Rheumatic Heart Disease In Australia Research Articles

Excess Mortality Among People With Rheumatic Heart Disease in Australia

Excess Mortality Among People With Rheumatic Heart Disease in Australia

Is the Australian primary healthcare system ready for the Rheumatic Heart Disease Endgame strategy? Data synthesis and recommendations

Is the Australian primary healthcare system ready for the Rheumatic Heart Disease Endgame strategy? Data synthesis and recommendations

Roadmap to incorporating group A Streptococcus molecular point-of-care testing for remote Australia: a key activity to eliminate rheumatic heart disease.

Roadmap to incorporating group A Streptococcus molecular point-of-care testing for remote Australia: a key activity to eliminate rheumatic heart disease.

1219Translating epidemiological findings to end rheumatic heart disease in Australia: the ERASE project

Abstract Focus of Presentation Rheumatic fever (RF) and rheumatic heart disease (RHD) are endemic among Indigenous Australians. End RHD in Australia: Study of Epidemiology (ERASE) aimed to characterize contemporary RF/RHD epidemiology. Using multi-jurisdictional linked data from several administrative sources, we undertook sub-studies covering diverse epidemiological questions, requiring substantial methods development. Mixed methods further identified barriers/facilitators to inform system redesign. Our multi-disciplinary collaboration supported diverse initiatives to contribute to policy at government, service and community/stakeholder levels. We show how findings from ERASE were applied/translated to address the impact of RF/RHD in Australia. Findings Academic: >15 papers and commentaries/editorials provided the backbone to translational outputs and methods sharing. PhD students have ongoing projects using ERASE datasets. Advocacy: ERASE epidemiological and economic information supported the Endgame Strategy (roadmap for eliminating RHD in Australia by 2031) presented to government. Health professionals: ERASE data contributed to Australian RF/RHD guidelines. Slides of results/interpretation are publically-available on the RHDAustralia website. Student lectures integrate biomedical and culturally-informed perspectives. Indigenous stakeholder engagement: involves (1)presentations to peak Indigenous-controlled organisations (2)co-designed resources (booklets/slides) for capacity-building of RHDAustralia’s national Champions4Change network (3)research workshops to promote two-way learning and health literacy/numeracy. Challenges remain regarding strengths-based approaches when reporting high disparities. Conclusions/Implications Strong translational commitment and national multi-disciplinary networks of Indigenous and non-Indigenous collaborators ensured ERASE generated multiple outputs that continue to inform training, practice, policy and community health literacy. Key messages Build translation and broad collaboration into study from the start.

Primary prevention of acute rheumatic fever.

Acute rheumatic fever (ARF) is an abnormal immune reaction following Streptococcus pyogenes (Strep A) infection of the throat, and likely the skin. Primary prevention is the prompt and appropriate antibiotic treatment of StrepA infection, and it can reduce the risk of developing ARF and subsequent rheumatic heart disease. This article explores current recommendations for primary prevention of ARF in Australia. People at increased risk of ARF should beoffered empirical antibiotic treatment of Strep A infections to reduce this risk. People at increased ARF risk include young Aboriginal and Torres Strait Islander people in remote Australia as well as those with a personal or family history of ARF and people from migrant communities in urban areas, including Māori and Pacific Island people. Risk-stratified primary prevention can reduce the inequitable burden of ARF and rheumatic heart disease in Australia.

Decolonizing Indigenous health: Generating a productive dialogue to eliminate Rheumatic Heart Disease in Australia

In common with colonized Indigenous people worldwide, many Australian Aboriginal people experience inequitable health outcomes. While the commitment and advocacy of researchers and health practitioners has resulted in many notable improvements in policy and practice, systemic and structural impediments continue to restrain widespread gains in addressing Indigenous health injustices. We take Rheumatic Heart Disease (RHD), a potent marker of extreme health inequity, as a case study, and critically examine RHD practitioners' perspectives regarding the factors that need to be addressed to improve RHD prevention and care. This study is an important explanatory component of a broader study to inform new clinical practices, and health system strategies and policies to reduce RHD. A decolonising, critical medical anthropology (CMA) analysis of findings from 22 RHD practitioner in-depth interviews conducted in May 2016 revealed both practitioners' perceptions of health system shortcomings and a sense of hopelessness and powerlessness to transform existing health system inequities, the negative impacts of which were subsequently confirmed in a separate study of RHD patients' lived realities. We reveal how biomedical dominance, normalized deficit discourses and systemic racism influence the current policy and practice landscape, narrowing the intercultural space for productive dialogue and reinforcing the conditions that cause disease. To counter biomedical approaches that contribute to existing health inequities in health care, we recommend localized, strength-based, community-led research projects focused on actions that use critical decolonizing social science approaches to achieve system change. We demonstrate the importance of integrating biological and social sciences approaches in research, education/training, and practice to: 1) be guided by Indigenous strengths, knowledges and worldview, and 2) adopt a critical reflexive stance to examine systems, structures and practices. Such an approach facilitates productive cross-cultural dialogue and social transformation; providing direction and hope to practitioners, enhancing their knowledge, skills and capacity and improving Aboriginal health outcomes.

