Introduction Acute rheumatic fever (ARF) and rheumatic heart disease (RHD) cause significant morbidity and premature mortality among Australian Aboriginal and Torres Strait Islander peoples. RHDAustralia has produced a fully updated clinical guideline in response to new knowledge gained since the 2012 edition. The guideline aligns with major international ARF and RHD practice guidelines from the American Heart Association and World Heart Federation to ensure best practice. The GRADE system was used to assess the quality and strength of evidence where appropriate. Main recommendations The 2020 Australian guideline details best practice care for people with or at risk of ARF and RHD. It provides up‐to‐date guidance on primordial, primary and secondary prevention, diagnosis and management, preconception and perinatal management of women with RHD, culturally safe practice, provision of a trained and supported Aboriginal and Torres Strait Islander workforce, disease burden, RHD screening, control programs and new technologies. Changes in management as a result of the guideline Key changes include updating of ARF and RHD diagnostic criteria; change in secondary prophylaxis duration; improved pain management for intramuscular injections; and changes to antibiotic regimens for primary prevention. Other changes include an emphasis on provision of culturally appropriate care; updated burden of disease data using linked register and hospitalisations data; primordial prevention strategies to reduce streptococcal infection addressing household overcrowding and personal hygiene; recommendations for population‐based echocardiographic screening for RHD in select populations; expanded management guidance for women with RHD or ARF to cover contraception, antenatal, delivery and postnatal care, and to stratify pregnancy risks according to RHD severity; and a priority classification system for presence and severity of RHD to align with appropriate timing of follow‐up.

Despite dramatic gains of the last century, acute rheumatic fever and rheumatic heart disease remain major preventable causes of morbidity and mortality in many parts of the world

ARF may be more common than previously thought among low-risk children. These data should prompt an awareness of ARF diagnosis and management across all regions, including strategies for primary prevention. There should be renewed emphasis on treatment of sore throat in high-risk groups.

Most children presenting to the hospital with ARF lived in disadvantaged areas of Sydney. Pacific Islander and Aboriginal and Torres Strait Islander children were over-represented. Mitigation of RHD requires early identification of ARF and appropriate delivery of secondary prophylaxis.