National Guide

Chapter 12 | Acute rheumatic fever and rheumatic heart disease







National Guide to preventive healthcare for Aboriginal and <br/>Torres Strait Islander people
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    13. Chapter 12 | Acute rheumatic fever and rheumatic heart disease

Acute rheumatic fever and rheumatic heart disease

Dr James Doran, Ms Vicky Wade   
Key messages
  • Acute rheumatic fever (ARF) and its chronic sequela rheumatic heart disease (RHD) are preventable.1
  • Primordial prevention strategies that focus on the social, cultural and environmental determinants of health are critical in reducing rates of Streptococcus A (Strep A)-associated diseases, including ARF and RHD. Improving living conditions is associated with decreasing ARF and RHD in most high-income countries, including Australia.
  • Aboriginal and Torres Strait Islander people face many barriers to accessing healthcare. Providing culturally safe care can increase access to healthcare and help reduce the burden of ARF and RHD.
  • The co-design of services and systems across sectors that support the physical wellbeing and social, emotional and mental health of Aboriginal and Torres Strait Islander children and youth requires integration within primary care ARF/RHD models of care.
  • Early treatment for sore throat, and the prevention and treatment of skin infections, can prevent primary and recurrent ARF.1
  • ARF can be a challenging diagnosis involving various symptoms and signs that do not necessarily co-occur.1 Failure to diagnose can result in missing the opportunity for secondary prophylaxis, thereby increasing the risk of recurrent ARF and long-term heart valve damage (RHD). The revised Jones criteria include features specific to high-risk communities.2–4
  • Echocardiography is required for the diagnosis and monitoring of RHD. Cardiac auscultation is neither specific nor sensitive enough to be used as a screening test for RHD.1
  • Many people living with ARF and RHD have multiple medical comorbidities. It is essential to design models of care in partnership with Aboriginal and Torres Strait Islander communities that recognise RHD does not occur in isolation and integrate care into the longitudinal, comprehensive management of other comorbidities.5
  • Self-determination and shared decision making are vital to improve effective design and delivery of ARF/RHD programs.
  • Respectful partnerships between Aboriginal and Torres Strait Islander Community Controlled Health Service Organisations (ACCHSOs)/primary healthcare services and tertiary care institutions support adequate and timely targeted treatment for those who already have ARF or RHD.
  • Accurate and timely data access remains problematic for clinicians: multiple electronic medical record systems in place do not allow streamlined access of information for different services.
  • Public health initiatives and priorities as identified by Aboriginal and Torres Strait Islander leadership that are incorporated into comprehensive primary healthcare and delivered in locally adapted and appropriate ways are key to preventing ARF and RHD.
Type of preventive activity - Screening
Who/target population What When Strength of recommendation Key source(s) and reference(s) Rationale/key considerations informing recommendation
People from high-risk groups or living in high-risk settings for ARF/RHD Undertake cardiac auscultation as part of a general check-up, but do not rely on cardiac auscultation to screen for RHD due to poor sensitivity and spec    ificity Opportunistically Strong National guideline1 Cardiac auscultation is recommended as part of a general check-up and will identify some people with RHD and murmurs from other causes, but is not sensitive or specific enough to effectively screen for RHD

Echocardiography is required for effective screening, but there is limited access in primary care settings
People from high-risk groups or living in high-risk settings for ARF/RHD (targeted screening) Use echocardiography to screen for RHD Opportunistically Conditional National guideline1 Echocardiography can accurately detect previously undiagnosed RHD
All people with a past history of ARF, or cardiac murmurs suggestive of valve disease Refer for echocardiography and subsequent follow-up*
Refer to management guidelines for specific advice		 As per management guidelines Strong National guideline1 Ideally, echocardiography is available in the primary care setting but otherwise (usually) referral to specialist sonography/cardiology services is required

Undiagnosed or late-diagnosed RHD results in high morbidity and mortality
All women as part of prepregnancy planning and all pregnant women coming from high-risk groups or living in high-risk settings for ARF/RHD Targeted screening using echocardiography* Opportunistically and as part of routine preconception and pregnancy care Strong National guideline1 Preconception diagnosis of RHD allows optimal management, including surgery, if needed, before pregnancy