Repeated Prosthetic Valve Thrombosis—A Case Report Illustrating the Need for Consensus in a Time Sensitive Therapy

Of patients with rheumatic heart disease in Australia, 88.8% are Indigenous and often in remote or under-resourced communities [ [1] Katzenellenbogen J.M. Bond-Smith D. Seth R.J. Dempsey K. Cannon J. Stacey I. et al. Contemporary incidence and prevalence of rheumatic fever and rheumatic heart disease in Australia using linked data: the case for policy change. J Am Heart Assoc. 2020; 9: e016851 Crossref PubMed Scopus (33) Google Scholar ]. Surgical intervention remains an important treatment but survival and freedom from complications for Indigenous patients is reportedly lower than in similar cohorts from developing countries [ [2] Carapetis J.R. Powers J.R. Currie B.J. Sangster J.F. Begg A. Fisher D.A. et al. Outcomes of cardiac valve replacement for rheumatic heart disease in aboriginal Australians. Asia Pacific Heart J. 1999; 8: 138-147 Abstract Full Text PDF Scopus (20) Google Scholar ]. Prosthetic valve thrombosis is a rare but serious complication and thrombolysis is known to be successful in the Indigenous population [ [3] Milne O. Barthwal R. Agahari I. Ilton M. Kangaharan N. Management and outcomes of prosthetic valve thrombosis. An Australian case series from the Northern Territory. Heart Lung Circ. 2020; 29: 469-474 Abstract Full Text Full Text PDF PubMed Scopus (7) Google Scholar ]. There is, however, no consensus as to which thrombolytic therapy regimen to prescribe.

Rheumatic heart disease: A review of the current status of global research activity.

Rheumatic heart disease (RHD) is a serious and long-term consequence of acute rheumatic fever (ARF), an autoimmune sequela of a mucosal infection by Streptococcus pyogenes (Group A Streptococcus, Strep A). The pathogenesis of ARF and RHD is complex and not fully understood but involves host and bacterial factors, molecular mimicry, and aberrant host innate and adaptive immune responses that result in loss of self-tolerance and subsequent cross-reactivity with host tissues. RHD is entirely preventable yet claims an estimated 320 000 lives annually. The major burden of disease is carried by developing nations and Indigenous populations within developed nations, including Australia. This review will focus on the epidemiology, pathogenesis and treatment of ARF and RHD in Australia, where: streptococcal pyoderma, rather than streptococcal pharyngitis, and Group C and Group G Streptococcus, have been implicated as antecedents to ARF; the rates of RHD in remote Indigenous communities are persistently among the highest in the world; government register-based programs coordinate disease screening and delivery of prophylaxis with variable success; and researchers are making significant progress in the development of a broad-spectrum vaccine against Strep A.

Pioneering Methods Developed to Investigate the Burden of Acute Rheumatic Fever and Rheumatic Heart Disease Using Multiple Linked Data Sources

IntroductionAcute Rheumatic Fever (ARF) and Rheumatic Heart Disease (RHD) remain a major public health concern in Australia. Government action requires reliable burden estimates, however data from single or unlinked sources are only partial and likely to be skewed, exacerbated by systemic problems with ICD-10 codes for RHD. Linked data provide an opportunity to address these shortcomings.
 Objectives and ApproachObjectives: to develop a methodology using harmonised linked data across five Australian jurisdictions to determine the burden of ARF and RHD <55 years, in particular robust case definitions for calculating incidence and prevalence.
 For identifying RHD in hospital-only patients, validated case and non-cases from non-hospital sources were used with linked inpatient hospital admissions to develop a RHD prediction model. Additional data sources (register and surgery databases) were used to identify cases for reporting RHD prevalence. A unique ARF episode was defined as an ARF record >90 days from the previous one across both register and hospital data. For first-ever episodes we applied a lookback to mid-2001 for both ARF and RHD. For Western Australia, we evaluated the effect of look-back period on prevalence pooling.
 ResultsFor total ARF incidence over 3 years (2015-2017), there was 1425 episodes compared to 1027 episodes for first-ever ARF. There was an annual average of 5241 cases of RHD identified using our new methods (0-54yrs) – substantially higher than 2634 and 4255 RHD cases reported by Global Burden of Disease Study and Australian Institute of Welfare estimates respectively for 2017. Increased lookback had no effect on first-ever ARF but increased RHD prevalence >25 years.
 Conclusion / ImplicationsBy using multiple sources and cross-jurisdictional data we were able to provide contemporary and robust estimates for the burden of ARF and RHD in Australia. The prediction algorithm we developed can also be used in other countries, where only hospital data is available, to quantify RHD burden.

