Health services for Aboriginal and Torres Strait Islander people: handle with care

This special Indigenous health issue of the MJA features stories of successful health care services and programs for Aboriginal and Torres Strait Islander people. As we seek to build on the wealth of experience outlined, it is worth considering what these contributions have to tell us about the characteristics and value of effective Indigenous health services.

It is more than 40 years since the founders of the first Aboriginal health service recognised a need for “decent, accessible health services for the swelling and largely medically uninsured Aboriginal population of Redfern [New South Wales]” (http://www.naccho.org.au/about-us/naccho-history#communitycontrol). There are now about 150 Aboriginal community controlled health services (ACCHSs) in Australia: services that arise in, and are controlled by, individual local communities, and deliver holistic, comprehensive and culturally appropriate health care. Panaretto and colleagues (doi: 10.5694/mja13.00005) describe how these services have led the way in high-quality primary care, as well as enriching both the community and the health workforce.

With the ACCHS model setting the standard, the values of responding to community need, Indigenous leadership, cultural safety, meticulous data gathering to guide improvement, social advocacy and streamlining access have gradually been adopted in other health care settings. The progress of the Southern Queensland Centre of Excellence in Aboriginal and Torres Strait Islander Primary Health Care (a Queensland Health-owned service also known as Inala Indigenous Health Service), is an example (doi: 10.5694/mja14.00766). Among the hallmarks of the service’s vitality are its ever-increasing patient numbers, research output, building of community capacity, expansion into specialist and outreach services and multidisciplinary educational role.

The East Arnhem Scabies Control Program, described by Lokuge and colleagues (doi: 10.5694/mja14.00172), is a dramatic example of innovation inspired by local need. This part of Australia has the highest rates of crusted scabies in the world, and the program involved collaboration between two external organisations, an ACCHS and the Northern Territory Department of Health. Importantly, it was able to be integrated into existing health services and largely delivered by local health workers, using active case finding, ongoing cycles of treatment and regular long-term follow-up.

Mainstream health services are now beginning to take the lead from Indigenous-specific ones. For example, the repeated observation that Indigenous men and women with acute coronary syndromes are missing out on interventions and are at risk of poor outcomes inspired a working group from the National Heart Foundation of Australia to develop a framework to ensure that every Indigenous patient has access to appropriate care (doi: 10.5694/mja12.11175). The framework includes clinical pathways led by Indigenous cardiac coordinators, and it is already producing results.

There is growing evidence for the value of sound and accessible primary care for Indigenous Australians. A letter by Coffey and colleagues (doi: 10.5694/mja14.00057) highlights the significant progress towards closing the mortality gap between Indigenous and non-Indigenous Australians in the NT since 2000, temporally associated with investment in primary health care. A research report from Thomas and colleagues shows that patients with diabetes living in remote communities were more likely to avoid hospital admission if they accessed regular care at one of the remote clinics, saving both lives and money (doi: 10.5694/mja13.11316).

While the diversity of health services and the evidence of effectiveness is indeed something to celebrate, it is a fragile success. In their editorial, Murphy and Reath (doi: 10.5694/mja14.00632) highlight the need for sustained, long-term financial investment in primary health care services for Indigenous Australians and the uncertainty arising from changes to health care funding and Indigenous programs announced in the recent federal Budget (http://nacchocommunique.com/category/close-the-gap-program). The detail of how funding will be reallocated with the “rationalisation” of Indigenous programs has not yet fully emerged. Analysis indicates the cuts over 5 years include $165.8 million to Indigenous health programs, which will be added to the Medical Research Future Fund. New spending on Indigenous programs includes $44 million in 2017–18 for health as part of the Department of the Prime Minister and Cabinet Budget (Adjunct Associate Professor Lesley Russell, Menzies Centre for Health Policy, University of Sydney, NSW, personal communication).

Changes to primary care funding are of particular concern. Knowing, as they do, the importance of removing barriers to access, there is increasing public discussion that ACCHSs and large Aboriginal medical services will not pass on the proposed $7 copayment to patients (http://theage.com.au/act-news/health-service-facing-budget-blackhole-by-not-charging-copayment–20140527-zrpb7.html). This will result in a decrease in funding to services that provide vital programs and deliver high-quality outcomes. The government has stated that everyone should share the deficit burden, yet the copayment has only been targeted at general practitioners and not specialist consultations. Is this fair and equitable?

It seems ironic that this threat to access and resourcing has arisen just as it is emerging that our investment in primary care for Indigenous Australians has been well made. In an Australia where many Aboriginal and Torres Strait Islander people still face significant socioeconomic and health disadvantage, the need for “decent, accessible health services” is greater than ever.

A framework for overcoming disparities in management of acute coronary syndromes in the Australian Aboriginal and Torres Strait Islander population. A consensus statement from the National Heart Foundation of Australia

Cardiovascular disease, particularly coronary heart disease (CHD), is the major cause of premature death for Aboriginal and Torres Strait Islander peoples, accounting for 26% of all deaths.1 Cardiovascular disease is also a major contributor to the gaps in life expectancy between Indigenous and non-Indigenous Australians,2 with recent statistics suggesting that Australian Aboriginal and Torres Strait Islander men and women can expect to live 10.6 and 9.5 fewer years, respectively, than other Australians.3

Acute coronary syndromes (ACS) include a broad spectrum of clinical presentations including ST-elevation myocardial infarction (STEMI), non-STEMI and an accelerated pattern of angina without evidence of myonecrosis (unstable angina); the latter two are often grouped as non-ST-elevation acute coronary syndromes (NSTEACS). Current National Heart Foundation of Australia/Cardiac Society of Australia and New Zealand guidelines highlight the importance of effective systems of care in delivering optimal management of ACS.4–6

In 2006, the Australian Institute of Health and Welfare (AIHW) released a landmark report on access to ACS treatment by Aboriginal and Torres Strait Islander patients. The report found that, compared with other Australians, Indigenous Australians hospitalised with ACS had:

more than twice the rate of death from CHD
a 40% lower rate of being investigated by angiography
a 40% lower rate of percutaneous coronary intervention (PCI)
a 20% lower rate of coronary artery bypass graft (CABG) surgery.7

Further studies have indicated that, over and above inhospital treatment disparities for ACS, Aboriginal and Torres Strait Islander patients have greater mortality 6–12 months after a coronary event than non-Indigenous Australians, owing to suboptimal follow-up care.8–11 These disparities in outcomes underpin the development of this patient-oriented framework for the diagnosis and management of ACS in Aboriginal and Torres Strait Islander peoples. The process of developing the framework is described in Appendix 1.

Aboriginal and Torres Strait Islander people represent 2.5% of the Australian population,7 dispersed at varying density levels across urban, regional and remote locations. In 2006, almost one-third of the Indigenous population lived in major cities; 21% in inner regional areas; 22% in outer regional areas; 10% in remote areas; and 16% in very remote areas.12 While Aboriginal and Torres Strait Islander people make up a small proportion of the population in the large cities, there are higher population densities of Indigenous Australians in regional and remote communities — in the Torres Strait, 83% of the population is Torres Strait Islander; in the Northern Territory, 32% of the total population is Indigenous (> 75% in Arnhem Land); and in Central Australia, Aboriginal people make up 50% of the population.11,13 The diversity of this linguistic, cultural and geographic landscape provides many challenges to the delivery of appropriate cardiac services for these populations.

