular pathology is provided.
• The role of new echocardiography technology in RHD diagnosis, including hand-held and portable options, is described.

Secondary prophylaxis • New recommendations are made for the duration of secondary prophylaxis for some groups.
• A focus is provided on the responsibility of health services to provide a culturally safe service, and for staff to be culturally
competent in the management of secondary prophylaxis.
• The new term benzathine benzylpenicillin G replaces benzathine penicillin G.
• BPG is given in units, not weight (g or mg) in keeping with Therapeutic Goods Administration requirements for labelling.
• Instructions for intramuscular injection of BPG at the ventrogluteal site are provided.
• New approaches are given to managing injection pain, fear and distress, including the option of medically prescribed lidocaine
(lignocaine) and procedural sedation.

Management of RHD • Updated priority definitions have been developed to align with appropriately timed follow-up.
• Transcatheter valve replacement is discussed as an option in younger individuals.
• A focus on transition from paediatric to adult services is provided.
• A definition of non-valvular atrial fibrillation is provided and the role of CHA 2DS 2-VA score to determine thromboembolic risk
described.
• The role of non-vitamin K antagonist oral anticoagulants (novel oral anticoagulants) in RHD is detailed.
Women and girls with • Care pathways for girls and women with RHD are provided.
RHD • Care pathways and a referral algorithm are provided for pregnant women with RHD.
• A new section on transition to adult care, reproductive health, and preconception care is included.
• Strategies for a well-planned pregnancy and delivery are discussed.
• Anticoagulation during pregnancy has been revised and expanded.

New chapters • Culture and workforce

• Burden of ARF and RHD
•
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Primordial prevention and social determinants of ARF

• Primary prevention
• Screening for RHD
• RHD control programs
• New technologies

2 ARF = acute rheumatic fever; BPG = benzathine benzylpenicillin G; C HA 2DS 2-­VA = congestive heart failure, hypertension, age, diabetes, stroke, vascular, age; ESR = erythrocyte ­s edimentation
rate; RHD = rheumatic heart disease. ◆
 Guideline summary
semi-­f inal draft to ensure inclusion of any recently
published or in-­press literature, consistency across 2  Socio-­ecological model underpinning the guidelines
chapters, clarity for practitioners, and alignment
with other Australian and international guidelines.
Where recommendations departed from other local
guidelines (eg, Australian Therapeutic Guidelines:
Antibiotic [https://www.tg.org.au] or the Central
Australian Rural Practitioners Association Standard
Treatment Manual16), this was communicated to
respective editors to encourage alignment in their
next edition.

Culture and workforce

The guideline is underpinned by a strong focus on
the provision of culturally safe care, in line with na-
tional recommendations.17 Cultural safety requires
health care providers and institutions to recognise
their own culture and ameliorate approaches which
diminish, demean or disempower the cultural iden-
tities and wellbeing of patients.18 A socio-­ecological
model was developed for the guideline (Box 2), de-
scribing influences shaping Aboriginal and Torres
Strait Islander peoples’ health care interactions. The
value of fostering a strong Aboriginal health work-
force in delivering care is highlighted to support ef-
fective client engagement.19,20