The use of cardiac valve procedures for rheumatic heart disease in Australia; a cross-sectional study 2002–2017

BackgroundAustralia, although a high income economy, carries a significant burden of rheumatic heart disease (RHD). Acute rheumatic fever (ARF) and RHD are endemic in the Indigenous population. Immigrants from low/lower-income countries (‘non-Indigenous high-risk’) are also at increased risk compared with ‘non-Indigenous low-risk’ Australians. This study describes the utilisation of surgical and percutaneous procedures for RHD-related valve disease among patients aged less than 50 years, from 2002 to 2017. MethodsA descriptive study using data from the ‘End RHD in Australia: Study of Epidemiology (ERASE) Project’ linking RHD Registers and hospital inpatient data from five states/territories, and two surgical databases. Trends across three-year periods were determined and post-procedural all-cause 30-day mortality calculated. ResultsA total of 3900 valves interventions were undertaken in 3028 procedural episodes among 2487 patients. Over 50% of patients were in the 35–49 years group, and 64% were female. Over 60% of procedures for 3-24 year-olds were for Indigenous patients. There were few significant changes across the study period other than downward trends in the number and proportion of procedures for young Indigenous patients (3–24 years) and ‘non-Indigenous/low risk’ patients aged ≥35 years. Mitral valve procedures predominated, and multi-valve interventions increased, including on the tricuspid valve. The majority of replacement prostheses were mechanical, although bioprosthetic valve use increased overall, being highest among females <35 years and Indigenous Australians. All-cause mortality (n = 42) at 30-days was 1.4% overall (range 1.1–1.7), but 2.0% for Indigenous patients. ConclusionsThe frequency of cardiac valve procedures, and 30-day mortality remained steady across 15 years. Some changes in the distribution of procedures in population groups were evident. Replacement procedures, the use of bioprosthetic valves, and multiple-valve interventions increased. The challenge for Australian public health officials is to reduce the incidence, and improve the early detection and management of ARF/RHD in high-risk populations within Australia.

Ending rheumatic heart disease in Australia: the evidence for a new approach.

■The RHD Endgame Strategy: the blueprint to eliminate rheumatic heart disease in Australia by 2031 (the Endgame Strategy) is the blueprint to eliminate rheumatic heart disease (RHD) in Australia by 2031. Aboriginal and Torres Strait Islander people live with one of the highest per capita burdens of RHD in the world. ■The Endgame Strategy synthesises information compiled across the 5-year lifespan of the End Rheumatic Heart Disease Centre of Research Excellence (END RHD CRE). Data and results from priority research projects across several disciplines of research complemented literature reviews, systematic reviews and narrative reviews. Further, the experiences of those working in acute rheumatic fever (ARF) and RHD control and those living with RHD to provide the technical evidence for eliminating RHD in Australia were included. ■The lived experience of RHD is a critical factor in health outcomes. All future strategies to address ARF and RHD must prioritise Aboriginal and Torres Strait Islander people's knowledge, perspectives and experiences and develop co-designed approaches to RHD elimination. The environmental, economic, social and political context of RHD in Australia is inexorably linked to ending the disease. ■Statistical modelling undertaken in 2019 looked at the economic and health impacts of implementing an indicative strategy to eliminate RHD by 2031. Beginning in 2019, the strategy would include: reducing household crowding, improving hygiene infrastructure, strengthening primary health care and improving secondary prophylaxis. It was estimated that the strategy would prevent 663 deaths and save the health care system $188 million. ■The Endgame Strategy provides the evidence for a new approach to RHD elimination. It proposes an implementation framework of five priority action areas. These focus on strategies to prevent new cases of ARF and RHD early in the causal pathway from Streptococcus pyogenes exposure to ARF, and strategies that address the critical systems and structural changes needed to support a comprehensive RHD elimination strategy.

