The high prevalence and impact of rheumatic heart disease in pregnancy in First Nations populations in a high-income setting: a prospective cohort study.

OBJECTIVE: To describe the epidemiology of rheumatic heart disease (RHD) in pregnancy in Australia and New Zealand (A&NZ). DESIGN: Prospective population-based study. SETTING: Hospital-based maternity units throughout A&NZ. POPULATION: Pregnant women with RHD with a birth outcome of ≥20 weeks of gestation between January 2013 and December 2014. METHODS: We identified eligible women using the Australasian Maternity Outcomes Surveillance System (AMOSS). De-identified antenatal, perinatal and postnatal data were collected and analysed. MAIN OUTCOME MEASURES: Prevalence of RHD in pregnancy. Perinatal morbidity and mortality. RESULTS: There were 311 pregnancies associated with women with RHD (4.3/10 000 women giving birth, 95% CI 3.9-4.8). In Australia, 78% were Aboriginal or Torres Strait Islander (60.4/10 000, 95% CI 50.7-70.0), while in New Zealand 90% were Maori or Pasifika (27.2/10 000, 95% CI 22.0-32.3). One woman (0.3%) died and one in ten was admitted to coronary or intensive care units postpartum. There were 314 births with seven stillbirths (22.3/1000 births) and two neonatal deaths (6.5/1000 births). Sixty-six (21%) live-born babies were preterm and one in three was admitted to neonatal intensive care or special care units. CONCLUSION: Rheumatic heart disease in pregnancy persists in disadvantaged First Nations populations in A&NZ. It is associated with significant cardiac and perinatal morbidity. Preconception planning and counselling and RHD screening in at-risk pregnant women are essential for good maternal and baby outcomes. TWEETABLE ABSTRACT: Rheumatic heart disease in pregnancy persists in First Nations people in Australia and New Zealand and is associated with major cardiac and perinatal morbidity.

Hyperendemic rheumatic heart disease in a remote Australian town identified by echocardiographic screening.

OBJECTIVES: Using echocardiographic screening, to estimate the prevalence of rheumatic heart disease (RHD) in a remote Northern Territory town. DESIGN: Prospective, cross-sectional echocardiographic screening study; results compared with data from the NT rheumatic heart disease register. SETTING, PARTICIPANTS: People aged 5-20 years living in Maningrida, West Arnhem Land (population, 2610, including 2366 Indigenous Australians), March 2018 and November 2018. INTERVENTION: Echocardiographic screening for RHD by an expert cardiologist or cardiac sonographer. MAIN OUTCOME MEASURES: Definite or borderline RHD, based on World Heart Federation criteria; history of acute rheumatic fever (ARF), based on Australian guidelines for diagnosing ARF. RESULTS: The screening participation rate was 72%. The median age of the 613 participants was 11 years (interquartile range, 8-14 years); 298 (49%) were girls or women, and 592 (97%) were Aboriginal Australians. Definite RHD was detected in 32 screened participants (5.2%), including 20 not previously diagnosed with RHD; in five new cases, RHD was classified as severe, and three of the participants involved required cardiac surgery. Borderline RHD was diagnosed in 17 participants (2.8%). According to NT RHD register data at the end of the study period, 88 of 849 people in Maningrida and the surrounding homelands aged 5-20 years (10%) were receiving secondary prophylaxis following diagnoses of definite RHD or definite or probable ARF. CONCLUSION: Passive case finding for ARF and RHD is inadequate in some remote Australian communities with a very high burden of RHD, placing children and young people with undetected RHD at great risk of poor health outcomes. Active case finding by regular echocardiographic screening is required in such areas.

Rheumatic heart disease in pregnancy: How can health services adapt to the needs of Indigenous women? A qualitative study.