There is high risk of adverse maternal and fetal outcomes if RHD is not managed during pregnancy and labour

Women with some valve lesions (moderate or greater mitral stenosis, severe mitral or aortic regurgitation, severe aortic stenosis) have a high risk of cardiac events during pregnancy and of adverse fetal outcomes. They require specialist care and close monitoring
Type of preventive activity - Behavioural
Who/target population What When Strength of recommendation Key source(s) and reference(s) Rationale/key considerations informing recommendation
People in high-risk groups or living in high-risk settings for ARF/RHD or with a history of ARF or known RHD Emphasise the importance of early treatment for sore throat, and the prevention and treatment of skin infections

Advise about healthy living and health risks (smoking, diet, exercise, dental health) and the need for regular clinical reviews (refer to Chapter 2: Healthy living and health risks)		 Opportunistically Strong National guideline1 Identifying and managing Strep A infections and their spread is an important opportunity to prevent ARF in individuals and communities

Lifestyle management helps reduce communications of RHD
Women in high-risk groups or living in high-risk settings for ARF/RHD or with a past history of ARF or known RHD Promote effective contraception for all girls and women, especially if pregnancy poses a health risk

Avoid oestrogen-containing contraceptives		 As clinically indicated Strong National guideline1 Women with RHD can have safe pregnancies and deliveries if managed well
Type of preventive activity - Medication
Who/target population What When Strength of recommendation Key source(s) and reference(s) Rationale/key considerations informing recommendation
People in high-risk groups for ARF and RHD with throat and skin infections Treat promptly as per guidelines and determine a family’s preferred method of treatment As clinically indicated on presentation Strong National guideline1 Prompt and targeted treatment of Strep A infections prevents the autoimmune response that is ARF
People with a diagnosis of ARF or RHD Notify to the local RHD register for coordination of treatment
Treat promptly as per guidelines and determine a family’s preferred method of treatment		 Regimen according to national guidelines with support for self-management at every health assessment Strong National guideline1 Secondary prevention of recurrent ARF prevents or delays the progression of RHD
People with a diagnosis of RHD Symptom management of cardiac disease and extended care according to disease priority classification As clinically indicated, as per individual care plan Strong National guideline1 Symptom-based monitoring and care help prevent and manage the complications of RHD
People with a diagnosis of RHD Bacterial endocarditis prevention prior to identified procedures As clinically indicated Strong National guideline1 Invasive procedures are associated with a high risk of endocarditis
 



*There are many challenges to adhering to current guidelines in an under-resourced rural and remote primary care setting.7,8 With the decreasing cost of portable cardiac ultrasound, primary care providers have an emerging role screening and monitoring for RHD progression and its diagnosis. Collaborative relationships with specialist cardiac services that upskill and support primary care providers to meet the care needs of their communities should be sought where appropriate in high-risk areas.

  • Where ARF and RHD are notifiable conditions, clinicians are required to notify the relevant public health authority as soon as possible after confirming the diagnosis (including possible and probable ARF and borderline RHD).
  • Individuals and families should be involved in all decisions regarding the prevention and management of recurrent ARF and RHD.
  • Minimising the pain and distress of intramuscular injections is a key factor in successful treatment. Benzathine penicillin G (BPG) injections should be administered by trained and experienced staff according to the patient’s preference and choice for injection site and method of pain management.1
  • Most people with ARF and RHD are preteens, adolescents and young adults. Attention to continuity of care and avoiding implementation of child-only or adult-only RHD programs within primary care services are critical components of a successful prevention program.9–11
  • Health services should adopt a flexible approach to where injections are administered (eg clinic, home, clinic vehicle, school, workplace)9 and should arrange ‘fast tracking’ for young people who are attending the clinic for their injections to reduce wait times.
  • Communities with the highest incidence of ARF also experience a high turnover of medical and nursing staff. Thus, resident primary healthcare staff, particularly Aboriginal and Torres Strait Islander health workers, must be appropriately trained and supported to maintain their skills.
  • In clinical practice, true penicillin allergy is rare.12 Given a lack of adequate prophylaxis can have disastrous sequelae, it is important to verify the type and severity of the allergic reaction, because many reactions are mild (eg nausea or local injection irritation). Referral to an allergist or immunologist is recommended where possible to determine whether there is an absolute contraindication to penicillin.
  • Conduct clinical audits to support continuous quality improvement (eg recording of ARF and/or RHD in health records, treatment of skin infections, treatment of throat infections, timeliness and acceptability of secondary prevention/antibiotic administration, referral to environmental services).