Patient pathways: barriers and enablers

It is a commonly held misconception that Aboriginal and Torres Strait Islander peoples living in urban centres have outcomes similar to the non-Indigenous urban population. There are a number of systemic issues that detrimentally affect the outcomes of all Indigenous Australians, although these issues are likely to be exacerbated by remoteness. Contributing to negative outcomes across the geographic spectrum are issues of fear, institutional racism,14 cultural misunderstandings, waiting times, transport issues, financial constraints and poor health literacy.

Generic pathways to timely diagnosis and management as recommended by best-practice guidelines for patients with ACS living in urban communities are shown in Appendix 2. The more complex pathway for those living in regional or remote communities is tracked in Appendix 3.

Key barriers contribute to the disparities in care and outcomes experienced by Aboriginal and Torres Strait Islander patients regardless of whether they follow a regional/remote or less convoluted urban pathway. These, along with key strategies for addressing them, are discussed below.

Early response and access

There is strong evidence that Aboriginal and Torres Strait Islander patients with a suspected heart attack may delay presentation to hospital.11 Urgent access to care is firstly dependent on patients and/or their families understanding the potentially serious nature of the situation and the need to seek medical help urgently.

Aboriginal and Torres Straits Islander peoples’ culture, spirituality and health literacy all play major roles in potential communication gaps, particularly in remote and isolated communities where English is likely to be a second language. Qualitative internal research undertaken by the Heart Foundation in 2010 identified factors linked with patient delay (unpublished report; Heart Foundation of Australia. Warning signs Aboriginal and Torres Strait Islander Research. Report of qualitative research. Melbourne: The Social Research Centre, 2010) for Aboriginal and Torres Strait Islander people with ACS, including limited knowledge of symptoms and a lack of awareness of the need for urgent treatment: the symptoms associated with ACS are often not acted on until they are unbearable. Other factors contributing to delay include:15,16

competing personal and family demands that may be seen as higher priorities than the individual’s own health (eg, caring for family members, and cultural or community events)
cultural beliefs
fear of hospitals, which may be perceived as places to go to die or as being unfriendly to Aboriginal and Torres Strait Islander patients based on previous personal or family encounters
lack of available or affordable transport
lack of communication options for calling for assistance
long distance to the nearest hospital
lack of understanding of the diagnostic and treatment pathways, and inability to have family involvement in decision making
going to the nearest Aboriginal community controlled health service before going to hospital or calling an ambulance
inappropriate triage at first contact between a health service provider and patient due to inadequate understanding of the symptoms and signs of ACS on the part of the health care provider.

To overcome these barriers, health care providers must look at effective strategies to improve Aboriginal and Torres Strait Islander peoples’ understanding of the warning signs of a heart attack. This requires the development and provision of culturally appropriate health education through community and primary health care networks, including media organisations. Such programs must not only improve understanding of the warning signs of heart attack within local communities, but also advise how to access immediate help and provide information about the patient journey so that patients and their families understand what might happen once they seek help, and can make informed decisions about treatment. In an urban setting, this includes culturally specific education around the triple zero (000) emergency service and the availability and potential cost of the ambulance service.

Minimising delay: pre-tertiary pathways

Beyond health education and basic access to emergency services, patients require access to timely and effective treatment through a system of care that supports early diagnosis and is responsive to patient need, including digital 12-lead electrocardiogram capability, point-of-care pathology testing and, where relevant, prehospital fibrinolysis.

It is essential that centres providing initial care for patients with suspected ACS are part of a clinical network for the provision of cardiac care, and are appropriately equipped to follow up-to-date evidence-based protocols17 (Appendix 4). For urban populations, tertiary “receiving” hospitals need to develop formalised networks with smaller secondary “referring” hospitals that provide initial triage, as well as with the local ambulance services. In regional and remote settings where patient transfer times to centres with PCI capability are greater than 1 hour, it is essential that there is a formalised “designated provider clinical network” of cardiac care providers — essentially a mechanism for providing or obtaining expert clinical advice at short notice — that includes appropriate infrastructure and treatment and transfer protocols.18,19

NSTEACS

All patients with NSTEACS should have their risk of adverse clinical outcomes assessed to direct management decisions.4 Patients identified as being at high risk should be treated with dual antiplatelet therapy and an antithrombin agent, and arrangements should be made for coronary angiography and (where indicated) revascularisation as soon as possible. Regional and remote communities have the added difficulty of seeking distant, expert medical advice with appropriate and timely transfers to facilities that have at least full diagnostic and risk stratification capability. NSTEACS patients stratified as intermediate risk or high risk are best managed within a structured clinical pathway,4 as ad hoc management can lead to delays in seeking advice and the potential for inappropriate and/or conflicting advice, as well as inefficient use of emergency medical transport services.

Early reperfusion for STEMI

The initial diagnosis, treatment and subsequent outcomes for patients experiencing STEMI are determined by the patient’s location, the level of medical expertise of the first emergency medical contact, and the infrastructure available. Patients with STEMI who present within 12 hours of the onset of ischaemic symptoms should have reperfusion therapy implemented as soon as possible, using either fibrinolytic therapy or PCI.4

Urban and some inner regional populations often have direct access or immediate access through interhospital transfer to primary PCI facilities with first emergency medical contact to PCI delays of less than 90 minutes. Where such access exists, clearly, primary PCI is the preferred modality for reperfusion. Patients in other inner regional, most outer regional and all remote areas will not have such access and require early fibrinolysis (door-to-needle time of less than 30 minutes4) with immediate or early transfer (within 24–48 hours) to a tertiary cardiac facility with revascularisation capability (PCI and/or CABG surgery). For most patients in non-urban settings, the local hospital emergency department will be the most appropriate facility for early STEMI diagnosis and fibrinolysis. However, where inherent delays between first emergency medical contact and access to any facility capable of delivering reperfusion therapy exceed 60 minutes, routine access to prehospital fibrinolysis is strongly recommended.

There are essential infrastructure and standards requirements for prehospital fibrinolysis, either in an ambulance or a remote health clinic (Appendix 5). Currently, in the Northern Territory, remote health clinics provide prehospital fibrinolysis without a doctor being present, and several state ambulance services are effectively providing prehospital fibrinolysis via intensive care paramedics.20

Designated provider clinical networks should be configured to reduce the number of medical transport legs and time delays to tertiary care (including bed access block) to a minimum, given that remote area patients will often unavoidably require up to three transfers (Appendix 3) over very long distances. Historically, this has resulted in tertiary access delays of 5 days or more (own unpublished data; M K I, 2012).