Burden of disease
We developed a new chapter on burden of dis- * Reproduced with permission from Menzies School of Health Research, Charles Darwin University,
ease, using original data from the End RHD in Darwin, Australia, which holds copyright: https://www.rhdau​s tral​ia.org.au/resou​rces/2020-guide​line-
21 preve​ntion-diagn​osis-and-manag​ement-acute-rheum​atic-fever-and-rheum​atic.9 ◆
Australia Study of Epidemiology (ERASE) Project,
Australian Institute of Health and Welfare data22
and other sources. Since the early 1990s, ARF has occurred al- penicillin the first line choice (GRADE 1B).26–28 Impetigo caused
most exclusively in young Aboriginal and Torres Strait Islander by group A streptococcus is very common among Aboriginal
people (94% of cases; 33% affecting 5–14-­year-­olds). The recog- and Torres Strait Islander children living in remote areas, with
nised female predominance in rates of ARF (56%) and RHD (61%) almost one in two affected at any time.29 Identification, treatment
is reiterated.22 and prevention of group A streptococcal skin infections may
help reduce ARF burden (GRADE 1B).30–34 Group A streptococ-
Recommendations cal skin infections should be treated with cotrimoxazole orally or
benzathine benzylpenicillin G intramuscularly (GRADE 1A).35
Primordial prevention and social determinants of ARF Dosing regimens are provided in the full guideline (Table 5.3).9
Primordial prevention reduces community-­
based risk factors to prevent the occurrence of
a disease. ARF is attributable to social deter- 3  Risk groups for acute rheumatic fever (ARF) and rheumatic heart disease (RHD)*
minants of health, including quality of hous- Risk Setting
ing, level of household occupancy, and access
to health hardware including washing facili-
High risk • Living in an ARF-endemic setting†

ties.2,23 The Nine Healthy Living Practices24,25

• Aboriginal and Torres Strait Islander peoples living in rural or remote
settings
are simple recommendations to reduce the • Aboriginal and Torres Strait Islander peoples, and Māori and/or Pacific
risk of injury, communicable diseases and Islander peoples living in metropolitan households affected by crowding
environmental diseases in household set- and/or lower socio-economic status

tings. They are used as the framework for this • Personal history of ARF/RHD and age < 40 years
chapter. Each Practice was reviewed and the May be high • Family or household recent history of ARF/RHD
evidence graded regarding their likely associ- risk • Household overcrowding (> 2 people/bedroom) or low socio-economic
status
ation with reducing streptococcal infection at
community level. The two practices for which
• Migrant or refugee from low or middle income country and their children
the evidence is graded as strong are “washing Additional • Prior residence in a high ARF risk setting
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people” and “reducing negative impacts of considerations • Frequent or recent travel to a high ARF risk setting

overcrowding”.
which increase • Aged 5–20 years (peak years for ARF)
risk

Primary prevention *Reproduced with permission from Menzies School of Health Research, Charles Darwin University, Darwin,
Australia, which holds copyright: https://www.rhdau​s tral​ia.org.au/resou​rces/2020-guide​line-preve​ntion-diagn​
osis-and-manag​ement-acute-rheum​atic-fever-and-rheum​atic.9 †Populations where community ARF/RHD rates are
People at high risk of ARF (Box 3) require em- 3
pirical antibiotic treatment for sore throat, with
◆
known to be high; eg, ARF incidence > 30/100 000 per year in 5–14-­year-­olds or RHD all-­age prevalence > 2/1000.
 Guideline summary

4  2020 Australian criteria for acute rheumatic fever (ARF) diagnosis*

High risk groups† Low risk groups

Definite initial episode • 2 major manifestations + evidence of preceding group A streptococcal infection, OR
of ARF • 1 major + 2 minor manifestations + evidence of preceding group A streptococcal infection‡

Definite recurrent ‡ • 2 major manifestations + evidence of preceding group A streptococcal infection, OR

episode of ARF • 1 major + 2 minor manifestations + evidence of preceding group A streptococcal infection,‡ OR
in patient with • 3 minor manifestations + evidence of a preceding group A streptococcal infection‡
documented history of
ARF or RHD