Clinical guidelines

ARF and RHD registries

Other resources for health professionals

Professional development

Environmental health

Health promotion materials

Background

The aim of this topic is to provide practical guidance for primary care in the prevention of ARF and RHD. The recommendations aim to move beyond disease-specific strategies, which reflect biomedical concepts of health as the absence of illness and disease, and place greater emphasis on comprehensive and culturally appropriate primary healthcare as the standard of care.

ARF is almost entirely preventable, with opportunities for primordial, primary, secondary and tertiary prevention at several stages along the ARF–RHD disease pathway (see Box 1 ). ARF is caused by an abnormal and exaggerated immune response to infection with Group A β-haemolytic streptococcus (Strep A, Group A Streptococcus [GAS] or Streptococcus pyogenes). Among high-risk groups, Strep A infections commonly occur in the throat (37% of throat infections; strep throat, tonsillitis) and the skin (82% of impetigo, pyoderma) with close to half of Aboriginal and Torres Strait Islander children in some communities having skin infections at any one time.1,13 

Box 1. Overview of the prevention of ARF–RHD

Over 10,000 people are currently living with ARF and/or RHD in Australia, most of whom are Aboriginal and Torres Strait Islander people.14 Based on current trends, the prevalence of RHD is predicted to increase by 2031, with a large number expected to require valve surgery and a significant loss of life.15 A major shift in the approach to ARF and RHD in Australia is required. It is important to acknowledge that although primary care services have the potential to play a valuable role in improving outcomes for those at risk and living with ARF and/or RHD, major progress will be limited until the underlying fundamental social, cultural and environmental determinant drivers of inequity are addressed.16 Primary care providers, in particular Aboriginal Community Controlled Health Organisations (ACCHOs), are ideally positioned to identify how to address the drivers of inequity entrenched in high-risk communities where ARF and RHD flourish.

Aboriginal and Torres Strait Islander peoples’ conceptualisation of health and wellbeing can be defined as the cultural, social, psychological and physical wellbeing of an individual and their kinship network, reflecting the interconnectedness of the spiritual and physical aspects of an individual’s health to culture, kinship and Country.17,18 This understanding of health and wellbeing is integral to each prevention activity along the disease pathway. However, there are no studies reporting the impact of management of social and emotional wellbeing factors for RHD patients.19 Although much is known in communities about what works, published evidence related to models of care that show benefit and are accepted within the Aboriginal and Torres Strait Islander healthcare setting is limited.

Mainstream care models and their associated programs often struggle to meet the needs and realities of Aboriginal and Torres Strait Islander communities. Current RHD Australia guidelines acknowledge the impact of the social determinants of health and wellbeing on the incidence and course of ARF/RHD;1 however, many of the RHD programs delivered to communities historically have had a narrow biomedical focus, emphasising disease monitoring and secondary prophylaxis.20 These clinically orientated programs have had limited impact on the incidence of ARF and prevalence of RHD across northern and Central Australia in the past two decades.21,22

It is important to note that the new National Rheumatic Fever Strategy commits to the National Agreement on Closing the Gap, particularly in relation to Priority Reform One: Formal Partnerships, with shared decision making addressing local priorities with local solutions on Country.23 Where local ACCHOs are not operating, collaboration with local communities, with leadership and expertise of Aboriginal and Torres Strait Islander health workers and health practitioners to drive local action, is vitally important. Collaboratively developed community-based programs increase engagement in primary healthcare and improve health outcomes.1,24