Hospital treatment

As highlighted in the 2006 AIHW and subsequent reports, the inhospital disparities in ACS care experienced by Aboriginal and Torres Strait Islander peoples warrant special consideration by health service providers.7,18

It is well documented that Aboriginal and Torres Strait Islander people suffering from ACS are less likely to undergo angiography in hospital and receive PCI or CABG surgery than non-Indigenous patients.7 Although there has been no extensive study of why these disparities exist, potential reasons include:18

poor health literacy and understanding of invasive procedures
inadequate cultural competence of health care providers and hospitals
bias favouring conservative management based on perceptions of more comorbidities among Aboriginal and Torres Strait Islander patients and lower compliance with medications
disengagement of family members in the decision-making process10
delayed patient transfer to hospitals with PCI facilities compounded by the complexity of the patient journey (highlighted in Appendix 3) in requiring multiple transfers
patient choice.

Difficulties in transferring patients to hospitals with revascularisation facilities may be overcome with improved communication to enhance uptake of these crucial interventions. Patients frequently have a poor understanding and fear of these procedures, and studies indicate that they often feel disengaged from medical staff.8 The support of Indigenous cardiac coordinators (described below) is important in this process.

Other key strategies for improvement, involving Aboriginal health practitioners (where access is available), general hospital staff, retrieval services and the state health departments, include:

establishing a coordinated transfer system as part of the overall designated provider clinical network (fewer steps in the patient pathway will reduce the number of transfers and inherent delays)
providing a system that allows a family member to accompany the patient throughout the continuum of care
developing a patient care plan in conjunction with the patient and his or her family, with involvement of the hospital Aboriginal liaison officer (ALO) and interpreters as needed
commencing patient education in hospital using culturally appropriate educational tools, so that both the patient and his or her family are fully informed of the clinical process and timelines for returning home
ensuring financial and social support to facilitate transfer to other hospitals, even in the urban environment.

Discharge against medical advice

Aboriginal and Torres Strait Islander patients have a much higher rate than other patients of discharge from hospital against medical advice, which increases with remoteness.21 This reflects problems in communication and a lack of engagement with and respect for Aboriginal and Torres Strait Islander patients and their families that contributes significantly to a fear of hospitals and invasive procedures. A lack of culturally competent health care providers accentuates this problem.

Aboriginal and Torres Strait Islander patients and their families need considerable explanation of their diagnosis and proposed procedures to minimise the risk of discharge against medical advice. Patients who have had PCI procedures or CABG surgery should be encouraged to become patient advocates and discuss their story with other patients and family members.

In the urban environment, language is usually not presumed to be an issue (although literacy levels may be22), but for patients referred from regional and remote settings English is likely to be a second language, resulting in additional communication barriers.18 It is essential that interpreters are made available on request and that considerably more time is set aside for discussions with Aboriginal and Torres Strait Islander patients and their families than might be expected for non-Indigenous patients.

Follow-up care, cardiac rehabilitation and secondary prevention

Proven short- and long-term health benefits and reductions in mortality form the basis for recommendations that all patients with ACS be referred for appropriate cardiac rehabilitation.23,24 Unfortunately, rates of participation in cardiac rehabilitation programs by Aboriginal and Torres Strait Islander peoples are extremely low due to extended family responsibilities, sociocultural inappropriateness of programs, poor understanding of cardiac rehabilitation, the connection between colonialism and health services, heart health messages in the media and the younger age of the affected Indigenous population.25

Patients returning home after ACS must have an understanding of their medical condition, and require education regarding medication use, wound care (in the event of recent surgery), risk factor modification, potentially reduced functional capacity, and prognosis. Therefore, patient education must be commenced while in hospital and include culturally appropriate education tools26 and the development of an individualised care plan that provides the basis of the outpatient and maintenance cardiac rehabilitation programs after patients return to their communities (Appendix 6).

The care plan is essential in providing support to the local medical officer and clinics regarding a patient’s:

medications
targeted program for outpatient and maintenance cardiac rehabilitation
overall prognosis
returning to work and personal independence issues (eg, driving).

This requires the treating hospital to have a comprehensive understanding of the services available on the patient’s return home as well as his or her risk-factor profile, home circumstances (specifically, family support, occupation and capacity to return to work) and education and literacy levels. To improve outcomes it is also important to assess the patient’s and family’s understanding of the importance of ongoing cardiac rehabilitation and adherence to an individualised, formalised care plan.

The care plan should be developed with the patient and his or her family in liaison with the Indigenous cardiac coordinator. At discharge, the plan should be forwarded to the appropriate cardiac rehabilitation services nearest to the patient’s home. Follow-up care and outpatient and ongoing maintenance programs should include the elements highlighted in Appendix 7. Appendix 8 outlines key considerations in planning cardiac rehabilitation programs for Indigenous patients, as recommended by National Health and Medical Research Council guidelines.26

The longer-term maintenance of secondary prevention strategies is perhaps the most important element of postacute care for Aboriginal and Torres Strait Islander patients after an ACS, as ineffective strategies result in poorer long-term outcomes among Indigenous patients even when short-term hospital outcomes are similar.11,27 Cardiac coordinators are required to facilitate regular appointments for general and specialist review and investigation (including echocardiography when necessary). In addition, regular coordinated outreach clinics are required to service Aboriginal and Torres Strait Islander populations in urban, regional and remote areas because of inherent difficulties in attending hospital outpatient clinics and private cardiology practices.

Pathway coordination

The ACS continuum-of-care pathway needs coordination, enabling timely access to investigation and treatment and making efficient use of the resources available within that clinical network. Appropriate infrastructure, protocols and policies that are specific to each designated provider clinical network provide the framework for investigations and decision making28 (for requirements, see Appendix 4). However, there is also a need to coordinate patient and escort transfers for coronary procedures and the return home.

Indigenous cardiac coordinators have been trialled in the Northern Territory and at Flinders Medical Centre in South Australia, with significant improvements in patient flow, attendance for procedures and revascularisation rates (unpublished report; Heart Foundation of Australia; as above). Coordinators receive support from ALOs or interpreters as appropriate, as well as administrative support — a key factor in the success of the network. The Indigenous cardiac coordinators:

review patients and have contact with their families within 24 hours of hospital admission
monitor and coordinate patients’ progress before and after diagnosis, including procedures and clinical reviews up to 3 months after an event
liaise with cardiologists, remote health clinics and general practitioners
coordinate transfers of Indigenous cardiac patients, with the relevant patient information, between hospitals, health clinics and other relevant agencies
assess patients returning from tertiary services, including clinical assessment and consideration of social issues
ensure cultural factors are considered when organising patients’ appointments.

The success of the designated provider clinical network requires coordination of patient information from all elements of the network. Electronic transfer of patient records, including referrals, discharge information (to the local medical officer and local cardiac rehabilitation service or equivalents), medications and follow-up care plans, needs to be incorporated, and the development of electronic records will contribute significantly to this process.

Conclusion

To improve the quality of care across the health care continuum for Aboriginal and Torres Strait Islander patients with ACS, a properly structured and clearly defined patient-oriented clinical pathway (comprising adequate human, pharmacological and equipment resources) is essential for the delivery of evidence-based, high-quality care. The generic ACS management framework outlined is designed to provide guidance to policymakers, health planners and health care providers on the establishment of jurisdiction-specific designated provider clinical networks that significantly reduce inappropriate delays to treatment and enhance the uptake of medical services.