Probable or possible A clinical presentation in which ARF is considered a likely diagnosis but falls short in meeting the criteria by either:
ARF (first episode or • one major or one minor manifestation, OR
recurrence§) • no evidence of preceding group A streptococcal infection (streptococcal titres within normal limits or titres not measured)
Such cases should be further categorised according to the level of confidence with which the diagnosis is made:
• probable ARF (previously termed “probable: highly suspected”)
• possible ARF (previously termed “probable: uncertain”)
Major manifestations • Carditis (including subclinical evidence of rheumatic • Carditis (including subclinical evidence of rheumatic valvulitis on
valvulitis on echocardiogram) echocardiogram)
• Polyarthritis¶ or aseptic monoarthritis or • Polyarthritis¶
polyarthralgia • Sydenham chorea** ††
• Sydenham chorea** †† • Erythema marginatum
• Erythema marginatum • Subcutaneous nodules
• Subcutaneous nodules
Minor manifestations • Fever‡‡ ≥ 38°C §§ • Fever ≥ 38.5°C
• Monoarthralgia • Polyarthralgia or aseptic monoarthritis§§
• ESR ≥ 30 mm/h or CRP ≥ 30 mg/L • ESR ≥ 60 m/h or CRP ≥ 30 mg/L
• Prolonged PR interval on ECG¶¶ • Prolonged PR interval on ECG¶¶

CRP = C-­reactive protein; ECG = electrocardiogram; ESR = erythrocyte sedimentation rate; RHD = rheumatic heart disease.*Reproduced with permission from Menzies School of Health
Research, Charles Darwin University, Darwin, Australia, which holds copyright: https://www.rhdau​s tral​ia.org.au/resou​rces/2020-guide​line-preve​ntion-diagn​osis-and-manag​ement-acute-
rheum​atic-fever-and-rheum​atic.9 †High risk groups are those living in communities with high rates of ARF (incidence > 30/100 000 per year in 5–14-­year-­olds) or RHD (all-­age prevalence
> 2/1000). Aboriginal and Torres Strait Islander peoples living in rural or remote settings are known to be at high risk. Data are not available for other populations but Aboriginal and Torres
Strait Islander peoples living in urban settings, Māori and Pacific Islanders, and potentially immigrants from developing countries, may also be at high risk. ‡Elevated or rising antistreptolysin
O or other streptococcal antibody, or a positive throat culture or rapid antigen or nucleic acid test for group A streptococcal infection. §Recurrent definite, probable or possible ARF requires
a time period of more than 90 days after the onset of symptoms from the previous episode of definite, probable or possible ARF. ¶A definite history of arthritis is sufficient to satisfy this
manifestation. Note that if polyarthritis is present as a major manifestation, polyarthralgia or aseptic monoarthritis cannot be considered an additional minor manifestation in the same
person. ** Chorea does not require other manifestations or evidence of preceding group A streptococcal infection, provided other causes of chorea are excluded. ††Care should be taken not
to label other rashes, particularly non-­specific viral exanthems, as erythema marginatum. ‡‡In high risk groups, fever can be considered a minor manifestation based on a reliable history
(in the absence of documented temperature) if anti-­inflammatory medication has already been administered. §§If polyarthritis is present as a major criterion, monoarthritis or arthralgia
cannot be considered an additional minor manifestation. ¶¶If carditis is present as a major manifestation, a prolonged PR interval cannot be considered an additional minor manifestation. ◆