OBJECTIVES: To study rheumatic heart disease health literacy and its impact on pregnancy, and to identify how health services could more effectively meet the needs of pregnant women with rheumatic heart disease. MATERIALS AND METHODS: Researchers observed and interviewed a small number of Aboriginal women and their families during pregnancy, childbirth and postpartum as they interacted with the health system. An Aboriginal Yarning method of relationship building over time, participant observations and interviews with Aboriginal women were used in the study. The settings were urban, island and remote communities across the Northern Territory. Women were followed interstate if they were transferred during pregnancy. The participants were pregnant women and their families. We relied on participants' abilities to tell their own experiences so that researchers could interpret their understanding and perspective of rheumatic heart disease. RESULTS: Aboriginal women and their families rarely had rheumatic heart disease explained appropriately by health staff and therefore lacked understanding of the severity of their illness and its implications for childbearing. Health directives in written and spoken English with assumed biomedical knowledge were confusing and of limited use when delivered without interpreters or culturally appropriate health supports. CONCLUSIONS: Despite previous studies documenting poor communication and culturally inadequate care, health systems did not meet the needs of pregnant Aboriginal women with rheumatic heart disease. Language-appropriate health education that promotes a shared understanding should be relevant to the gender, life-stage and social context of women with rheumatic heart disease.

A review of outcome following valve surgery for rheumatic heart disease in Australia.

BACKGROUND: Globally, rheumatic heart disease (RHD) remains an important cause of heart disease. In Australia it particularly affects younger Indigenous and older non-Indigenous Australians. Despite its impact there is limited understanding of the factors influencing outcome following surgery for RHD. METHODS: The Australian and New Zealand Society of Cardiac and Thoracic Surgeons Cardiac Surgery Database was analysed to assess outcomes following surgical procedures for RHD and non-RHD valvular disease. The association with demographics, co-morbidities, pre-operative status, valve(s) affected and operative procedure was evaluated. RESULTS: Outcome of 1384 RHD and 15843 non-RHD valve procedures was analysed. RHD patients had longer ventilation, experienced fewer strokes and had more readmissions to hospital and anticoagulant complications. Mortality following RHD surgery at 30 days was 3.1% (95% CI 2.2 - 4.3), 5 years 15.3% (11.7 - 19.5) and 10 years 25.0% (10.7 - 44.9). Mortality following non-RHD surgery at 30 days was 4.3% (95% CI 3.9 - 4.6), 5 years 17.6% (16.4 - 18.9) and 10 years 39.4% (33.0 - 46.1). Factors independently associated with poorer longer term survival following RHD surgery included older age (OR1.03/additional year, 95% CI 1.01 - 1.05), concomitant diabetes (OR 1.7, 95% CI 1.1 - 2.5) and chronic kidney disease (1.9, 1.2 - 2.9), longer invasive ventilation time (OR 1.7 if greater than median value, 1.1- 2.9) and prolonged stay in hospital (1.02/additional day, 1.01 - 1.03). Survival in Indigenous Australians was comparable to that seen in non-Indigenous Australians. CONCLUSION: In a large prospective cohort study we have demonstrated survival following RHD valve surgery in Australia is comparable to earlier studies. Patients with diabetes and chronic kidney disease, were at particular risk of poorer long-term survival. Unlike earlier studies we did not find pre-existing atrial fibrillation, being an Indigenous Australian or the nature of the underlying valve lesion were independent predictors of survival.

Acute rheumatic fever in First Nations communities in northwestern Ontario: Social determinants of health "bite the heart".

OBJECTIVE: To document a case series of 8 young First Nations patients diagnosed with acute rheumatic fever (ARF), a preventable disease that resulted in the death of 2 patients, in northwestern Ontario in the context of late diagnosis, overcrowded housing, and inadequate public health response. DESIGN: Retrospective case series over an 18-month period. SETTING: Remote First Nations communities in northwestern Ontario. PARTICIPANTS: Eight patients with ARF. MAIN OUTCOME MEASURES: Incidence, mortality, residual rheumatic heart disease, time to diagnosis, barriers to diagnosis and treatment, housing situation of patients, patient demographic characteristics (age, sex), and investigation results. RESULTS: The incidence of ARF in this population was 21.3 per 100,000, which is 75 times greater than the overall Canadian estimated incidence. The average patient age was 9.4 years. Most cases developed joint findings, and 5 of the surviving patients had rheumatic heart disease when they received echocardiography. The average time to diagnosis was 88 days. Two 4-year-old children died from ARF. Most patients lived in inadequate and crowded housing. CONCLUSION: This rare disease still exists in remote First Nations communities. These communities demonstrate an incidence equal to that in aboriginal communities in Australia and New Zealand, which have among the highest international incidence of ARF. Primordial prevention, including improved on-reserve housing, is urgently needed. Case detection and ongoing surveillance for primary and secondary prophylaxis requires a well resourced regional strategy.