Coordinated pathways of care — involving Indigenous cardiac coordinators, facilitated by designated provider clinical networks and supported by ALOs — ensure the following for all Aboriginal and Torres Strait Islander patients, regardless of where they live:

availability of culturally appropriate information regarding warning signs
appropriate inhospital treatment
individualised inpatient care plans developed jointly with the patient and his or her family
education involving culturally appropriate tools within the hospital setting and inclusion of families, with support from ALOs and interpreters as appropriate
adequate follow-up care, secondary prevention programs and specific outpatient cardiac rehabilitation programs.

As the designated provider clinical networks need to be specific for each jurisdiction, they must be developed in collaboration with all involved in the patient pathway. This includes discussions and coordination with patients, families, Aboriginal health practitioners, primary care teams, retrieval services, emergency and cardiology services and health educators. The system should provide adequate cultural support throughout the process so that patients and families are engaged and adequately informed. Although cost-effectiveness has not been investigated in this consensus statement, the recommended framework should offer greater efficiencies in the use of existing resources.

With the advent of the ACS National Goal (announced by the Australian Commission on Safety and Quality in Health Care) and the recently funded Lighthouse Hospital initiative, improved data collection for monitoring effectiveness (including uptake and outcomes) is essential for the successful ongoing management and modification of the networks. The data from the designated provider clinical networks will also provide a more complete understanding of the issues confronting Indigenous patients. However, any delays in establishing the networks will only maintain the current disparities in service delivery and gaps in life expectancy. Therefore, the current lack of data should not delay the establishment of these networks.

With great power comes great responsibility

To the Editor: In October 2013, the Australian Broadcasting Corporation television program Catalyst featured a two-part documentary series entitled Heart of the matter. The first episode questioned the role of dietary saturated fat in the development of heart disease, and the second debated the use of
3-hydroxy-3-methylglutaryl coenzyme
A (HMG-CoA) reductase inhibitors (statins) as a suitable treatment for hypercholesterolaemia.1

Justin Coleman, a general practitioner and senior lecturer in medicine at Griffith University, provided an excellent summary of the two episodes, highlighting the bias of several of the medical experts featured in the program.2 Coleman drew particular attention to their undisclosed conflicts of interest, describing one expert as having his own commercial line of alternative treatments for heart disease. The views portrayed in the program could not have been published in any reputable medical journal without adequate disclosure of the experts’ conflicts of interest. So why should this be allowed on television, when the audience is potentially so much larger and more impressionable?

Perhaps the worst of the views aired was from an expert who suggested that starting statins means weighing up the risk of getting diabetes as a trade-off
for preventing a cardiovascular event. Such an opinion shows ignorance of international standards for scientific evidence — pitting the weak observational data suggesting a slight increase in risk of diabetes among statin users3 against the overwhelmingly strong evidence from multiple randomised controlled trials demonstrating the benefits of statins when used for secondary prevention after an acute coronary syndrome.4 Why are television stations allowed
to broadcast material on so-called scientific programs that includes
such misleading statements?

This program significantly hinders progress in tackling problems such as non-adherence with evidence-based medicines after acute coronary syndromes. It has been demonstrated that patients who stop taking their medications after myocardial infarction have a 10% higher chance of dying within 1 year.5 Resolving this problem requires patient education and empowerment, and a strong doctor–patient relationship — a relationship that is, unfortunately, often tested
by skewed views from the media.

Age, CKD and other kidney messages

Chronic kidney disease in the elderly is common, potentially harmful and amenable to nuanced management

Every March for the past 8 years, the International Society of Nephrology (ISN) and the International Federation of Kidney Foundations have jointly organised World Kidney Day (WKD; http://www.worldkidneyday.org). The purpose of WKD is to increase the awareness of kidney disease among politicians, the general public, general practitioners, physicians, nephrologists and other health care workers. WKD has been taken up with gusto in an increasing number of countries around the world, including Australia, and it is evident that the messages are being heard and to some extent have influenced policy. This year’s WKD will be held on 13 March, around the theme of “Chronic kidney disease (CKD) and aging”. Is this a message as substantive as those of previous WKDs?

Previous messages have been standouts. In 2007, the message was that kidney disease is common (affecting 10% of adults worldwide), harmful (not only from complications of CKD and end-stage kidney disease [ESKD], but also from a substantially increased risk of premature death, especially from cardiovascular causes) and treatable.1 In 2009 and 2010, the nexus between kidney disease and hypertension and diabetes, respectively, was stressed. Hypertension is a major risk factor for CKD and a key therapeutic target.2 The prediction that there will be 1.5 billion hypertensive people on our planet by 20252 is indeed a sobering one! Diabetes, especially type 2, is now the commonest cause of CKD and ESKD in most countries3 — and things are only going to get worse before they get better. By 2025, there will be almost 400 million people worldwide with type 2 diabetes3 and a similar number with impaired glucose tolerance, many of whom will go on to develop CKD. CKD, especially in the presence of proteinuria, is a principal risk factor for cardiovascular disease.4 Cardiovascular risk rises proportionally as kidney function declines; for example, Stage 3 CKD independently carries an almost 50% increased risk of cardiac death, a risk greater than that of diabetes or previous cardiovascular disease. Considering all stages of CKD together, there is a 100-fold greater chance of premature death (especially cardiovascular) than of developing ESKD. Importantly, interventions designed to slow CKD progression and reduce proteinuria appear also to reduce cardiovascular risk.

What began as a risky experiment about 50 years ago has now become routine practice in more than 80 countries around the world; there is no doubt at all that renal transplantation is the best way to replace kidney function in patients with ESKD.5 But, currently, only 10% of global need for kidney transplantation is met, restricted by economic considerations, insufficient donors, too small a trained workforce, immunological barriers and ethical considerations.5 Last year’s WKD focused on acute kidney failure (AKF). The prevalence of AKF is increasing worldwide and most cases are preventable,6 yet prevention and dialytic support during AKF are pipedreams for many countries. In response, the ISN has launched an ambitious program called “0 by 25” with the lofty aim that by 2025 no one should die of untreated AKF in the poorest parts of Africa, Asia and South America.

So is 2014’s WKD message as weighty as its antecedents (Box)? The latest message concerns CKD and ageing.7 Just like the body that surrounds them, kidneys age; a contemporary question is whether this ageing represents progressive CKD. Another key question is whether the automatic reporting of estimated glomerular filtration rate (eGFR) has spawned an epidemic of worried but well elderly people. The answer to the second question is yes and no. Otherwise healthy elderly people with a mild reduction in eGFR are likely to see out their years without any increased risk. However, whatever the cause of impaired eGFR, especially in the presence of proteinuria and with a rate of decline more rapid than expected for age alone, there will be increased risk.8 Despite their age, elderly patients with CKD will benefit from therapies aimed at slowing disease progression, better controlling metabolic derangements, reducing cardiovascular complications and allowing informed choices about ESKD therapy. That greater life expectancy does not necessarily equate with increased years of good health probably applies more to patients with CKD than any other ageing population. But treatment of selected older patients can increase survival, whether that treatment is conservative, dialytic or by transplantation. Older patients have less chance of undergoing transplantation, usually because of their comorbidities, but they can respond well, and various strategies (such as use of marginal and older donors) have increased the donor pool. Older patients with limited comorbidities can do well on dialysis, with improved survival; yet those with comorbidities may do better with non-dialytic, conservative therapy. So messages about CKD are just as important for older patients, and their wise application among older patients may do more for global health than among younger age groups.