The following principles from the social and emotional wellbeing model for the delivery of health and wellbeing support to Aboriginal and Torres Strait Islander people have been outlined in the National Strategic Framework:25 

  • emphasis on a holistic view of health
  • importance of self-determination
  • culturally valid understandings shaping service provision
  • the impacts of intergenerational trauma
  • a human rights-based approach
  • ongoing effects of social and economic disadvantage and of racism
  • centrality of Aboriginal and Torres Strait Islander family and kinship.

Box 1 provides an overview of the levels of prevention of ARF and RHD. Most prevention strategies take place in communities and rely on a culturally safe and competent workforce with integrated approaches across sectors. Providing culturally safe and responsive care while strengthening Aboriginal and Torres Strait Islander-led models of health are major determinants of quality care and improved outcomes.26,27 As such, services operating in a community must be endorsed by the community. Given the diversity of people and settings, there is no single model of care that will lead to a long-term solution for all. However, successful strategies most likely are those built and owned by affected Aboriginal and Torres Strait Islander communities. Understanding Aboriginal and Torres Strait Islander concepts of comprehensive primary healthcare with community initiation and leadership of such concepts, as discussed, is critical.23,24

In addition, prevention activities can be strengthened by partnerships between health services, schools, workplaces and other Aboriginal and Torres Strait Islander organisations in the community. Some prevention activities can be initiated in schools. Continuous quality improvement and a skilled workforce are also very important. The need for a comprehensive community-led primary healthcare approach that is adequately coordinated and resourced is well recognised; however, actual models and costings are yet to be defined.23,28,29

Primordial prevention: Preventing exposure to Strep A

Repeated exposure early in life to Strep A infection leads to immune priming, where the immune system recognises the pathogen/infective agent, which increases the risk of developing ARF.1 Primordial prevention aims to address the prevention of risk factors for Strep A infection, focusing on the social determinants of health, which are the conditions in which people are born, grow, work, live and age. Addressing these determinants is likely to be the most effective strategy to contribute to a sustained reduction in community-acquired Strep A infections.1Strategies that link primary care services and community-led initiatives with the ability to refer for services to address the social determinants of health have shown promise, with improved reporting of household maintenance issues and awareness of prevention opportunities for Strep A infections.30 Addressing environmental factors alone leads to a reduction in primary healthcare demand by 20%, whereas 25% of presentations for Aboriginal and Torres Strait Islander children could be prevented.6,23,31 Primary healthcare services have a role in asking about environmental and living conditions, including health promotion as part of primary healthcare, followed by referral to services to improve conditions (eg environmental health workers), where available, as well as patient and community advocacy for the right to access risk-free living conditions.

It is important to use a strengths-based approach to health promotion and prevention strategies. These approaches focus on the positive attributes of individuals and communities, address power imbalances, and consider the environment and resources at hand in communities.32 Health promotion about healthy living practices is futile if the means to effect change, such as access to functional household infrastructure and cleaning agents, are not accessible.

Human trials of a vaccine for Strep A infection have started.33–35 However, a widely available vaccine is not likely to be available in the foreseeable future.

Community-led primordial prevention activities that could be considered by primary healthcare services are outlined in Box 2.

Box 2. Key primordial prevention activities that can be implemented and/or promoted by the primary care team

Healthcare service features

  • Improve access to culturally safe primary health care36
  • Integrate environmental health into clinical care (referral pathways for environmental health assessment and services for homes, schools and places of social gathering)37
  • Ensure coordinated approach to service delivery
  • Prioritise a strengths-based approach to health promotion32
  • Develop service priorities as determined by communities that address the inequitable access to resources and care
  • Embed staff education into clinical orientation programs, including systems for regular clinical updates
  • Support the rheumatic heart disease clinical team members with strategies, such as team building exercises and practical skills to achieve goals and overcome challenges to the delivery of quality patient care38
  • Conduct clinical audits to understand the prevalence of acute rheumatic fever and rheumatic heart disease and health service response/provision of care