Influence of protein tyrosine phosphatase gene (PTPN22) polymorphisms on rheumatic heart disease susceptibility in North Indian population.

This study was aimed to assess the association of Protein tyrosine phosphatase non-receptor22 (PTPN22) gene single nucleotide polymorphisms (SNPs) with rheumatic heart disease (RHD) susceptibility in 400 RHD patients and 300 controls. The PTPN22 polymorphisms (rs2476601, rs1217406 and rs3789609) were genotyped using Taqman probes (Applied Biosystems, Foster City, CA). Statistical analysis was performed by spss and haplotype analysis by snpstat. The frequencies of variant alleles were not different between controls and cases (rs2476601: 2.00% & 1.05%; rs1217406: 36.33% & 34.75%; and rs3789609: 38.17% & 40.00%, respectively]. However, G rs2476601 A rs1217406 T rs3789609 haplotype turned out to be a low risk factor for RHD (P = 0.0042) predisposition in females and adult patients. This study suggests PTPN22 haplotype may modulate the risk to RHD in North Indians.

A review of valve surgery for rheumatic heart disease in Australia.

BACKGROUND: Globally, rheumatic heart disease (RHD) remains an important cause of heart disease. In Australia it particularly affects older non-Indigenous Australians and Aboriginal Australians and/or Torres Strait Islander peoples. Factors associated with the choice of treatment for advanced RHD remain variable and poorly understood. METHODS: The Australian and New Zealand Society of Cardiac and Thoracic Surgeons Cardiac Surgery Database was analysed. Demographics, co-morbidities, pre-operative status and valve(s) affected were collated and associations with management assessed. RESULTS: Surgical management of 1384 RHD and 15843 non-RHD valve procedures was analysed. RHD patients were younger, more likely to be female and Indigenous Australian, to have atrial fibrillation (AF) and previous percutaneous balloon valvuloplasty (PBV). Surgery was performed on one valve in 64.5%, two valves in 30.0% and three valves in 5.5%. Factors associated with receipt of mechanical valves in RHD were AF (OR 2.69) and previous PBV (OR 1.98) and valve surgery (OR 3.12). Predictors of valve repair included being Indigenous (OR 3.84) and having fewer valves requiring surgery (OR 0.10). Overall there was a significant increase in the use of mitral bioprosthetic valves over time. CONCLUSIONS: RHD valve surgery is more common in young, female and Indigenous patients. The use of bioprosthetic valves in RHD is increasing. Given many patients are female and younger, the choice of valve surgery and need for anticoagulation has implications for future management of RHD and related morbidity, pregnancy and lifestyle plans.

Mortality attributable to rheumatic heart disease in the Kimberley: a data linkage approach.