What should developed countries be doing to lessen the global burden of kidney disease? Many developed countries must focus on their own groups at high risk of kidney disease, such as the Aboriginal population in Australia. In addition, they can play a key role in helping low-to-middle income countries tackle their kidney disease burden. The ISN directs a large proportion of its budget and efforts towards transformational capacity-building programs, including its Fellowship,9 Sister Renal Centre and other programs.10,11 By an increasing contribution to these programs, Australia can play a key role in the global response to kidney disease.

Important global messages* about kidney disease

2007	CKD is common, harmful and treatable
2009	Hypertension is a major risk factor for CKD
2010	Diabetes is a major risk factor for CKD
2011	CKD is a principal risk factor for cardiovascular disease
2012	Transplantation is the best therapy for ESKD, but is in short supply
2013	AKF is common and preventable
2014	CKD in the elderly is common, and amenable to discerning management

* Messages of World Kidney Day. CKD = chronic kidney disease. ESKD = end-stage kidney disease. AKF = acute kidney failure.

Renal sympathetic denervation for resistant hypertension in a patient with a single kidney

To the Editor: We report a case
of successful renal sympathetic denervation (RSD) treatment for resistant hypertension in a patient with a single kidney.1–3 RSD is performed via femoral arterial access, using a radiofrequency ablation catheter to deliver energy
to the renal artery wall. When delivering RSD to a single kidney, potential renal artery vascular complications can have a significant negative impact on a patient’s renal function, compared with patients with two functioning kidneys.

Our patient was a 71-year-old man with resistant hypertension for many years. He had a congenitally absent left kidney. He was on
five antihypertensive agents: candesartan/hydrochlorothiazide
32/12.5 mg daily, lercanidipine 20 mg daily, atenolol 50 mg daily, moxonidine 0.4 mg daily and prazosin 0.5 mg twice daily. Despite this, his blood pressure was poorly controlled, with systolic blood pressure readings frequently above 200 mmHg. Renal ultrasound demonstrated a normal right kidney with normal renal artery, and the
left kidney could not be identified.
A computed tomography renal angiogram confirmed a single right renal artery with no plaque or stenosis. Other secondary causes
of hypertension had been excluded. Given refractory hypertension on medical therapy and suitable anatomy on imaging, the patient underwent RSD treatment to his right renal artery in 2012. Because
of its exceptional length, 12 ablations were delivered along
the artery.

On the day of the procedure, the patient’s sitting blood pressure was 180/76 mmHg. At 1-year follow-up, his sitting blood pressure was 155/66 mmHg. To date, our patient has benefited from sustained blood pressure reduction after RSD, with maintenance of his renal function (Box). He has continued on the same oral antihypertensive regimen.

To our knowledge, this is the second report of RSD for resistant hypertension in a patient with a single kidney.4 The previous report was limited by a follow-up period of only 3 months.4 The success of our case demonstrates the role of RSD
as an effective and well tolerated treatment for resistant hypertension, with an enduring result up to 12 months, even in patients with a single kidney, when it is performed at an expert centre.

Blood pressure and renal function immediately before renal sympathetic denervation (RSD) and at follow-up

eGFR = estimated glomerular filtration rate.

The cat and the nap

A patient’s apnoea is discovered by his “owner”

We report the case of a 72-year-old man who presented to his general practitioner with cat scratch — not cat-scratch disease, but trauma to the face and nose caused by repeated savage night-time attacks perpetrated by none other than his trusty loyal cat. The patient had a history of stable coronary artery disease, type 2 diabetes mellitus, diabetic neuropathy and hypertension.

Why the cat would be doing this puzzled his GP, who concluded that perhaps the cat was witnessing something which it deemed required intervention. The GP subsequently requested overnight polysomnographic assessment. This revealed moderate obstructive sleep apnoea (OSA) with an apnoea–hypopnoea index of 30, and bradycardia with 7-second cardiac pauses. Although 7-second cardiac pauses do not normally require cardiopulmonary resuscitation, the patient’s cat rushed in, knowing no better, to perform C(at)PR. Biting the nose that sneezes at you is not normally a recipe for success, but in this case it appears that the patient has had nine lives thanks to his cat. Happily, at routine follow-up after starting treatment with continuous positive airway pressure, the patient reported that the cat was no longer traumatising his face.

It is not unusual in a patient with these comorbidities to have both OSA and bradycardia with cardiac pauses,1 but what makes the case fascinating is the fact that sleep apnoea can trigger cardiac pauses.1 This raises the possibility of a unifying hypothesis — the cat was responding to and intervening in the patient’s apnoeic and asystolic episodes.

OSA has been causally related to cardiac arrhythmias and sudden cardiac death. Several mechanisms seem to underpin the association between OSA and cardiac arrhythmias,2 including intermittent hypoxia associated with autonomic nervous system activation, alteration in myocardial excitability, recurrent arousals with sympathetic activation and increased negative intrathoracic pressure which may mechanically stretch myocardial walls. There is a high prevalence of OSA in patients with cardiac arrhythmias.

So it appears that this cat, although unable to identify the exact cause of the apnoeic and asystolic episodes, was aware of the patient’s ill health and impending doom. Perhaps we should not be surprised by this, given the anecdotal stories that appear in the popular press from time to time — for example, stories of cats being “aware” of a woman’s pregnancy.

How could this all work? Animals live in a sensory world that is very different to our primarily visual world. While dogs have been shown to be able to detect various forms of human cancers,3–5 cats can detect smells and vapours that humans cannot detect.

In times of economic austerity, at least in Europe, let us commend this “natural” intervention. We hope that guideline groups will take note of this case and recommend the prescription of felines to patients at risk of OSA rather than home oximetry.

Tachycardia of unknown Origin

This is the first published case of onset of tachycardia despite triple rate-controlling medication during a rugby league match. The case highlights the importance of a thorough history to exclude Origin-induced tachycardia.

Clinical record

A 40-year-old Queensland man on continuous telemetry for decompensated idiopathic dilated cardiomyopathy following explantation of an infected implantable cardiodefibrillator was noted to have abnormal tracing (Box) between 19:00 and 22:00 on 4 July 2012. The patient was well, symptom-free and in excellent spirits. His medication included digoxin 250 μg daily, amiodarone 200 mg daily and bisoprolol 7.5 mg daily, with a resting heart rate of 60 beats per minute.

Telemetry showed asymptomatic variable persistent sinus tachycardia over a 3-hour period on the previous evening.