Education

  • Develop programs to improve health literacy interventions and identify barriers to maximising the benefits of health promotion strategies39
  • Develop culturally appropriate learning tailored to a person's specific learning needs, including for those with learning disability
  • Provide appropriate and respectful education addressing hand hygiene and personal, family, community, care provider and clinical staff hygiene measures
  • Promote protective behaviours around nutrition and exercise, such as avoiding a high intake of sugar-sweetened beverages, the addition of iron rich foods and maintaining physical activity19,36,40
  • Promote health-seeking behaviours
  • Support school attendance (to Year 12)41

Referral and/or advocacy (for individuals and communities)

  • Refer and/or advocate for
    • adequate housing
    • health hygiene infrastructure, such as reliable power, water and washing facilities
    • regular and freely available access to washing machines with hot water
    • clean linen and aired mattresses
  • Promote the social and emotional well-being of Aboriginal and Torres Strait Islander children and youth42
  • Advocate for policy that supports the alleviation of poverty and socioeconomic marginalisation43
  • Support youth-guided community change

Evaluation and reporting

  • Continuous reporting of health outcomes that are affected by a community’s socioeconomic status and health economic assessments
  • Evaluation and reporting of primordial prevention strategies to support the implementation, translation and upscaling of successful projects to the wider community

Primary prevention: Preventing Strep A infection

Primary care teams have a key role in primary prevention. Primary prevention aims to interrupt the link between Strep A infection and the subsequent abnormal immune response that causes ARF by the early identification and treatment of Strep A infections. An intramuscular injection of BPG is the first-line treatment of Strep A infection in people at high risk of ARF and contributes to an 80% reduction in the risk of ARF for patients presenting with sore throat and symptoms suggestive of Strep A infection.44 A person’s choice of injection and how it will be administered is very important. Options should be discussed with the patient.

The incidence of ARF peaks in the 5- to 15-year age group. Symptoms and signs of ARF develop within two to three weeks after the initial sore throat.45 ARF tends to run in families; however, only 60% of patients who experience an episode of ARF develop RHD. This is due primarily to shared living conditions, environmental factors and the communicable nature of Strep A infections.46–48 Individuals who have had ARF are at high risk of recurrences, especially in the first year; however, this increased risk extends over the next 10 years.1,49

The diagnosis of ARF is a clinical decision based on the revised Jones criteria1,50 and requires interpretation of relevant symptoms and signs, plus evidence of a recent Strep A infection. Additional diagnoses of probable and possible ARF are defined when manifestations do not satisfy the diagnostic criteria for definite ARF but the clinician feels ARF is the most likely diagnosis. The varied presentations add to the diagnostic challenge. Other features of ARF include that individuals with ARF and more common ARF recurrences can experience asymptomatic acute carditis (which may go undetected) and, although rare, erythema marginatum, subcutaneous nodules and the neurological disorder Sydenham chorea, which are highly specific for ARF.1

Community-led primary prevention activities that could be considered by primary healthcare services are outlined in Box 3.

Box 3. Primary prevention of Strep A infection in people who have not been diagnosed with ARF/RHD
  • Early engagement of those at risk of ARF in preventing the disease30
  • Embedding preventive care within primary care services23
  • Flexible access options to assessment and treatment (eg practical and physical support to help people access health services and after-hours care30)
  • Home visits, which can reduce barriers to access and generate an understanding of the home environment while allowing access to siblings and carers for treatment and health promotion
  • Outreach services to provide throat and skin messaging and monitoring in schools and homes, including remote outstations, with a potential role of telehealth in supporting outreach workers to communicate with clinic-based staff30
  • Promoting language and culturally relevant, stigma-free health information about skin sores: addressing attitudes to skin sores, including reducing stigma and denormalising skin sores
  • Treatment of skin sores, scabies, head lice and associated secondary infections
  • Supporting people at risk of and with ARF/RHD to engage in their own healthcare and self-management30
  • Culturally appropriate, and in appropriate language, education and counselling to individuals and families newly diagnosed with ARF/RHD
  • Adequate training of healthcare practitioners in administering BPG injections and managing pain and distress using patient-preferred treatment strategies1
  • Engaging those with lived experience to support those who are newly diagnosed, increase community awareness and provide education to the healthcare team on the lived experience
  • Improving cultural safety among healthcare practitioners and encouraging them to actively engage with traditional knowledge and practices unique to each community, making healthcare delivery more acceptable30,51
  • Referrals to environmental health workers/services to improve environmental and living conditions when people present with ARF and or recurrent skin sores or scabies

