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The Australian approach to dealing with acute rheumatic fever (ARF) and rheumatic heart disease (RHD) in Aboriginal and Torres Strait Islander people makes an interesting case study. Prior to the mid-1990s, there had been a few publications, largely based on hospital series or individual community experience that hinted at an enormous issue in remote Aboriginal communities of northern Australia. The year 1996 saw the publication of the first population-based data, from the Northern Territory, documenting the highest rates in the world of ARF and among the highest in the world of RHD, in the Aboriginal population.[1] Simple epidemiological analyses confirmed two obvious factors contributing to these high rates: recurrences represented more than 40% of all ARF episodes (highlighting poor delivery of benzathine penicillin G secondary prophylaxis injections); and approximately 40% of patients with RHD presented with no prior history of ARF (highlighting poor mechanisms to diagnose ARF and enter people into prevention programs in the first place).

Researchers at the Menzies School of Health Research worked with colleagues in the NT Department of Health to develop a strategy based on a World Health Organization recipe for RHD control – establishment of register-based RHD control programs. A proposal was made to the Commonwealth Government, some money was cobbled together from various pots, and the Top End RHD Control Program was born in 1997. The story between then and 2009 was one of continuing research linked strongly to service delivery. The NT Control Program expanded to include Central Australia, and the two registers were combined in 2007. This program had many documented successes, particularly the ability to track existing patients, and to ensure the availability of specialist services, including cardiac surgery, to those who need it the most. However, it had trouble tackling the number one priority of control programs – improving delivery of secondary prophylaxis – in a coordinated fashion, for many reasons. Most important among these impediments was the uncertainty of funding from the Commonwealth, meaning that the program staff were not in a position to plan long-term multi-year strategies, because they weren't certain that they would be around from one year to the next.

During this period, the momentum for a more national approach gathered steam, still with a strong link to research. The National Heart Foundation and the Cardiac Society of Australia and New Zealand pulled together the major researchers, clinicians and public health practitioners working in ARF/RHD in Australia and New Zealand to produce Australia's first evidence-based guidelines on this topic, published in 2006.[2] This document quickly became the source material for all approaches to ARF/RHD in Australia, and became recognised in many places as the most authoritative guideline in the world (the updated 2012 edition has just been released[3]). One important feature of the Australian guideline was that, for the first time, it recognised that a population may include subgroups with very different baseline risks for these diseases, and that this should translate to different approaches to diagnosis and management. Neither the World Health Organization Criteria or the Jones Criteria for ARF diagnosis do this, with the result that neither of those guidelines is perceived by practitioners working in high incidence settings to fully meet their local needs.

In 2009, the most important advance in Australian RHD control occurred – the announcement of the National RF Strategy, which had two core elements: Commonwealth funding for control programs in the most-affected jurisdictions (NT, WA, Qld); and establishment of a National Coordination Unit (NCU), with responsibility for data management and reporting at a national level, production of education and health promotion materials, and updating of the guidelines. The establishment of the NCU, known as RHDAustralia (see http://www.rhdaustralia.org.au) was unique in the world, and is a critical strength of the Australian approach.

All along, research has continued – some of it investigator initiated and conducted essentially independent of service delivery (e.g. studies of pathogenesis of streptococcal infections, development of a rheumatic fever vaccine in the laboratory), but most of it occurs together with, sometimes embedded in, service delivery. Examples of the latter include a large RHD echocardiographic screening study in NT, WA and Qld (recently completed, with result to be reported in the next 6–12 months), a follow up study currently underway, aiming to document the longer-term outcomes of children with possible abnormalities detected at ultrasound screening, a community-randomised trial of a health services intervention to improve secondary prophylaxis delivery soon to commence in the NT, a study of genetic susceptibility to RHD currently underway in the NT, a range of intervention studies aimed at reducing scabies and skin sores, partly in the hope of affecting ARF rates, and the list goes on. Moreover, the evidence base emerging from Australia, and the way in which researchers have been able to influence public policy and practice, have placed Australia at the forefront of the ARF/RHD movement globally. It should be noted with pride by Australians that the surge in interest and activities in ARF and RHD in this country has had a significant role in stimulating related interest in sub-Saharan Africa, Asia and the Pacific.

Things are also gathering pace across the Tasman. New Zealand understood the importance of ARF and RHD in Māori and Pacific Islander populations well before Australia did, and made great strides in implementing secondary prophylaxis programs during the 1980s and 1990s. More recently, again with a strong link to research, New Zealand has embarked on an ambitious program of primary prevention, and even more remarkably the New Zealand government has recently announced a target of reducing ARF incidence by two-thirds within 5 years, and is putting its money where its mouth is. If we in the Antipodes can demonstrate to the world that these diseases can be controlled using active public health measures, we will be both the envy of, and a model for, the world.

One strategy that seems to be gaining steam around the world, particularly in developing countries, is the use of portable echocardiography to screen school-aged children. The theory is that one can identify new cases of RHD before they progress to heart failure, and improve the outcomes for those children by starting secondary prophylaxis early in the natural history of the disease, when its benefit should be the greatest. The pivotal studies in this field came out of Africa, South-East Asia and the Pacific a few years ago.[4, 5] Since then, we have seen a mushrooming of echo screening studies in dozens of countries.