ARF diagnosis • definite ARF meets revised Jones criteria with alternative diag-
noses excluded;
There is now alignment between Australian diagnostic crite-
ria for ARF and the AHA revised Jones criteria11 (Box 4), out- • probable ARF is an acute presentation not fulfilling criteria,
lining differences for high and low risk populations (Box 3). missing one major or one minor criterion or lacking evidence
The changes to diagnostic criteria in low risk groups include a of preceding streptococcal infection, but where ARF is still
higher temperature (≥ 38.5°C rather than ≥ 38°C), higher eryth- considered the most likely diagnosis; and
rocyte sedimentation rate (≥ 60 mm/h rather than ≥ 30 mm/h), • possible ARF applies to the same presentation type as probable
and echocardiographic evidence of valvulitis with carditis. A ARF, but where ARF is considered uncertain but cannot be
combination of major and minor criteria is used to diagnose ruled out.
ARF; major criteria including arthritis, carditis, chorea and
skin manifestations are strongly associated with ARF, while
ARF management
minor criteria such as fever and raised inflammatory markers
support the diagnosis. For all populations, definite recurrent The pillars of ARF management are eradication of the inciting
ARF now requires two major, or one major and two minor, or group A streptococcal infection using penicillin (or an alterna-
three minor criteria, rather than two major, or one major and tive if allergic to penicillin) and management of symptoms with
one minor, or three minor criteria. Alignment with the AHA is analgesic–antipyretic agents as needed (GRADE 1B). The guide-
important to promote a consistent approach to ARF diagnosis line discusses the use of corticosteroids as a potential disease-­
globally, and the changes also improve specificity of ARF di- modifying agent in severe rheumatic carditis (GRADE 2B), and
agnosis, especially in low risk populations where ARF is very to reduce severity of Sydenham chorea (GRADE 2B).
uncommon.
For definite ARF, a priority grade based on the severity of any
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All patients with suspected ARF should be hospitalised, in- accompanying RHD should be assigned, using a revised pri-
vestigated with electrocardiography and echocardiography, ority classification (Supporting Information, Table 3). The time
and have differential diagnoses excluded (GRADE 1B). Each since ARF and the severity of RHD determine the duration
episode should be categorised as initial or recurrent ARF, of secondary prophylaxis (Box 5) and the priority grade de-
4 with certainty of diagnosis indicated as definite, probable or termines frequency of reviews and echocardiograms. People
possible: diagnosed with ARF must be notified to the local public health
 Guideline summary

5  Recommended duration of secondary prophylaxisfor acute rheumatic fever (ARF) and rheumatic heart disease (RHD)*
Timing of
Conditions for ceasing echocardiography
Diagnosis Definition Duration of prophylaxis prophylaxis† after cessation‡

Possible ARF (no Incomplete features of ARF with 12 months (then reassess) • No signs and symptoms of At 1 year
cardiac involvement) normal echocardiogram and normal ARF within the previous 12
ECG§ throughout ARF episode months
• Normal echocardiogram
Probable ARF Highly suspected ARF with normal Minimum of 5 years after most recent • No probable or definite ARF At 1, 3 and 5 years
echocardiogram episode of probable ARF, or until age within the previous 5 years
21 years (whichever is longer) • Normal echocardiogram
Definite ARF (no ARF with normal echocardiogram and Minimum of 5 years after most recent • No probable or definite ARF At 1, 3 and 5 years
cardiac involvement) normal ECG§ throughout ARF episode episode of ARF, or until age 21 years within the previous 5 years
(whichever is longer) • Normal echocardiogram
Definite ARF(with ARF with carditis or RHD According to relevant RHD severity
cardiac involvement) on echocardiogram, or with
atrioventricular conduction
abnormality on ECG§ during ARF
episode

Borderline RHD Borderline RHD on echocardiogram Not usually recommended¶ Medical review
(≤ 20 years of age only) without a documented history of ARF and repeat
echocardiogram
at 1, 3 and 5 years
after diagnosis

Mild RHD†† Echocardiogram showing: • If documented history of ARF: • No probable or definite At 1, 3 and 5 years
• mild regurgitation or mild stenosis minimum of 10 years after the most ARF within the previous
of a single valve, OR recent episode of ARF, or until age 10 years, no progression of
• atrioventricular conduction 21 years (whichever is longer) RHD
abnormality on ECG§ during ARF • If NO documented history of ARF • Stable echocardiographic
episode and aged <35 years: ‡‡ minimum of features for 2 years
5 years following diagnosis of RHD
or until age 21 years (whichever is
longer)