BACKGROUND: Acute rheumatic fever (ARF) and its sequelae, rheumatic heart disease (RHD) are now uncommon in the general Australian population. However, these preventable and treatable diseases continue to affect Aboriginal Australians disproportionately, especially in remote communities. In the Kimberley region of Western Australia (WA), the prevalence of RHD is approximately 1% among Aboriginal residents. Yet an accurate and comprehensive picture of RHD-related mortality is lacking. AIM: This study aims to determine the mortality burden attributable to ARF and RHD in the Kimberley using linked hospitalisation and death registry data. METHODS: A retrospective cohort study was undertaken comprising all Kimberley residents with a WA hospital admission for ARF or RHD between 1970 and 2010, linked with the WA Death Register. We manually classified RHD-attributable deaths ('definite' or 'probable') to determine mortality burden. Hospitalisation prior to death, including valvular surgery was also ascertained. RESULTS: There were 35 RHD-attributable deaths in the Kimberley between 1990 and 2010, with 94% occurring in Aboriginal people. Their median age of death was 40 years. The age-standardised RHD annual death rate was 15.6 per 100 000 with a total of 1100 premature years of life lost before age of 75 within this group. Conventional International Classification of Diseases-generated mortality data underestimated mortality burden. CONCLUSION: RHD remains a significant cause of premature mortality for Aboriginal people in the Kimberley, with mortality rates unmatched in the general Australian population since the first half of the 20th century. Efforts to reduce progression of this disease through RHD Register and Control Programs are crucial alongside action to address underlying socioeconomic and environmental inequities.

Cardiac surgery in Indigenous Australians--how wide is 'the gap'?

BACKGROUND: Cardiovascular disease remains the leading cause of mortality in the Indigenous Australian population. Limited research exists in regards to cardiac surgery in the Aboriginal and Torres Strait Islander (ATSI) population. We aimed to investigate risk profiles, surgical pathologies, surgical management and short term outcomes in a contemporary group of patients. METHODS: Variables were assessed for 557 consecutive patients who underwent surgery at our institution between August 2008 and March 2010. RESULTS: 19.2% (107/557) of patients were of Indigenous origin. ATSI patients were significantly younger at time of surgery (mean age 54.1±13.23 vs. 63.1±12.46; p=<0.001) with higher rates of preventable risk factors. Rheumatic heart disease (RHD) was the dominant valvular pathology observed in the Indigenous population. Significantly higher rates of left ventricular impairment and more diffuse coronary artery disease were observed in ATSI patients. A non-significant trend towards higher 30-day mortality was observed in the Indigenous population (5.6% vs. 3.1%; p=0.244). CONCLUSIONS: Cardiac surgery is generally required at a younger age in the Indigenous population with patients often presenting with more advanced disease. Despite often more advanced disease, surgical outcomes do not differ significantly from non-Indigenous patients. Continued focus on preventative strategies for coronary artery disease and RHD in the Indigenous population is required.

Rheumatic mitral stenosis in children: more accelerated course in sub-Saharan patients.

BACKGROUND: Mitral stenosis, one of the grave consequences of rheumatic heart disease, was generally considered to take decades to evolve. However, several studies from the developing countries have shown that mitral stenosis follows a different course from that seen in the developed countries. This study reports the prevalence, severity and common complications of mitral stenosis in the first and early second decades of life among children referred to a tertiary center for intervention. METHODS: Medical records of 365 patients aged less than 16 and diagnosed with rheumatic heart disease were reviewed. Mitral stenosis was graded as severe (mitral valve area < 1.0 cm2), moderate (mitral valve area 1.0-1.5 cm2) and mild (mitral valve area > 1.5 cm2). RESULTS: Mean age at diagnosis was 10.1 ± 2.5 (range 3-15) years. Of the 365 patients, 126 (34.5%) were found to have mitral stenosis by echocardiographic criteria. Among children between 6-10 years, the prevalence of mitral stenosis was 26.5%. Mean mitral valve area (n = 126) was 1.1 ± 0.5 cm2 (range 0.4-2.0 cm2). Pure mitral stenosis was present in 35 children. Overall, multi-valvular involvement was present in 330 (90.4%). NYHA functional class was II in 76% and class III or IV in 22%. Only 25% of patients remember having symptoms of acute rheumatic fever. Complications at the time of referral include 16 cases of atrial fibrillation, 8 cases of spontaneous echo contrast in the left atrium, 2 cases of left atrial thrombus, 4 cases of thrombo-embolic events, 2 cases of septic emboli and 3 cases of airway compression by a giant left atrium. CONCLUSION: Rheumatic mitral stenosis is common in the first and early second decades of life in Ethiopia. The course appeared to be accelerated resulting in complications and disability early in life. Echocardiography-based screening programs are needed to estimate the prevalence and to provide support for strengthening primary and secondary prevention programs.