Further history revealed that the patient had been watching the deciding match of the annual State of Origin rugby league series between Queensland and New South Wales on television. The Box shows the onset of the tachycardia at the start of the match coverage at 19:00, with a second peak at kick-off (20:15). The recorded rhythm was sinus with left bundle branch block (QRS duration, 140 ms). The sinus tachycardia persisted throughout the first half until the half-time interlude, when normocardia resumed. The tachycardia reoccurred at the start of the second half, reaching a peak in the dying minutes of the game, when the patient’s home state, Queensland, scored a field goal at 75 minutes — followed by a missed long-range field goal by the NSW halfback in the final minute of the game — securing victory by one point for Queensland (21:55).

Discussion

This case illustrates a sustained run of tachycardia in a continuously monitored inpatient with cardiomyopathy, which terminated itself after trying circumstances and which occurred despite multiple rate-limiting medications.

Had the patient’s defibrillator still been in place, it mercifully would not have been activated, as his defibrillation threshold was set at 180 beats per minute. Origin-induced tachycardia should be a differential diagnosis for asymptomatic sinus tachycardia in passionate Queensland rugby league supporters, whose only cure when watching televised coverage is to turn off their televisions.

Cardiac telemetry trace

Sotalol-associated cardiogenic shock in a patient with asymptomatic transient rate-related cardiomyopathy

To the Editor: An 80 kg, 73-year-old man with type 2 diabetes managed with gliclazide and metformin was discharged on a dose of 40 mg twice daily of sotalol and a therapeutic dose
of warfarin after an admission for
atrial flutter. Transoesophageal echocardiography during his admission showed normal left ventricular (LV) size with mild to moderate global systolic dysfunction and moderate mitral incompetence.

Two weeks later, he developed generalised weakness, malaise, diaphoresis, nausea and dull left-sided chest pain, progressing to severe shock within 45 minutes of onset, evident on arrival at a hospital emergency department. Two hours before onset, he had taken a planned increase in his oral dose of sotalol to 80 mg.

His systolic blood pressure was 74 mmHg without evidence of respiratory compromise. Results of an arterial blood gas analysis were consistent with severe tissue hypoperfusion. Electrocardiography showed a sinus rhythm of 60 beats
per minute, normal conduction, a lengthened QTc interval (510 ms), and anterolateral T-wave inversion without ST-segment abnormalities. Results of bedside ultrasonography suggested global LV dysfunction.

The patient was given 2 litres of fluids and high doses of noradrenaline and adrenaline intravenously, but his condition did not improve. Intravenous calcium chloride (10 mmol) and glucagon (2 mg) restored his blood pressure to 120/70 mmHg, maintained with adrenaline (5 μg/min) alone. The initial relative bradycardia, prolonged QTc interval and response to calcium and glucagon were consistent with cardiogenic shock secondary to sotalol therapy. Other investigations excluded circulatory, cardiac, pulmonary, infective and drug causes of sudden cardiovascular collapse. The patient’s initial mild to moderate LV systolic dysfunction abated after adequate rate control, consistent
with a transient rate-related cardiomyopathy. Echocardiography showed normal LV function 2 months after discharge.

This is the first reported case of acute cardiogenic shock associated with low-dose sotalol alone (note that therapeutic doses range up to 640 mg/day). Cardiogenic shock has been reported in a patient taking a combination of sotalol and diltiazem,1 and in another patient taking digoxin, sotalol and verapamil.2 I have seen a similar case of reversible life-threatening cardiovascular collapse after sotalol (40 mg) was given orally to a 26-year-old with known atrial fibrillation. In that case, non-compliance with digoxin therapy resulted in an unrecognised rate-related cardiomyopathy.

As with all β-blockers, the official prescribing information for sotalol
in 2013 cautions about use in patients with known LV dysfunction, but does not warn of the risk of
life-threatening cardiogenic shock.
It is contraindicated only in “uncontrolled congestive heart failure”. “Hypo-tension” is considered a side effect.3

Research is indicated to determine whether rate-related cardiomyopathy is an independent risk factor for sotalol-induced cardiogenic shock and/or sudden death. Prescribing information for commencing sotalol or increasing the dose may need to be amended to warn of the risk of cardiogenic shock in this subset of patients.

Arrhythmia management distilled

The authors of this book work in Australia, the United Kingdom and Canada, and are well known to and respected by many Australian cardiologists working in the field of cardiac arrhythmia. The first edition was published in 2010. In view of the rapid advances in arrhythmia management, particularly the new medication and guidelines for management of atrial fibrillation (AF), as well as in catheter ablation, the authors considered a second edition warranted.

The target audience includes general practitioners, nurses, medical students, technicians and cardiology trainees. The layout is as simple as possible for a very complex subject. As is to be expected, because of its prevalence, AF is given prominence. The use of two important guidelines for assessing risk of thromboembolism and bleeding in patients with AF is emphasised: the CHA2DS2-VASc (congestive heart failure, hypertension, age >75 years [doubled], diabetes mellitus, stroke [doubled], vascular disease, age 65–74 years, sex category) score; and the HAS-BLED (hypertension, abnormal renal/liver function, stroke, bleeding history or predisposition, labile international normalised ratio, elderly, drugs/alcohol concomitantly) score.

Some antiarrhythmic drugs mentioned are unavailable in Australia, and the newer oral anticoagulants currently have only limited availability here. Hence, it is a little difficult to strictly follow the European and North American guidelines.

The vast majority of the information provided is correct; however, there are a few points with which I disagree. For example, lidocaine (lignocaine) is stated as having only minor negative inotropy, but experiments by Professor Robert McRitchie (personal communication) have shown it to be one of the most powerful negative inotropes of all antiarrhythmics.

In my opinion, the book is a concentrated distillation of the major points in arrhythmia management, and is potentially very useful for the target audience; it is certainly good value for money.

Utility of auscultatory screening for detecting rheumatic heart disease in high-risk children in Australia’s Northern Territory

Rheumatic heart disease (RHD), the long-term sequel of acute rheumatic fever, is a leading cause of heart disease in children in low- and middle-income countries.1 Poverty and overcrowding are known risk factors for RHD,2 and with improvements in socioeconomic conditions, the disease has essentially disappeared from industrialised countries, with the exceptions of the Indigenous populations of Australia and New Zealand.3 Indigenous Australians continue to experience among the highest rates in the world, with an acute rheumatic fever incidence of up to 380 per 100 000 children aged 5–14 years, and an estimated RHD prevalence of 8.5 per 1000 children in this age group.4 A recent government report shows that young Indigenous Australians (< 35 years) in the Northern Territory have a 122-fold greater prevalence of RHD than non-Indigenous Australians.5

In populations with high prevalence, RHD satisfies many of the criteria for a disease to be deemed suitable for screening,6 and RHD has long been a target of public health screening internationally. Cardiac auscultation was the traditional approach,7 but with the evolution of portable echocardiography there has been increasing interest in echocardiographic screening for RHD.8–15 In the echocardiographic era, a new category of RHD has been recognised: “subclinical RHD”, defined as structural or functional changes consistent with RHD evident on an echocardiogram in the absence of a pathological cardiac murmur.6 By definition, it is not possible to identify children who have subclinical RHD using auscultatory screening alone, and published data consistently show that auscultation is considerably less sensitive than echocardiography, missing up to 90% of cases of RHD in some studies.8 Also of concern is the high false-positive rate associated with auscultation, resulting in many children undergoing further unnecessary diagnostic evaluation.9,16

Auscultatory screening for RHD commenced in the NT in 1997 and is still used today. Cardiac auscultation is performed by primary care doctors on schoolchildren aged 10 and 15 years who live in remote Indigenous communities; those with a cardiac murmur are referred for echocardiography.17 The NT is the only jurisdiction in Australia with a formal RHD screening program.