In this edition of the Journal of Paediatrics and Child Health, Remond and colleagues give us cause for pause in Australia.[6] They point out that there are many unknowns about echo screening. Firstly, it is not known that early institution of secondary prophylaxis will prevent the subsequent occurrence of more severe RHD in patients with subclinical disease. Unfortunately, this question will take many years to resolve, either through large multicentre randomised trials (which are being contemplated, but face many obstacles) or through less rigorous prospective observational studies. In the meantime, given that secondary prophylaxis leads to disease resolution much more commonly in people with mild than severe RHD, it seems sensible to assume that delivery of secondary prophylaxis earlier in the disease process will have the greatest impact. So the major concerns about echo screening outlined by Remond et al. are: the lack of specificity of the diagnosis (potentially leading to many more false positives than true positives, which in turn not only leads to additional costs and inconvenience in confirming the diagnosis, but also causes significant stress and distress in individuals and families found to have an abnormal screening test); suboptimal assessment and follow-up of existing patients with RHD (raising concerns over our ability to adequately care for the new cases that may be detected by screening); poor delivery of secondary prophylaxis to existing patients on ARF/RHD registers (which is needed if there is to be any benefit to individuals with RHD detected by screening); logistical constraints to being able to screen in rural and remote areas and the impact this may have on other health services; and the lack of clarity about the cost, and cost-effectiveness of screening (although the authors suggest that this is not likely to be a major concern because of previous evidence of the cost-effectiveness of secondary prophylaxis). Remond and colleagues conclude that ‘it is not yet time’ to advocate for screening in this country.

On the whole I agree with this sentiment, but I would put it slightly differently. The issues raised by Remond et al. all require addressing, and there are others. For example, what is the true yield of new cases detected by screening, and will they all be ‘subclinical’ (implying the absence of clinically-significant disease, both in terms of impairment of cardiac function and the presence of a pathological heart murmur) or will some detected cases have evidence of clinical disease? And we definitely must take into account economic considerations as well as the opportunity costs – how funds for screening programs might otherwise be spent. But decisions about public health programs in this country are not based on economic considerations alone. There are plenty of examples of programs in Australia – universal Meningococcal Group C vaccination is one example – that are implemented without strong evidence of cost-effectiveness. There may be other considerations to be taken into account for RHD, including the fact that it is a classic disease of social inequity and has real potential to be eliminated in this country. It may be worth a few extra dollars to reach this goal.

Screening has other potential benefits that may lead to reduced ARF/RHD burden beyond the impact of identifying individual new cases. Some developing countries without any existing infrastructure for secondary prophylaxis delivery are choosing to focus on screening as a way of raising the profile of the disease in their country, and to gain some disease burden data to use in health priority setting. This aspect should not be underestimated in Australia – we don't necessarily need the data to make a case, but screening programs are often opportunities to highlight the importance of ARF and RHD in individual communities and among health services and health departments, and they may be a catalyst for addressing a range of aspects of ARF/RHD care in communities.

There is one other critical point that the reader may not glean from Remond et al.'s article. The information we need to address most of their concerns is already on the way. Indeed, the authors could give more weight to the recent publication of evidence based guidelines for the diagnosis of RHD on echocardiography by the World Heart Federation.[7] This exercise, for the first time, attempted to bring a higher level of science to something that has often been seen as an art form. Although the criteria will undoubtedly evolve as more evidence comes to light, they are an enormous advance in reaching some clarity about what is, and what is not, RHD on echocardiography. And there are many more screening and follow up projects in Australia and around the world that should be reporting their results in the next 2 to 3 years. Moreover, there is early evidence that the National RF Strategy is beginning to lead to improvements in secondary prophylaxis delivery and consequent reductions in ARF recurrence rates.

In other words, while the currently available published data support Remond et al.'s conclusions that the time is not yet right to start routine screening for RHD in Aboriginal and Torres Strait Islander communities, we will have a lot more evidence on which to base such a decision in the next couple of years. Any decision will require careful examination of this evidence, but should also include consideration of other factors. We can be more optimistic than ever that control of RHD in Australia's Aboriginal and Torres Strait Islander populations is within reach.

References

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  2. References
  • 1
    Carapetis JR, Wolff DR, Currie BJ. Acute rheumatic fever and rheumatic heart disease in the Top End of Australia's Northern Territory. Med. J. Aust. 1996; 164: 146149.
  • 2
    National Heart Foundation of Australia and the Cardiac Society of Australia and New Zealand. Diagnosis and Management of Acute Rheumatic Fever and Rheumatic Heart Disease in Australia: An Evidence-Based Review. Melbourne: National Heart Foundation of Australia, 2006.
  • 3
    RHDAustralia (ARF/RHD Writing Group), National Heart Foundation of Australia and the Cardiac Society of Australia and New Zealand. Australian Guideline for Prevention, Diagnosis and Management of Acute Rheumatic Fever and Rheumatic Heart Disease, 2nd edn. Darwin: Menzies School of Health Research, 2012.
  • 4
    Carapetis JR, Hardy M, Fakakovikaetau T et al. Evaluation of a screening protocol using auscultation and portable echocardiography to detect asymptomatic rheumatic heart disease in Tongan schoolchildren. Nat. Clin. Pract. Cardiovasc. Med. 2008; 5: 411417.
  • 5
    Marijon E, Ou P, Celermajer DS et al. Prevalence of rheumatic heart disease detected by echocardiographic screening. N. Engl. J. Med. 2007; 357: 470476.
  • 6
    Remond MGW, Wark EK, Maguire GP. Screening for rheumatic heart disease in Aboriginal and Torres Strait Islander children. J. Paediatr. Child Health 2012; 49: 526531.
  • 7
    Remenyi B, Wilson N, Steer A et al. World Heart Federation criteria for echocardiographic diagnosis of rheumatic heart disease – an evidence-based guideline. Nat. Rev. Cardiol. 2012; 9: 297309.