Moderate RHD††,§§ Echocardiogram showing: • If documented history of ARF: • No probable or definite ARF Initially every
• moderate regurgitation or moderate minimum of 10 years after the most within the previous 10 years 12 months
stenosis of a single valve, OR recent episode of ARF or until age • Stable echocardiographic
• combined mild regurgitation and/or 35 years (whichever is longer) features for 2 years
mild stenosis of one or more valves • If no documented history of ARF
and aged < 35 years: ‡‡ minimum of
Examples:
5 years following diagnosis of RHD
• mild mitral regurgitation and mild
or until age 35 years (whichever is
mitral stenosis
longer)
• mild mitral regurgitation and mild
aortic regurgitation

Severe RHD§§,¶¶ Echocardiogram showing: • If documented history of ARF: • Stable valvular disease/ Initially every
• severe regurgitation or severe minimum of 10 years after the most cardiac function on serial 6 months
stenosis of any valve, OR recent episode of ARF or until age echocardiogram for 3 years,
• combined moderate regurgitation 40 years (whichever is longer) OR
and/or moderate stenosis of one or • If no documented history of ARF:††† • Patient or family preference
more valves minimum of 5 years following to cease due to advancing
diagnosis of RHD or until age 40 age and/or end of life care
Examples:
years (whichever is longer)
• moderate mitral regurgitation and
moderate mitral stenosis
• moderate mitral stenosis and
moderate aortic regurgitation, OR
• past or impending valve repair or
prosthetic valve replacement

AV = atrioventricular; ECG = electrocardiogram.*Reproduced with permission from Menzies School of Health Research, Charles Darwin University, Darwin, Australia, which holds copyright:
https://www.rhdau​s tral​ia.org.au/resou​rces/2020-guide​line-preve​ntion-diagn​osis-and-manag​ement-acute-rheum​atic-fever-and-rheum​atic.9 †All people receiving secondary prophylaxis
require a comprehensive clinical assessment and echocardiogram before cessation. Risk factors including future exposure to high streptococcal burden environments need to be considered.
‡Echocardiography may be more frequent based on clinical status and specialist review. §Normal ECG means no AV conduction abnormality during the ARF episode — including first degree
heart block, second degree heart block, third degree (complete) heart block and accelerated junctional rhythm. ¶Secondary prophylaxis may be considered in some circumstances, including
family preference, family history of rheumatic heart valve surgery, or suspected retrospective history of ARF. If prophylaxis is commenced, consider ceasing after 1–3 years if no history of
MJA 2020