Variability in disease burden and management of rheumatic fever and rheumatic heart disease in two regions of tropical Australia.

BACKGROUND: Acute rheumatic fever (ARF) and rheumatic heart disease (RHD) contribute to Aboriginal Australian and Torres Strait Islander health disadvantage. At the time of this study, specialist ARF/RHD care in the Kimberley region of Western Australia was delivered by a broad range of providers. In contrast, in Far North Queensland (FNQ), a single-provider model was used as part of a coordinated RHD control programme. AIMS: To review ARF/RHD management in the Kimberley and FNQ to ascertain whether differing models of service delivery are associated with different disease burden and patient care. METHODS: An audit of ARF/RHD management. Classification and clinical management data were abstracted from health records, specialist letters, echocardiograms and regional registers using a standardised data collection tool. RESULTS: Four hundred and seven patients were identified, with 99% being Aboriginal and/or Torres Strait Islanders. ARF without RHD was seen in 0.4% of Aboriginal and/or Torres Strait Islander residents and RHD in 1.1%. The prevalence of RHD was similar in both regions but with more severe disease in the Kimberley. More FNQ RHD patients had specialist review within recommended time frames (67% vs 45%, χ(2) , P < 0.001). Of patients recommended benzathine penicillin secondary prophylaxis, 17.7% received ≥80% of scheduled doses in the preceding 12 months. Prescription and delivery of secondary prophylaxis was greater in FNQ. CONCLUSIONS: FNQ's single-provider model of specialist care and centralised RHD control programme were associated with improved patient care and may partly account for the fewer cases of severe disease and reduced surgical procedures and other interventions observed in this region.

Using community-led development to build health communication about rheumatic heart disease in Aboriginal children: a developmental evaluation.

OBJECTIVE: A high prevalence of acute rheumatic fever (ARF) and rheumatic heart disease (RHD) among Aboriginal children in northern Australia is coupled with low understanding among families. This has negative impacts on children's health, limits opportunities for prevention and suggests that better health communication is needed. METHODS: During an RHD echocardiography screening project, Aboriginal teachers in a remote community school created lessons to teach children about RHD in their home languages, drawing on principles of community-led development. Access to community-level RHD data, previously unknown to teachers and families, was a catalyst for this innovative work. Careful, iterative discussions among speakers of four Aboriginal languages ensured a culturally coherent narrative and accompanying teaching resources. RESULTS: The evaluation demonstrated the importance of collective work, local Indigenous Knowledge and metaphors. As a result of the lessons, some children showed new responses and attitudes to skin infections and their RHD treatment. Language teachers used natural social networks to disseminate new information. A community interagency collaboration working to prevent RHD commenced. Conclusions and implications for public health: Action to address high rates of RHD must include effective health communication strategies that value Indigenous Knowledge, language and culture, collaborative leadership and respect for Indigenous data sovereignty.

Association of Novel Locus With Rheumatic Heart Disease in Black African Individuals: Findings From the RHDGen Study.

