Primordial prevention

Improvements in socioeconomic and environmental conditions are known to reduce acute rheumatic fever (ARF) and rheumatic heart disease (RHD) in communities with high rates of disease.1^,2^,3 Specifically, overcrowded housing and lack of access to washing facilities are strongly associated with higher rates of group A streptococcal (Strep A) infections and ARF.4

The risk of Strep A infections, ARF and RHD for people living in crowded housing is up to 1.7-2.8 times higher than for people in uncrowded housing.5 Washing hands and bodies is directly associated with reducing Strep A throat and skin infections, and other conditions including diarrhoeal diseases, food-borne infections, and respiratory infections.6^,7  Adequate washing facilities include clean water, taps, sinks, soap and towels, and the capacity to safely remove waste water.

Strategies to improve housing, washing and other social determinants of health are likely to reduce the transmission of Strep A and Strep A infections, and require a comprehensive community environmental health approach.8^,9

Primary prevention

Primary prevention of ARF requires antibiotics to treat a Strep A infection before ARF develops. Infections associated with ARF include strep throat and streptococcal skin infections (skin sores, pyoderma, impetigo).

Some groups in Australia are at high risk of ARF following Strep A infections.10

• Aboriginal and Torres Strait Islander peoples, particularly those living in rural or remote settings across central and northern Australia, are known to be at very high risk.
• Aboriginal and Torres Strait Islander peoples, and Māori and Pacific Islander groups living in urban settings, particularly where there is household crowding, are also at high risk.
• People with a history of ARF or rheumatic heart disease are at high risk of developing recurrent ARF.

Antibiotic treatment for strep throat is either one intramuscular benzathine benzylpenicillin injection, or antibiotic tablets/syrup for 5 or 10 days. Antibiotic treatment for Strep A skin infection is either antibiotic tablets/syrup for 3 days or one intramuscular benzathine benzylpenicillin injection.11

Antibiotics for strep throat in people at high risk of ARF can reduce development of ARF by up to two-thirds.12 However, Strep A is present in only between 10% and 40% of children presenting with a sore throat.13

Identifying the cause of the throat or skin infection is important to make sure the correct treatment is given, but waiting for test results for people (particularly children) at high risk for ARF may result in ARF developing before treatment is started. It is therefore recommended in Australia that all children in high risk groups receive primary prevention with antibiotic therapy for sore throats and skin sores immediately after a swab has been taken. People who are not at high risk of ARF should not have primary prevention treatment started until a Strep A infection has been confirmed.14

Secondary prevention (ARF prophylaxis)

Regular secondary (antibiotic) prophylaxis is recommended for people diagnosed with ARF and RHD.15^,16^,17^,18 Antibiotics are most effectively delivered as benzathine benzylpenicillin (BPG) injections at least every 28 days (13 injections every 12 months). Each injection needs to be given no later than day 28 after the last injection, because without the constant protection of the penicillin, there is a high risk of another Strep A infection and recurrent ARF.19

Alternatives to the injections are available, although they may be less effective in preventing ARF and require careful monitoring:20^,21

• Penicillin tablets - one tablet every morning and every night – may be prescribed for people who are not able to receive injections, such as people who experience significant bleeding with injections.
• Erythromycin tablets - one tablet every morning and every night – are prescribed for people who have a confirmed penicillin allergy. (True penicillin allergy is rare, so suspected allergy needs to be carefully investigated)

The length of time required for secondary prophylaxis treatment depends on several factors including age, diagnosis (of ARF or RHD), severity of RHD if it is present, and potential harm to the heart from future ARF. Most people require secondary prophylaxis for at least 10 years.22 BPG injections and tablets are safe during pregnancy and breastfeeding, and should continue as prescribed.23 An echocardiogram and review by a medical specialist are required before secondary prophylaxis can be ceased.24

Providing and receiving regular treatment over many years can be difficult. Injections may be painful or inconvenient,25 and tablets may not be taken as prescribed.

Health services can support people receiving secondary prophylaxis and their families by:

• Prioritising secondary prophylaxis service delivery
• Providing skilled injection technique and culturally safe care
• Giving people the opportunity to receive injections in their preferred site and with their preferred method of pain relief (if required)
• Employing recall and reminder systems for injections due
• Providing outreach injection services where possible
• Placing the expertise, experience, community knowledge and language skills of Aboriginal Health Workers and Aboriginal Health Practitioners at the centre of service
• Promoting the importance of secondary prophylaxis through culturally safe education
• Identifying barriers to receiving secondary prophylaxis, and working within the system and with patients and families to address and overcome the barriers.

Tertiary Prevention

Tertiary prevention aims to slow the progression of RHD by preventing and managing complications to maintain quality of life and prevent premature death. Management is based on the type and severity of disease, and is tailored for each person.26

People with RHD need timely access to medical specialists including cardiologists, pathology services for blood tests, echocardiography, dentists, obstetricians, and heart surgeons.