As part of a large echocardiographic screening study undertaken in northern Australia, we performed cardiac auscultation on a subset of schoolchildren in remote Indigenous communities in the NT and compared clinical findings with echocardiographic findings. We aimed to establish whether cardiac auscultation is an appropriate tool for RHD screening to identify children who should be referred for echocardiography.

Methods

Setting and participants

Our study was conducted in 12 rural and remote communities in Central Australia and the Top End of the NT between September 2008 and June 2010. Children aged 5–15 years, identified by school enrolment records, were eligible to participate. These children were a subset of a larger group of children, from 17 communities in Northern Australia, who had echocardiography performed for a larger study. Nurse and doctor auscultators were present during visits to the 12 communities, and all the children in these communities who were participating in the larger study were eligible to participate in the auscultation component.

Written informed consent was obtained from parents and guardians, and written assent was obtained from children aged ≥ 13 years before they took part. Ethics approval was obtained from the Human Research Ethics Committee of the Northern Territory Department of Health and Community Services, and the Central Australian Human Research Ethics Committee.

Echocardiography

All children had a screening echocardiogram performed by an experienced cardiac sonographer using a Vivid e (GE Healthcare) portable cardiovascular ultrasound machine. Sonographers were blinded to the auscultators’ findings and to the clinical history of the children. Screening echocardiograms were performed according to an abbreviated protocol, previously used in Tonga and Fiji,9,16 that focused on the mitral and aortic valves, but would also enable detection of significant congenital lesions. If a potential abnormality was detected, a complete echocardiogram was performed.

Echocardiograms were recorded to DVD and reported offsite by a pool of 14 cardiologists who were blinded to the clinical findings. Detailed data about the mitral and aortic valves were entered into an electronic database.

Children were classified as having definite or borderline RHD according to the 2012 World Heart Federation (WHF) criteria for the echocardiographic diagnosis of RHD.18 This was done by extracting each individual echocardiographic feature, as objectively measured and recorded by reporting cardiologists, and combining features to determine whether WHF definitions were met. Children were also classified as having pathological mitral regurgitation or pathological aortic regurgitation according to these criteria.

Cardiac auscultation

Children underwent auscultation performed by a nurse and a doctor who were blinded to the sonographers’ findings, each others’ findings and to the clinical history of the children. Auscultation was performed by nurses with varying levels of experience and doctors of different specialties (including general practitioners, paediatricians and cardiologists). It was completed with children supine and sitting, in a quiet room where possible. The diaphragm and bell of the stethoscope were used at the apex and axilla, lower left sternal edge, upper left sternal edge and upper right sternal edge. The nurses and doctors who performed auscultation were asked to comment on the presence or absence of a murmur. The doctors were further asked to specify whether a murmur was “innocent”, “suspicious” or “pathological”. Suspicious and pathological murmurs were classified as “significant” murmurs. This enabled assessment of three screening approaches: one-stage auscultation by a nurse to detect any murmur; one-stage auscultation by a doctor to detect any significant murmur; and two-stage auscultation, with the first stage to detect any murmur by a nurse and the second stage to detect which of these was significant by a doctor.

Analysis

Statistical analysis was performed using Stata statistical package version 12.1 (StataCorp). Sensitivity, specificity, positive predictive value (PPV) and negative predictive value (NPV) were calculated for each screening approach.

Results

A total of 1986 NT children had a screening echocardiogram as part of the larger study, of whom 1015 had auscultation performed by a doctor and a nurse; 960 (94.6%) were Indigenous and 498 were girls (49.1%). The mean age was 9.3 years (SD, 2.5 years), and the median body mass index was 15.6 kg/m2 (interquartile range, 14.4–17.8 kg/m2). Children who had an echocardiogram but did not undergo auscultation were slightly older (mean age, 9.7 years), but were otherwise comparable based on sex and body mass index.

Echocardiographic findings

Thirty-four children (3.3%) had abnormalities identified on their echocardiogram. Fifteen (1.5%) of them had definite RHD, 9 (0.9%) had borderline RHD (including two who also had small atrial septal defects), and 10 (1.0%) had isolated congenital anomalies: ventricular septal defect (two), atrial septal defect (one), mitral valve prolapse (two), patent ductus arteriosus (two), dilated aortic root (two) and complex congenital heart disease (one). Of the 24 children with RHD, 14 had pathological mitral regurgitation, six had pathological aortic regurgitation, and one child had both.

Clinical findings

One-stage auscultation

A cardiac murmur (significant or not) was heard by nurses in 263 children (25.9%), by doctors in 257 children (25.3%), and by a doctor and a nurse in 137 children (13.5%). Compared with echocardiogram, one-stage auscultation to detect any murmur by a doctor or a nurse had a sensitivity of less than 50%, a specificity of about 75%, and a positive predictive value (PPV) of less than 10% (Box 1). Asking doctors to decide which murmurs were pathological or suspicious increased the specificity from 75.1% to 92.2%, but further dropped the sensitivity to 20.6%. The breakdown of medical specialists and their auscultation findings are presented in Box 2.

Two-stage auscultation

Only 52% (137/263) of the murmurs heard by nurses were also heard by doctors. Of these, 57 were considered pathological or suspicious. Using two-stage auscultation, 28 children with abnormalities were missed (sensitivity, 17.6%), and six children with abnormalities were correctly identified (PPV, 10.5%). This approach had a specificity of 94.8%.

Discussion

Our study confirms that cardiac auscultation has poor sensitivity, despite moderately high specificity, for detecting RHD and other cardiac abnormalities evident on echocardiograms, regardless of the experience of the examiner. More than 50% of children with abnormal echocardiography results did not have a murmur detected, and more than 90% of murmurs heard were false positives. The observed high NPVs and low PPVs are expected in a low-prevalence disease such as RHD, and are consistent with the results of previous studies (Box 3). Our findings highlight the paramount importance of sensitivity in determining the utility of auscultation as a screening test for RHD.

The current approach to screening for RHD in the NT is one-stage doctor auscultation by a GP, with referral of any child with a murmur for an echocardiogram.17 Program reports suggest that cardiac murmurs are heard in about 10% of those screened,19 but few data regarding follow-up and clinical outcomes for these children are available. In a detailed report on RHD screening in Central Australia during 2009, 67 of 1095 children who were screened (6.1%) had a murmur and were referred for echocardiography. One year later, only 38 of them had had their echocardiogram, of whom four had abnormalities (two RHD, two non-RHD abnormalities).19 This prevalence of RHD (2 per 1000 children) is considerably lower than expected in the Central Australian population and suggests that some disease went undetected. In addition, the fact that nearly half of referred children had not had their echocardiogram 12 months later also highlights difficulties with the current approach.

