Recurrence and progression following first-ever acute rheumatic fever in Queensland youth, 2001–2021: A retrospective cohort study using linked administrative data

Abstract

Background: The high burden of acute rheumatic fever (ARF), inflammatory precursor of rheumatic heart disease (RHD), persists among Australia Indigenous and migrant populations. Contemporary evidence of outcomes following first-ever ARF in population-based samples are sparse internationally. We determined the probability and risk predictors of ARF progression in Queensland, Australia (ages <35-years), during 2001–2021. <div> <br> </div> <div> Methods: Linked RHD Register, hospital and death data were used for this retrospective cohort analysis, incorporating ≤20-years follow-up. First-ever ARF episodes were identified and survival methods used to estimate probabilities and risk predictors of progression to ARF recurrence, any RHD, complicated RHD (heart failure, stroke, endocarditis, atrial fibrillation) and a composite Major Adverse Cardiovascular Endpoint (MACE), including cardiovascular-cause death. We also examined the subset of outcomes following first-ever ARF diagnosed concurrently with RHD. <div> <br> </div> <div> Findings: First-ever ARF was identified (n=1570, 84% Indigenous, 52% female, 59% 5-14-years, 25% very remote), incorporating n=582 (37%) concurrent ARF/RHD. Within 15-years of first-ever ARF-only, 24.1% experienced ARF recurrence, 53.4% experienced RHD, 11.6% experienced RHD-associated complications and 5.5% had a MACE. Comparatively, 27.4% developed RHD-associated complications and 14.3% MACE within 15-years of concurrent ARF/RHD. Major risk predictors of progression to RHD from ARF-only were age <15-years (HR=2.55) compared to 15–25-years and confirmatory echocardiogram later than 8-weeks of ARF diagnosis (HR=1.39) compared to within 4-weeks. For concurrent ARF/RHD, metro-southeastern hospital service (HR=2.23) was a risk predictor for progression to RHD-associated complications compared to health services in the far north. </div> <div> <br> </div> <div> Interpretation: Young people diagnosed with ARF remain at substantial risk of recurrence and progression to RHD for over 15 years, challenging assumptions of stabilisation after a decade. Findings highlight the importance of prompt echocardiographic confirmation and sustained follow-up. These insights refine prognostic understanding internationally and provide an evidence base to guide prevention, service benchmarks, and policy in high-burden settings. </div> </div>