Contemporary Incidence and Prevalence of Rheumatic Fever and Rheumatic Heart Disease in Australia Using Linked Data: The Case for Policy Change.

BackgroundIn 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 ResultsThis 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.ConclusionsThis 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.

The RHD Endgame Strategy; the blueprint to eliminate rheumatic heart disease in Australia by 2031

Abstract Background Rheumatic heart disease (RHD) is a preventable yet deadly condition resulting from untreated Group A Streptococcus infection. Despite being eliminated from most high-income countries, RHD and its precursor acute rheumatic fever (ARF) persist in developing countries and settings of disadvantage. In Australia, Aboriginal and Torres Strait Islander people experience among the world's highest rates. Following five years of research, investigation and advocacy, the Endgame Strategy provides a technical foundation to eliminate RHD in Australia by 2031. Methods A range of potential strategies to reduce ARF and RHD were identified. Approaches at the social and environmental, primary, secondary and tertiary prevention levels were evaluated using the GRADE Evidence to Decision framework, together with structural review of the health system. Recommendations were made according to level of prevention opportunity and responsibility. Modelling was undertaken to estimate the health and economic impact of an indicative bundle of the most promising strategies. Results Reducing household crowding, improving health infrastructure, strengthening primary healthcare and enhancing delivery of secondary prophylaxis were identified as having the greatest potential impact on RHD. They are also largely acceptable, practical and readily implementable with investment. Modelling indicates that this approach would reduce ARF and RHD by 69% and 71% respectively, preventing 471 deaths and saving $188 million on healthcare expenditure to 2031. Conclusions Eliminating RHD is only possible with a holistic approach led by Aboriginal and Torres Strait Islander people with communities at the core. This will entail funding communities to develop programs, resourcing a national RHD unit to coordinate efforts across Australia, guaranteeing access to healthy housing and built environments, establishing comprehensive skin and throat programs and improving the health of those living with ARF and RHD. Key messages RHD demands responses from the health sector strengthened by political advocacy and engagement to drive evidence-based decision making. The evidence and collective experience now exist to eliminate RHD, an exemplar of the gap in health outcomes between Aboriginal and Torres Strait Islander peoples and the non-Indigenous population.

Challenging “business as usual” for acute rheumatic fever and rheumatic heart disease in Australia

Challenging “business as usual” for acute rheumatic fever and rheumatic heart disease in Australia

702 Long-Term Survival and Predictors of Outcomes of Indigenous Northern Territorians Undergoing Primary and Re-Do Surgery for Rheumatic Heart Disease in Australia

Rheumatic Heart Disease (RHD) is the leading cause of heart valve dysfunction among the Indigenous population of Australia. Factors that contribute to late postoperative mortality have not been previously studied in Australia. 342 RHD Indigenous patients from the Northern Territory of Australia undergoing their primary surgical procedure between 1997 and 2016 were identified. Pre- and postoperative data was collated using clinical information systems. Multivariable survival analyses were performed using Cox Proportional Hazards model. Early 30-day mortality was 2% (CI 0.5-3.5). Mean follow-up time was 8.2 years (2,784 patient years). 63.5% were female. Mean age of RHD diagnosis was 25 years, mean age at first surgery was 31.6 years. Mean time interval from date of first RHD diagnosis to first surgical intervention was 6.3 years. 22% (n=74) underwent redo surgery, among these 75%, 20% and 5% underwent one, two and three redo surgeries respectively. There was a total of 93 (27.2%) deaths. Actuarial Survival at 5, 10, 15 and 20 years was 89% 70% 58% and 52% respectively. Preoperative Pulmonary Hypertension (ePASP >50mmHg), Chronic Kidney Disease (eGFR <60) and Coronary Heart Disease were independent predictors of mortality regardless of type of surgery and number of valves operated on. Indigenous Australians have poor long-term survival following cardiac surgery for RHD despite their young age. This is at least in part attributed to co-morbid conditions. Significant effort is required to prevent this disease, enable early detection and enhance early access to surgery with close long term follow-up.

The End Rheumatic Heart Disease in Australia Study of Epidemiology (ERASE) Project: data sources, case ascertainment and cohort profile.