ARF and if echocardiographic features have resolved or not progressed to definite RHD. ††Prophylaxis may be considered for longer in women considering pregnancy who live in high risk
circumstances for ARF. ‡‡If diagnosed with mild or moderate RHD aged ≥ 35 years (without ARF), secondary prophylaxis is not required. §§Rarely, moderate or severe RHD may improve on
echocardiogram without valve surgery. In these cases, the conditions for ceasing prophylaxis can change to follow the most relevant severity category. For instance, if moderate RHD im-
proves to mild on echocardiogram, recommendations for mild RHD can then be instigated. ¶¶Risk of ARF recurrence is low in people aged ≥ 40 years; however, lifelong secondary prophylaxis
is usually recommended for patients who have had, or are likely to need, heart valve surgery. †††If diagnosed with severe RHD aged ≥ 40 years (without ARF), specialist input is required
to determine the need for secondary prophylaxis. ◆
5
 Guideline summary
unit in accordance with Australian state and territory legis- Management of RHD
lation and be registered with the jurisdictional RHD control
program (GRADE 1B). Every patient with RHD should have access to specialist paediatric
or adult cardiology services, and coordinated transition from pae-
Diagnosis of RHD diatric to adult care (GRADE 2A). Non-­vitamin K antagonist oral
anticoagulants can be used in patients with RHD-­related atrial
The guideline provides more detail on the use of echocardio- fibrillation or elevated CHA2DS2-­VA (congestive heart disease, hy-
gram in accordance with World Heart Federation recommenda- pertension, age, diabetes, stroke, vascular) score, even if valvular
tions on echocardiographic diagnosis of RHD,36 which provide disease is present, provided there is no mitral stenosis of moder-
criteria distinguishing pathological RHD from physiological ate or greater severity and no mechanical valve in situ (GRADE
changes (GRADE 1B). Exercise testing or stress echocardiogra- 2B). For patients with moderate or severe mitral stenosis and atrial
phy is recommended when severity of symptoms and echocardi- fibrillation, vitamin K antagonists (eg, warfarin) currently remain
ographic findings are discordant (GRADE 1B). Transoesophageal the only indicated oral anticoagulant (GRADE 1B).41
echocardiography may help in planning surgical intervention
(GRADE 1B). Surgical decision making must take into consideration a patient’s
personal, social and cultural situation. Early engagement of a
RHD is also notifiable in Western Australia, South Australia, multidisciplinary team is essential to determine the appropri-
Northern Territory, Queensland, and New South Wales (RHD ate choice and timing of intervention. Surgical options include
for people aged < 35 years).5 repair, bioprosthetic or mechanical valve replacement, and tran-
scatheter valve replacement. Key considerations are the patient’s
Screening for RHD age, risks of anticoagulation, anticipated adherence, plans for
Population screening for RHD may provide more accurate future pregnancy, and durability of valve repair and prosthesis.
estimates of disease burden and an opportunity to initiate Antibiotic prophylaxis for endocarditis prevention with amoxy-
management for people with previously unrecognised RHD. cillin (first line) is recommended in all people with RHD un-
Population-­based screening using auscultation, inaccurate for dergoing invasive procedures as defined in Table 11.5 of the
detecting RHD, is not recommended (GRADE 1A). Screening guideline9 (GRADE 1C).
using echocardiography can accurately detect previously un-
diagnosed RHD (GRADE 1A). Echocardiographic screening Females with RHD
procedures have evolved using different technologies and op-
erators with varying levels of expertise.4 Echocardiographic About 61% of RHD cases in Australia occur in females.5 Women
screening for RHD meets some but not all public health crite- with moderate or greater mitral stenosis, severe mitral or aortic
ria for community screening.37 The disease does place a sig- regurgitation, severe aortic stenosis, pulmonary hypertension or
nificant burden on at-­risk populations, there is a latent stage heart failure are at high risk of cardiac events during pregnancy
that can be identified, and there is treatment in the form of sec- and have an elevated chance of adverse fetal outcomes. A left
ondary prophylaxis and cardiological or surgical intervention. ventricular ejection fraction < 30% or reduced systolic function
However, the impact of secondary prophylaxis on the trajectory with New York Heart Association class III–IV symptoms is as-
of screen-­detected RHD is not yet defined, and feasible com- sociated with high maternal morbidity or mortality, and preg-
munity screening tools have thus far demonstrated inadequate nancy is strongly discouraged.42 Conversely, selected women
sensitivity and specificity.4 While there remains insufficient with mild RHD can safely conceive and have children. In the
evidence to recommend routine population-­level echocardio- Northern Territory, 2–3% of annually recorded Aboriginal preg-
graphic screening for RHD in Australia as a method of disease nancies are in women with RHD. Women with mild RHD may
detection and control (GRADE 2B), it is recognised that echo- be able to give birth on Country, an important cultural practice
cardiographic community screening is valuable under specific for many Aboriginal and Torres Strait Islander people.43
circumstances such as clusters of ARF or suspected extreme Pre-­conception diagnosis of RHD is critical to optimise man-
rates of RHD.4 agement including potential surgery. Long acting, revers-
ible contraceptives (eg, intra-­
uterine contraceptive devices,
Secondary prophylaxis ARF etonogestrel implants) are recommended for women who
Secondary prophylaxis comprises regular administration of an- agree to avoid pregnancy after advice. Oestrogen-­containing
tibiotics after diagnosis of ARF or RHD to prevent future group contraceptives are associated with elevated risk of thrombosis
A streptococcal infections and ARF recurrence. Group A strep- (GRADE 1A) and should be avoided if additional thrombosis
tococcus does not develop resistance to penicillin, although one risks are present.
instance of acquisition of reduced ampicillin susceptibility has A pregnant woman in a high risk group for ARF and RHD who
been reported.38 Long acting benzathine benzylpenicillin G de- presents with breathlessness, orthopnoea, wheeze or worsening
livered every 28 days is the first line recommendation for ARF fatigue should be investigated with an echocardiogram (GRADE
prophylaxis (GRADE 1B). Previously, secondary prophylaxis was 1A). Normal vaginal delivery is generally preferred for women
recommended in Australia for at least 10 years after the most re- with RHD. Epidural anaesthesia (after appropriately timed,
cent episode of ARF or until 21 years of age, whichever comes short term cessation of any anticoagulation) may be indicated to
later. The 2020 guideline recommends secondary prophylaxis reduce tachycardia and hypertension that can precipitate acute
MJA 2020