Secondary prevention: Preventing Strep A recurrence in people with previous ARF or RHD

Secondary prevention aims to reduce recurrent ARF in people with a previous diagnosis of ARF or RHD.1 BPG intramuscular injection every 21–28 days is the antibiotic of choice to prevent episodes of recurrence. Patients need to receive at least 80% of scheduled doses to obtain the maximum benefit from being on treatment. For every 10% increase in adherence above 40%, the odds of an ARF recurrence reduce by 17%.52–54 Oral penicillin is less effective than intramuscular BPG and should only be considered for people who do not tolerate the injection after all attempts to address barriers have been explored. Erythromycin is recommended for people with definite penicillin allergy (see Useful resources, RHD guidelines).1

Intramuscular BPG injections are painful, and restraint should never be used when administering secondary prophylaxis in children. Both non-pharmacological and pharmacological strategies used alone or in combination can be integrated into each individual care plan (see Box 4). Lignocaine (also known as lidocaine) in combination with BPG injections significantly reduces pain during and in the first 24 hours after injection. There is an even greater reduction in pain when lignocaine is used in combination with Buzzy®. Patients should have a written plan on how they want their injection to be given.

Aboriginal health practitioners/workers are well placed to manage BPG recalls and administration given they are more likely to be based in communities, with closer links to people and, hopefully, longer tenure. Regular train-the-trainer courses for Aboriginal health practitioners/workers to train others in administering BPG injections in a culturally safe way and regular retraining to account for high staff turnover are needed.

All patients receiving secondary prophylaxis should have a comprehensive clinical assessment and an echocardiogram prior to stopping regular antibiotic prophylaxis.

Community-led secondary prevention activities that could be considered by primary healthcare are outlined in Box 4.

Box 4. Strategies to prevent the recurrence of Strep A infections once a diagnosis of ARF/RHD has been made
  • Strategies aimed at improving the delivery of injections, including shared decision making and injection administration technique
  • Culturally appropriate and accessible patient, family and community education about ARF and RHD
  • Support for patients and families to engage in self-management or community group management of treatment regimens and promote social and emotional wellbeing
  • Coordination of, and collaboration between, available health services, schools and places of employment as acceptable alternative locations for the administration of secondary prophylaxis
  • Culturally safe, structured and sustained routine care and follow-up
  • Integrated recall systems
  • Engagement with community members with a lived experience
  • Referrals to environmental health works/services to improve environmental and living conditions

Tertiary prevention: Living with RHD

Tertiary prevention includes surgical care as well as a whole-of-life approach to include many critical elements of long-term care, such as heart failure, cardiac arrhythmias, long-term anticoagulation, cardiac rehabilitation and supporting social and emotional wellbeing.1 Community-based cardiac rehabilitation programs such as the Heart Guide Aotearoa program, with its multidisciplinary approach to support self-management, have been shown to improve psychological and social functioning and adherence to recommended life-style changes and medications.55

Pregnancy planning is another critical element of long-term care for young high-risk women.1 Models of care that focus on the health and wellbeing of Aboriginal and Torres Strait Islander women, the Healthy Mums, Healthy Bubs program for example, have shown promise.29,56 It is essential that new models of RHD care that have shown benefit can be integrated into culturally safe primary care models.