Importance: Rheumatic heart disease (RHD), a sequela of rheumatic fever characterized by permanent heart valve damage, is the leading cause of cardiac surgery in Africa. However, its pathophysiologic characteristics and genetics are poorly understood. Understanding genetic susceptibility may aid in prevention, control, and interventions to eliminate RHD. Objective: To identify common genetic loci associated with RHD susceptibility in Black African individuals. Design, Setting, and Participants: This multicenter case-control genome-wide association study (GWAS), the Genetics of Rheumatic Heart Disease, examined more than 7 million genotyped and imputed single-nucleotide variations. The 4809 GWAS participants and 116 independent trio families were enrolled from 8 African countries between December 31, 2012, and March 31, 2018. All GWAS participants and trio probands were screened by use of echocardiography. Data analyses took place from May 15, 2017, until March 14, 2021. Main Outcomes and Measures: Genetic associations with RHD. Results: This study included 4809 African participants (2548 RHD cases and 2261 controls; 3301 women [69%]; mean [SD] age, 36.5 [16.3] years). The GWAS identified a single RHD risk locus, 11q24.1 (rs1219406 [odds ratio, 1.65; 95% CI, 1.48-1.82; P = 4.36 × 10-8]), which reached genome-wide significance in Black African individuals. Our meta-analysis of Black (n = 3179) and admixed (n = 1055) African individuals revealed several suggestive loci. The study also replicated a previously reported association in Pacific Islander individuals (rs11846409) at the immunoglobulin heavy chain locus, in the meta-analysis of Black and admixed African individuals (odds ratio, 1.16; 95% CI, 1.06-1.27; P = 1.19 × 10-3). The HLA (rs9272622) associations reported in Aboriginal Australian individuals could not be replicated. In support of the known polygenic architecture for RHD, overtransmission of a polygenic risk score from unaffected parents to affected probands was observed (polygenic transmission disequilibrium testing mean [SE], 0.27 [0.16] SDs; P = .04996), and the chip-based heritability was estimated to be high at 0.49 (SE = 0.12; P = 3.28 × 10-5) in Black African individuals. Conclusions and Relevance: This study revealed a novel candidate susceptibility locus exclusive to Black African individuals and an important heritable component to RHD susceptibility in African individuals.

Rheumatic fever in indigenous Australian children.

Rheumatic heart disease (RHD) caused by acute rheumatic fever (ARF) is a disease of poverty, poor hygiene and poor living standards. RHD remains one of the major causes of childhood cardiac disease in developing nations. Within developed nations, there has been a dramatic drop in the prevalence of RHD because of the improvement of living standards, access to health care and the widespread availability of penicillin-based drugs. Despite a dramatic reduction of RHD in Australia overall, it continues to be a major contributor to childhood and adult cardiac disease in Indigenous communities throughout northern and central Australia. Currently, Australia has among the highest recorded rates of ARF and RHD in the world. The most accurate epidemiological data in Australia come from the Northern Territory's RHD control programme. In the Northern Territory, 92% of people with RHD are Indigenous, of whom 85% live in remote communities and towns. The incidence of ARF is highest in 5-14-year-olds, ranging from 150 to 380 per 100,000. Prevalence rates of RHD since 2000 have steadily increased to almost 2% of the Indigenous population in the Northern Territory, 3.2% in those aged 35-44 years. Living in remote communities is a contributing factor to ARF/RHD as well as a major barrier for adequate follow-up and care. Impediments to ARF/RHD control include the paucity of specialist services, rapid turnover of health staff, lack of knowledge of ARF/RHD by health staff, patients and communities, and the high mobility of the Indigenous population. Fortunately, the recently announced National Rheumatic Fever Strategy, comprising recurrent funding to the Northern Territory, Queensland and Western Australia for control programmes, as well as the creation of a National Coordination Unit suggest that RHD control in Australia is now a tangible prospect. For the disease to be eradicated, Australia will have to address the underpinning determinants of poverty, social and living conditions.

Excellent cardiac surgical outcomes in paediatric indigenous patients, but follow-up difficulties.

BACKGROUND: Indigenous Australians' infant mortality is three times that of non-Indigenous Australians. Indigenous children's mortality from rheumatic heart disease is 17-21 times that of non-Indigenous male and female children, respectively. No studies have looked specifically at the operative outcomes of cardiac surgery in paediatric Indigenous patients in Australia and little is known about their follow-up. AIMS: To describe operative outcomes of all Indigenous paediatric cardiac surgical patients at a single Australian tertiary hospital and assess their follow-up. METHODS: Database review of retrospectively collected data of all Indigenous paediatric patients who had cardiac surgery performed at The Prince Charles Hospital, Brisbane between 2002 and 2009 (112 patients, 123 operations). Follow-up was assessed by chart review and time to first post-discharge echocardiogram recorded in the hospital database. RESULTS: Eighty-one percent of operations were congenital heart disease related and 19% of operations were rheumatic heart disease related. Common co-morbidities included respiratory (9.7%) and renal dysfunction (0.8%). Common complications were, bleeding/tamponade 4.1%, cardiac arrest 4.1% and new atrial arrhythmia 2.4%. Mortality was 1% for congenital operations and 4.4% for rheumatic operations. Only 33% of patients had follow-up within eight weeks documented through letters or chart entry. Only 77.5% of patients had a documented follow-up echocardiogram. DISCUSSION: Operative outcome in Indigenous paediatric patients is similar to that found in the global literature. The follow-up for such an excellent surgical outcome has been disappointing. A coordinated action within and between health, health related and social institutions with sufficient resources will assist.