According to the current NT screening model (one-stage doctor auscultation), 257 children in our study would have been referred for echocardiogram, with only 13 of them having abnormalities (eight with RHD, five with congenital heart disease). A high false-positive rate has important implications for screening programs, to both the individual and the health system. In the NT, limited paediatric cardiology services exist, and waiting times for echocardiography can be long. Such high false-positive rates would result in a substantial increase in referral of children to tertiary services for further evaluation, and would risk overburdening already-stretched paediatric cardiology services with children who do not have heart disease.

Of greatest concern, however, is that using the current approach to RHD screening, 16 of 24 children with RHD (10 with definite RHD, six with borderline RHD) would have been missed. While there is uncertainty about the significance of the borderline RHD category, the WHF recommends that all children meeting echocardiographic criteria for definite RHD be started on secondary prophylaxis.18 In our study, the 10 children who met these criteria but did not have murmur detected by one-stage doctor auscultation would not have had further evaluation and would not have commenced secondary antibiotic prophylaxis, leaving them at high risk of acute rheumatic fever recurrences and further valve damage.

The prognosis of RHD is best if secondary prophylaxis with long-acting intramuscular penicillin is commenced when the disease is mild; continuous adherence to treatment with penicillin can result in valve damage being halted or reversed.20–22 It is therefore imperative that the test used to screen for RHD is highly sensitive, so that children with the earliest stage of disease, who stand to gain the most from the only currently available preventive treatment, are identified.

It is widely accepted that echocardiography is more sensitive than auscultation. While there has been much discussion about echocardiographic definitions of RHD, including concerns about specificity, it is hoped that the publication of the WHF diagnostic criteria will minimise false-positive results. Whether echocardiographic screening for RHD is appropriate, feasible and cost-effective will vary between settings, and remains a topic of vigorous debate.6,23–25 A cost-effectiveness analysis of our data is underway and will contribute to our ultimate recommendations about the future of echocardiographic screening in Indigenous Australian children who are at high risk of RHD.

A limitation of this study is that auscultation was carried out by several different doctors and nurses, potentially leading to high interobserver variation. Similarly, the screening environment varied between communities, and the conditions under which auscultation was performed (eg, in a quiet room) were not the same for all participants. However, we believe that these limitations reflect the day-to-day reality of health care service provision in the participating communities, allowing valid extrapolation of our results to the current school screening procedure in the NT and many other settings.

We conclude that cardiac auscultation is not an effective method of RHD screening, regardless of the expertise of the auscultator. The risk of missing more than 50% of children with RHD, and the risk of overburdening cardiology services with false positives, preclude recommendation of one-stage or two-stage auscultation as a rational approach to RHD screening. We recommend that cardiac auscultation no longer be used to screen for RHD in high-risk schoolchildren in Australia.

1 Comparison of auscultation findings with echocardiographic findings for 1015 children from rural and remote parts of the Northern Territory,
2008–2010

Auscultation approach

No. of children
with abnormalities* (n = 34)

No. of children
without abnormalities
(n = 981)

Sensitivity
(95% CI)

Specificity
(95% CI)

PPV
(95% CI)

NPV
(95% CI)

AUC†
(95% CI)

One stage, by nurse

Any murmur

247

47.1%
(29.8%–64.9%)

74.8%
(72.0%–77.5%)

6.1%
(3.5%–9.7%)

97.6%
(96.2%–98.6%)

0.61
(0.52–0.70)

No murmur

734

One stage, by doctor

Any murmur

13‡

244

38.2%
(22.2%–56.4%)

75.1%
(72.3%–77.8%)

5.1%
(2.7%–8.5%)

97.2%
(95.8%–98.3%)

0.57
(0.48–0.65)

No murmur

21§

737

One stage, by doctor

Significant murmur¶

20.6%
(8.7%–37.9%)

92.2%
(90.3%–93.8%)

8.3%
(3.4%–16.4%)

97.1%
(95.8%–98.1%)

0.56
(0.49–0.63)

No significant murmur

904

Two stage**

Significant murmur

17.6%
(6.8%–34.5%)

94.8%
(93.2%–96.1%)

10.5%
(4.0%–21.5%)

97.1%
(95.8%–98.1%)

0.56
(0.50–0.63)

No significant murmur

930

PPV = positive predictive value. NPV = negative predictive value. AUC = area under the receiver operating characteristic curve. * Definite or borderline rheumatic heart disease and congenital abnormalities detected on echocardiogram; there was no difference in the findings when only definite rheumatic heart disease and congenital abnormalities were considered true cases (data not shown). † AUC is a measure of overall test accuracy; 0.5 indicates zero discrimination, and values approaching 1.0 indicate high sensitivity and specificity. ‡ Includes 8 children with rheumatic heart disease (5 definite, 3 borderline) and 5 with congenital heart disease. § Includes 16 children with rheumatic heart disease (10 definite, 6 borderline) and 5 with congenital heart disease. ¶ Includes 20 pathological and 64 suspicious cardiac murmurs. ** By a nurse to identify any murmur, then by a doctor to identify significant murmur.

2 Comparison of one-stage doctor auscultation findings with echocardiographic findings, by specialty of doctors who performed auscultation, for children from rural and remote parts of the Northern Territory, 2008–2010

	No. of children who underwent auscultation	No. of children with abnormalities*	No. (%) of children with any murmur	No. (%) of children with significant murmur	Sensitivity†	Specificity†

General practitioner	157	8	33 (21.0%)	14 (8.9%)	12.5%	91.3%
Paediatrician	637	17	159 (25.0%)	48 (7.5%)	17.7%	92.7%
Cardiologist	106	4	37 (34.9%)	2 (1.9%)	0	98.0%
Physician	45	2	14 (31.1%)	7 (15.6%)	100.0%	88.4%
Resident medical officer	70	3	14 (20.0%)	13 (18.6%)	33.3%	82.1%
Any doctor	1015	34	257 (25.3%)	84 (8.3%)	20.6%	92.2%

* Definite or borderline rheumatic heart disease and congenital abnormalities detected on echocardiogram. † Comparison of doctor identification of significant cardiac murmur with any abnormality detected on echocardiogram.

3 Comparison of auscultation findings with echocardiographic findings in three large rheumatic heart disease screening studies

	Country (auscultator)
	Mozambique (physician)8	Tonga (medical student)9	Tonga (paediatrician)9	Fiji (paediatrician)16

No. of children who underwent auscultation	2170	980	980	3462
No. of children who underwent echocardiography	2170	980	980	331
No. of children with abnormalities detected on echocardiogram	71	140	140	41
No. (%) of children with any murmur	456 (21%)	964 (98%)	779 (79%)	889 (26%)
No. (%) of children with significant murmur	91 (4%)	NA	358 (37%)	359 (10%)
Sensitivity*	14%	96%	46%	NA
Specificity*	96%	1%	65%	NA
Positive predictive value*	11%	14%	18%	14%
Negative predictive value*	97%	69%	88%	NA

NA = not applicable. * Comparison of significant murmurs (where reported) with any abnormality (rheumatic heart disease and congenital heart disease) detected on echocardiogram; echocardiographic definitions of rheumatic heart disease varied slightly between studies.