Mitral valve repair is generally superior to valve replacement in the young, with no requirement for anticoagulation and the associated teratogenic effects in later pregnancy. However, not all valves are repairable; hence, the requirement for bioprosthetic valve replacement to avoid the teratogenic effects of warfarin treatment (which is a requirement of mechanical valve replacement).57 (For further details, see Useful resources and RHD guidelines.1)

Prepregnancy counselling is well described in the Australian RHD guidelines.1 A focus on timing of surgery to allow women to make informed decisions related to pregnancy planning is required.5 Primordial, primary and secondary prevention with ongoing secondary prophylaxis continue to improve survival outcomes and time to redo surgery of those who have acquired a valve replacement or repair.58

Community-led tertiary prevention activities that could be considered by primary healthcare services are outlined in Box 5.

Box 5. Key tertiary preventive activities that can be implemented and/or promoted by the primary care team
  • Oral health
  • Pregnancy planning and women’s health
  • Practice recall systems to help coordinate benzathine penicillin G injections, warfarin monitoring, medical review, dental care and echocardiograms
  • Cardiac rehabilitation and monitoring programs
  • Support groups and other social and emotional wellbeing programs
  • Allied health clinics to address comorbidity risk
  • Integration of culturally safe health, social and emotional wellbeing services59
  • Culturally and gender-appropriate care
  • Alcohol and other drugs support
  • Specific support for people who live with rheumatic heart disease who require warfarin therapy
  • Protective behaviours around nutrition and physical activity
  • Placement of cardiac rehabilitation within primary care60

Other preventive strategies

Expanding scope of practice of clinicians

Despite the well-documented barriers to delivering holistic and culturally safe healthcare to Aboriginal and Torres Strait islander people with complex comorbidity, including RHD, there are models of care that show promise.8,61–63There is growing interest in the role of medical practitioners as rural generalists who have a broad range of primary care skills and can provide aspects of secondary and tertiary care in various settings, including the home.64,65Integration of Aboriginal health practitioners and workers at all levels of prevention and care, including secondary prophylaxis and culturally responsive care across the entire system, is also essential.1,63,66

Use of echocardiography for screening and monitoring of disease

Cardiac auscultation is not specific or sensitive enough to be used for screening for RHD. Echocardiography is required for effective screening.1,67,68 Traditionally, echocardiography has been dependent on specialist sonography and cardiology services, but echocardiography provided in high-prevalence primary care settings has been shown to be cost-effective.68More investigation into the feasibility and logistics of broader implementation, such as workforce composition and training, is needed.1 While research continues in this area, it is imperative that each community screened has the resources available to follow-up, monitor outcomes and optimally manage each new diagnosis of RHD. It is important that education is provided as part of the screening and that culturally appropriate resources are available for communities.

Significant changes from third edition recommendations outlined in the 2020 Australian ARF and RHD guidelines are presented in Box 6.

Box 6. Significant changes from recommendations outlined in the third edition (2020) Australia ARF and RHD guidelines
  • New recommendations for management of Strep A skin infections to prevent ARF
  • The criteria for ARF diagnosis in low-risk groups have been updated
  • Doses for benzathine penicillin G (BPG) are provided in units (not milligram) in response to Therapeutic Goods Administration requirements for labelling
  • Guidance for the ventrogluteal injection site is provided
  • Secondary prophylaxis recommendations for some classifications of ARF and borderline RHD have been updated in line with new evidence
  • Strategies for managing pain and distress associated with intramuscular BPG injections have been expanded in the Australian guidelines1 to provide a hierarchy of approaches
  • The priority definitions in the ‘priority classification system’ for the presence and severity of RHD have changed to align with appropriate timing of follow-up
  • Antibiotic prophylaxis to prevent infective endocarditis following dental procedures now comprises amoxicillin instead of clindamycin, even for people on regular penicillin-based treatment (eg regular BPG)69
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  3. Bordes SJ, Murray IVJ, Sylvester JA. Atypical presentation of acute rheumatic fever (ARF) in a 25-year-old woman in the Caribbean: A challenging diagnosis. BMJ Case Rep 2020;13(2):e231888. doi: 10.1136/bcr-2019-231888.
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