Rheumatic heart disease in indigenous populations.

Rates of acute rheumatic fever and chronic rheumatic heart disease in Aboriginal people, Torres Strait Islanders and Maori continue to be unacceptably high. The impact of rheumatic heart disease is inequitable on these populations as compared with other Australians and New Zealanders. The associated cardiac morbidity, including the development of rheumatic valve disease, and cardiomyopathy, with possible sequelae of heart failure, development of atrial fibrillation, systemic embolism, transient ischaemic attacks, strokes, endocarditis, the need for interventions including cardiac surgery, and impaired quality of life, and shortened life expectancy, has major implications for the individual. The adverse health and social effects may significantly limit education and employment opportunities and increase dependency on welfare. Additionally there may be major adverse impacts on family and community life. The costs in financial terms and missed opportunities, including wasted young lives, are substantial. Prevention of acute rheumatic fever is dependent on the timely diagnosis and treatment of sore throats and skin infections in high-risk groups. Both Australia and New Zealand have registries for acute rheumatic fever but paradoxically neither includes all cases of chronic rheumatic heart disease many of whom would benefit from close surveillance and follow-up. In New Zealand and some Australian States there are programs to give secondary prophylaxis with penicillin, but these are not universal. Surgical outcomes for patients with rheumatic valvular disease are better for valve repair than for valve replacement. Special attention to the selection of the appropriate valve surgery and valve choice is required in pregnant women. It may be necessary to have designated surgical units managing Indigenous patients to ensure high rates of surgical repair rather than valve replacement. Surgical guidelines may be helpful. Long-term follow-up of the outcomes of surgery in Indigenous patients with rheumatic heart disease is required. Underpinning these strategies is the need to improve poverty, housing, education and employment. Cultural empathy with mutual trust and respect is essential. Involvement of Indigenous people in decision making, design, and implementation of primary and secondary prevention programs, is mandatory to reduce the unacceptably high rates of rheumatic heart disease.

Surgical challenges in rheumatic heart disease in the Australian indigenous population.

BACKGROUND: Acute rheumatic fever, leading to rheumatic heart disease (RHD), is rare in Australia except amongst Aboriginal and Torres Strait Islander people. METHODS: Cardiac surgical procedures performed at Flinders Medical Centre on patients from the Top End of the Northern Territory from 1993 to 2008 were reviewed. This study compared Indigenous and non-Indigenous patients on short term morbidity and long term survival employing logistic regression and Cox proportional hazard models. We also outline the challenges of managing Aboriginal patients, as our unit services vast areas of northern Australia inhabited by Indigenous people. RESULTS: The total number of patients from the Northern Territory was 835. Amongst the Indigenous patients, there were 235 (55.6%) isolated coronary artery bypass graft procedures, 171 (40.4%) patients underwent isolated valvular surgery (91 mitral and 80 aortic), and 17 (4.0%) underwent combined valvular surgery with coronary artery bypass graft surgery. CONCLUSIONS: Aboriginal patients requiring valve surgery are younger and have greater comorbidity than non-Aboriginal people. Short term surgical results are similar to non-Aboriginal people but long term outcomes appear to be inferior. Age and socioeconomic conditions of Indigenous patients need to be considered. Cultural issues should be understood and acknowledged and surgery better focused around surgical units with appropriate infrastructure.