PurposeAcute rheumatic fever (ARF) and rheumatic heart disease (RHD) persist as public health issues in developing countries and among disadvantaged communities in high-income countries, with rates in Aboriginal and Torres Strait Islander peoples in Australia among the highest recorded globally. A robust evidence base is critical to support policy recommendations for eliminating RHD, but available data are fragmented and incomplete. The End RHD in Australia: Study of Epidemiology (ERASE) Project aims to provide a comprehensive database of ARF and RHD cases in Australia as a basis for improved monitoring and to assess prevention and treatment strategies. The objective of this paper is to describe the process for case ascertainment and profile of the study cohort.Patients and methodsThe ERASE database has been built using linked administrative data from RHD registers, inpatient hospitalizations, and death registry data from 2001 to 2017 (mid-year). Additional linked datasets are available. The longitudinal nature of the data is harnessed to estimate onset and assess the progression of the disease. To accommodate systematic limitations in diagnostic coding for RHD, hospital-only identified RHD has been determined using a purposefully developed prediction model.ResultsOf 132,053 patients for whom data were received, 42,064 are considered true cases of ARF or RHD in the study period. The patient population under 60 years in the compiled dataset is more than double the number of patients identified in ARF/RHD registers (12,907 versus 5049). Non-registered patients were more likely to be older, non-Indigenous, and at a later disease stage.ConclusionThe ERASE Project has created an unprecedented linked administrative database on ARF and RHD in Australia. These data provide a critical baseline for efforts to end ARF/RHD in Australia. The methodological work conducted to compile this database resulted in significant improvements in the robustness of epidemiological estimates and entails valuable lessons for ARF/RHD research globally.

Public health and economic perspectives on acute rheumatic fever and rheumatic heart disease.

Public health and economic perspectives on acute rheumatic fever and rheumatic heart disease.

Articulating the Endgame for Rheumatic Heart Disease in Australia

The END RHD Centre of Research Excellence (CRE) was funded in 2014 to develop a set of ‘costed stepwise strategies’ to end rheumatic heart disease (RHD) in Australia by 2031. Five years after the grant was awarded the END RHD CRE is in the late stages of preparing the Endgame Strategy. This abstract reviews outputs to date, modelling strategy and consultation plans around the Endgame Strategy. A foundational Cost of Inaction report was published in late 2018 outlining current estimates of RHD burden and modelling expected medical costs. This report is being used to underpin a Cost of Action estimate, by exploring opportunities to change disease trajectory at a primordial, primary, secondary and tertiary level. Literature reviews are underway to define disease modifying strategies, cost and magnitude of effect at each level of prevention. Potential strategies are subject to community consultation including a formal Delphi study to explore preferences around primary prevention. A new disease model, incorporating social determinants of health and primary prevention strategies, will be used to estimate the effect in the Australian setting Combining literature reviews and consultation activities and policy mapping it will be possible to determine high priority strategies, model their cost and impact and provide guidance about approaches to ending RHD in Australia. Bipartisan political commitments have been made to reduce the burden of RHD in Australia. The Endgame Strategy is expected to underpin implementation of national approaches to the disease and contribute to closing the gap in health outcomes.

Echocardiographic Screening Detects Extremely High Prevalence of RHD in Australia

Introduction: Globally, most patients with rheumatic heart disease (RHD) do not have a history of acute rheumatic fever (ARF). The Indigenous people of the Northern Territory (NT) of Australia have some of the highest rates of ARF and RHD in the world. Coordinated register-based delivery of secondary prophylaxis has been active for two decades. This study aimed to better define the burden of RHD using active case finding for RHD in school-aged children in a remote community in the NT. Methods: A community-driven approach to engagement and education on ARF/RHD was implemented on the invitation of the Maningrida community. For active case finding, participants aged 5–20y had echocardiography performed by cardiologists. Diagnoses of RHD were made according to World Heart Federation criteria. Echo data were combined with register data to describe the burden of ARF/RHD. Results: Estimated target population (aged 5–20y) was 849 people. Of these, 615 (72.4%) underwent echocardiography (median age 11y). An existing diagnosis of probable/definite ARF and/or RHD was recorded in 44/615 (7.2%). At screening, 13 known cases and 19 new cases were found to have definite RHD (32/615, 5.2%); 6/615 (1.0%) have had cardiac surgery for severe RHD, including 3 new cases. Conclusion: The burden of ARF/RHD in Maningrida is extremely high. Active case finding using echocardiography facilitates detection and access to treatment, even in settings with effective primary health care and ongoing disease surveillance. Community engagement and education are key to early detection and treatment, as well as primordial and primary prevention of ARF/RHD.

The Cost of Inaction on Rheumatic Heart Disease in Australia

The Cost of Inaction on Rheumatic Heart Disease in Australia