Specific management includes:27 

• Regular secondary prophylaxis to prevent recurrent acute rheumatic fever
• Regular medical review to monitor the disease
• Mediations to manage heart failure
• Antibiotics prior to some dental and surgical procedures to prevent endocarditis (an infection of the heart)9,13  
• Contraception and pregnancy planning28^,29 
• Carefully planned heart valve surgery (the right time and the right procedure)
• Close monitoring after surgery
• Disease education and self-management support
• Balanced management of other illnesses or diseases

• 1. Watkins DA, Johnson CO, Colquhoun SM, et al. Global, Regional, and National Burden of Rheumatic Heart Disease, 1990–2015. The New England Journal of Medicine. 2017;377:713-722. View Source
• 2. Brown A, McDonald MI, Calma T. Rheumatic fever and social justice. The Medical Journal of Australia. 2007;186(11):557-558. View Source
• 3. Aboriginal Environmental Health Unit - Population Health Division. Closing the gap: 10 Years of Housing for Health in NSW. An evaluation of a healthy housing intervention. North Sydney: NSW Department of Health, 2010.
• 4. Coffey PM, Ralph AP, Krause VL. The role of social determinants of health in the risk and prevention of group A streptococcal infection, acute rheumatic fever and rheumatic heart disease: A systematic review. PLOS Neglected Tropical Diseases. 2018;12(6):e0006577. View Source
• 5. Coffey PM, Ralph AP, Krause VL. The role of social determinants of health in the risk and prevention of group A streptococcal infection, acute rheumatic fever and rheumatic heart disease: A systematic review. PLOS Neglected Tropical Diseases. 2018;12(6):e0006577.
• 6. Curtis V, Camicross S. Effect of washing hands with soap on diarrhoea risk in the community: A systematic review. Lancet Infectious Diseases. 2003;3(5):275-281. View Source
• 7. Rabie T, Curtis V. Handwashing and risk of respiratory infections: a quantitative systematic review. Tropical Medicine and International Health. 2006;11(3):258-267. View Source
• 8. HealthHabitat, Housing for Health. View Source
• 9. Hardy B. A report on simple, low cost, effective house survey and repair programs in the Northern Territory, Australia. Journal of Rural and Remote Environmental Health. 2002;1(1):19-22. View Source
• 10. RHDAustralia (ARF/RHD writing group). The 2020 Australian guideline for prevention, diagnosis and management of acute rheumatic fever and rheumatic heart disease (3rd edition). 2020. View Source
• 11. RHDAustralia (ARF/RHD writing group). The 2020 Australian guideline for prevention, diagnosis and management of acute rheumatic fever and rheumatic heart disease (3rd edition). 2020.
• 12. Spinks A, Glasziou PP, Del Mar CB. Antibiotics for sore throat. Cochrane Database of Systematic Reviews. 2013;11:CD000023: View Source
• 13. Oliver J, Malliya Wadu E, Pierse N, et al. Group A Streptococcus pharyngitis and pharyngeal carriage: A meta-analysis. PLOS Neglected Tropical Diseases. 2018;12(3):e0006335 View Source
• 14. RHDAustralia (ARF/RHD writing group). The 2020 Australian guideline for prevention, diagnosis and management of acute rheumatic fever and rheumatic heart disease (3rd edition). 2020.
• 15. RHDAustralia (ARF/RHD writing group). The 2020 Australian guideline for prevention, diagnosis and management of acute rheumatic fever and rheumatic heart disease (3rd edition). 2020.
• 16. World Health Organization. Rheumatic fever and rheumatic heart disease: report of a WHO expert consultation, Geneva, 29 October–1 November 2001. WHO Technical Report Series 923 2004. View Source
• 17. Antibiotic Expert Group, Therapeutic guidelines: antibiotic. Vol. 15. 2014, Melbourne: Therapeutic Guidelines Limited.
• 18. Heart Foundation of New Zealand. New Zealand Guidelines for Rheumatic Fever: Diagnosis, Management and Secondary Prevention of Acute Rheumatic Fever and Rheumatic Heart Disease: 2014 Update. View Source
• 19. de Dassel JL, Malik H, Ralph AP, Hardie K, Reményi B, Francis JR. Four-weekly benzathine penicillin G provides inadequate protection against acute rheumatic fever for some children (in Australia’s Northern Territory). American Journal of Tropical Medicine and Hygiene. 2019;100(5):1118-1120. View Source
• 20. Feinstein A, Wood HF, Epstein JA, et al. A controlled study of three methods of prophylaxis against streptococcal infection in a population of rheumatic children. II. Results of the first three years of the study, including methods for evaluating the maintenance of oral prophylaxis. New England Journal of Medicine. 1959;260(14):697-702. View Source
• 21. Wood H, Feinstein AR, Taranta A, et al. Rheumatic fever in children and adolescents. A long term epidemiological study of subsequent prophylaxis, streptococcal infections and clinical sequelae. III. Comparative effectiveness of three prophylaxis regimes in preventing streptococcal infections and rheumatic recurrences. Annals of Internal Medicine. 1964;60(S5):31-46. View Source
• 22. RHDAustralia (ARF/RHD writing group). The 2020 Australian guideline for prevention, diagnosis and management of acute rheumatic fever and rheumatic heart disease (3rd edition). 2020.
• 23. Department of Health Therapeutic Goods Administration. Medicines and TGA classifications. 2019. View Source
• 24. RHDAustralia (ARF/RHD writing group). The 2020 Australian guideline for prevention, diagnosis and management of acute rheumatic fever and rheumatic heart disease (3rd edition). 2020.
• 25. Mitchell AG, Belton S, Johnston V, et al. Aboriginal children and penicillin injections for rheumatic fever: how much of a problem is injection pain? Australian and New Zealand Journal of Public Health. 2018;42:46-51. View Source
• 26. RHDAustralia (ARF/RHD writing group). The 2020 Australian guideline for prevention, diagnosis and management of acute rheumatic fever and rheumatic heart disease (3rd edition). 2020.
• 27. RHDAustralia (ARF/RHD writing group). The 2020 Australian guideline for prevention, diagnosis and management of acute rheumatic fever and rheumatic heart disease (3rd edition). 2020.
• 28. Roos-Hesselink JW, Cornette J, Sliwa K, et al. Contraception and cardiovascular disease. European Heart Journal. 2015;36(27):1728-1734. View Source
• 29. Regitz-Zagrosek V, Roos-Hesselink JW, Bauersachs J, et al. 2018 ESC Guidelines for the management of cardiovascular diseases during pregnancy. European Heart Journal. 2018;39(34):3165-3241. View Source