for 5 years after the most recent episode of ARF or until 21 years heart failure during delivery.
of age if there has been no acute cardiac involvement evident on
electrocardiograph or echocardiogram during ARF, and follow-
RHD control programs
­up and end-­of-­treatment echocardiograms confirm ongoing ab-
sence of valvular involvement (Box 5). This is more aligned with Comprehensive RHD control programs can provide effective ap-
6 international guidelines39,40 and is supported by Australian reg- proaches to reducing the burden of RHD (GRADE 1B). RHD con-
ister data. trol programs in Australia maintain register and recall systems
 Guideline summary
for secondary prophylaxis and optimum clinical management; Acknowledgements: We thank the writing group lead authors: A/Prof Asha
support patient care and education about ARF and RHD through Bowen, Dr Judith Katzenellenbogen, Dr Rosemary Wyber, Dr James Marangou,
Dr Kathryn Roberts, Dr Geraldine Vaughan and Professor Andrew Steer. We also
workforce education and training; promote primary preven- thank all additional co-­authors and reviewers who made major contributions to
tion aimed at preventing initial episodes of ARF; and provide the development the guideline (Supporting Information, Table 1), in particular,
the many Aboriginal and Torres Strait Islander people who imparted their cultural
jurisdiction-­wide data for epidemiological reporting (https:// knowledge. We thank Professor Jonathan Carapetis, who led the first two editions
www.rhdau​stral​ia.org.au/contr​ol-programs). of the guidelines as the former Director of RHDAustralia, and Professor Alex Brown,
current co-­director of RHDAustralia. Many thanks also to Bec Slade for overall
program management. RHDAustralia is funded under the Australian Government’s
New technologies Rheumatic Fever Strategy to provide guidelines, resources and education for health
care professionals and for patients, families and communities affected by ARF and
Research underway in Australasia aims to discover alternatives RHD. We thank the National Heart Foundation of Australia which contributed funding
to or improvement in delivery of benzathine benzylpenicillin G, to guideline development. Anna Ralph is supported by a National Health and Medical
Research Council fellowship (1142011).
develop a group A streptococcus vaccine, and develop a diag-
nostic test for ARF. Competing interests: Sara Noonan and Vicki Wade are employed by RHDAustralia,
funded by the Australian Government Department of Health and the National
Health Foundation of Australia. Anna Ralph is the Co-­d irector of RHDAustralia,
Conclusion supported by an NHMRC fellowship. Bart Currie is the immediate past Director of
RHDAustralia.
The 2020 ARF and RHD guideline places person and culture at Provenance: Not commissioned; externally peer reviewed. ■
the centre of care and synthesises the current evidence to provide
expert clinical guidance from prevention through to tertiary care. © 2020 AMPCo Pty Ltd

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Supporting Information
Additional Supporting Information is included with the online version of this article.
MJA 2020