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Background In 2018, the World Health Organization prioritized control of acute rheumatic fever (ARF) and rheumatic heart disease (RHD), including disease surveillance. We developed strategies for estimating contemporary ARF/RHD incidence and prevalence in Australia (2015–2017) by age group, sex, and region for Indigenous and non‐Indigenous Australians based on innovative, direct methods. Methods and Results This population‐based study used linked administrative data from 5 Australian jurisdictions. A cohort of ARF (age <45 years) and RHD cases (<55 years) were sourced from jurisdictional ARF/RHD registers, surgical registries, and inpatient data. We developed robust methods for epidemiologic case ascertainment for ARF/RHD. We calculated age‐specific and age‐standardized incidence and prevalence. Age‐standardized rate and prevalence ratios compared disease burden between demographic subgroups. Of 1425 ARF episodes, 72.1% were first‐ever, 88.8% in Indigenous people and 78.6% were aged <25 years. The age‐standardized ARF first‐ever rates were 71.9 and 0.60/100 000 for Indigenous and non‐Indigenous populations, respectively (age‐standardized rate ratio=124.1; 95% CI, 105.2–146.3). The 2017 Global Burden of Disease RHD prevalent counts for Australia (<55 years) underestimate the burden (1518 versus 6156 Australia‐wide extrapolated from our study). The Indigenous age‐standardized RHD prevalence (666.3/100 000) was 61.4 times higher (95% CI, 59.3–63.5) than non‐Indigenous (10.9/100 000). Female RHD prevalence was double that in males. Regions in northern Australia had the highest rates. Conclusions This study provides the most accurate estimates to date of Australian ARF and RHD rates. The high Indigenous burden necessitates urgent government action. Findings suggest RHD may be underestimated in many high‐resource settings. The linked data methods outlined here have potential for global applicability.

on behalf of the END RHD CRE Investigators Collaborators Acknowledgements: • Children, families and communities living with RHD-We thank the Aboriginal and Torres Strait Islander people for sharing their stories in the Endgame Strategy, and acknowledge that the research and data in this publication reflect the experiences of Aboriginal and Torres Strait Islander people and communities affected by the ongoing trauma of ARF and RHD. • END RHD Review Working Group-We thank the following members of the END RHD Alliance, who formed an expert working group to review content of the Endgame Strategy for feasibility and acceptability, including review from a cultural perspective:

BackgroundRheumatic heart disease (RHD) remains a disease of international importance, yet little has been published about disease progression in a contemporary patient cohort. Multi‐state models provide a well‐established method of estimating rates of transition between disease states, and can be used to evaluate the cost‐effectiveness of potential interventions. We aimed to create a multi‐state model for RHD progression using serial clinical data from a cohort of Australian patients.Methods and ResultsThe Northern Territory RHD register was used to identify all Indigenous residents diagnosed with RHD between the ages of 5 and 24 years in the time period 1999–2012. Disease severity over time, surgeries, and deaths were evaluated for 591 patients. Of 96 (16.2%) patients with severe RHD at diagnosis, 50% had proceeded to valve surgery by 2 years, and 10% were dead within 6 years. Of those diagnosed with moderate RHD, there was a similar chance of disease regression or progression over time. Patients with mild RHD at diagnosis were the most stable, with 64% remaining mild after 10 years; however, 11.4% progressed to severe RHD and half of these required surgery.ConclusionsThe prognosis of young Indigenous Australians diagnosed with severe RHD is bleak; interventions must focus on earlier detection and treatment if the observed natural history is to be improved. This multi‐state model can be used to predict the effect of different interventions on disease progression and the associated costs.