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Rheumatic Heart Disease Australia

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Burden of Disease Fact Checked

Written by 

Sara Noonan

Background

Acute rheumatic fever (ARF) and rheumatic heart disease (RHD) were once common across the world, including Australia, Europe, Great Britain, and North America.1,2,3 Rates of disease declined in most of these countries during the mid-20th century, primarily due to improved socioeconomic conditions and reduced household crowding, better access to healthcare, and the availability of penicillin to treat streptococcal infections.4,5 

A view of beds in the Irvington Home (USA) for rheumatic fever.

Denver Post, March 17, 1957

Today, there are populations of older people with RHD in many high-income countries. These people developed the disease when they were young, and before improvements in socioeconomic conditions and antibiotics. There are also some Indigenous populations living in high-income countries that continue to be affected by ARF and RHD, however, these people are most often children and young adults. 6

ARF and RHD are also common among young people across many low- and middle-income countries despite the availability of penicillin, particularly where widespread poverty persists and access to health care is difficult.7

ARF and RHD in Australia

In Australia, ARF and RHD continue to cause significant ill-health and premature death among Australia’s Aboriginal and Torres Strait Islander peoples and New Zealand Māori and Pacific Islanders. These groups also experience considerable inequalities across a wide range of social, educational and health outcomes compared with the general population.8,9,10

  • Since the early 1990s, ARF has occurred almost exclusively in young Aboriginal and Torres Strait Islander peoples, with highest rates among children.11 
  • Females are more likely to be diagnosed with ARF and RHD than males, and rates of disease are highest across northern Australia.12 
  • The number of Aboriginal and Torres Strait Islander peoples affected by ARF and RHD appears to be increasing, however the exact reason for this is not clear.13 
  • People who have had ARF are at high risk of having it again, with one in five people having a recurrent episode of ARF within 10 years of their first illness.14
  • The burden of disease often lasts most of a person’s lifetime, starting with ARF in childhood and progressing to RHD for the duration of adult life.
  • Complications from RHD are common, and include atrial fibrillation, endocarditis, heart failure and stroke.
  • Aboriginal and Torres Strait Islander people with RHD are more likely to die compared with non-Indigenous Australians with RHD; however, the death rate for both groups is decreasing.15

Photo courtesy of Take Heart

Data collection and reporting systems

Understanding the true burden of ARF and RHD in Australia, that is, the number of people with disease and where they live, is difficult. State and territory-based ARF/RHD disease registers have been established in the Northern Territory, Queensland, Western Australia, South Australia and New South Wales. Data from these registers are collected and reported by the Australian Institute of Health and Welfare, which is an independent Australian Government agency.16

While these reports are very useful, there are still challenges to fully understanding ARF and RHD in Australia. For example:

  1. Each ARF/RHD register was developed at a different time, and the processes for collecting data vary. 
  2. In some areas, there are only small numbers of people with ARF and RHD, and reporting small numbers is difficult.
  3. Indigenous population estimates are not stable below state and territory level, and this can make reporting difficult.
  4. There are various other sources of ARF and RHD information (e.g., hospital admission records, surgery records, death registers), however, these systems are not linked together and are not linked with the ARF/RHD registers, so an accurate number of people with disease is hard to predict. 

End RHD in Australia: Study of Epidemiology (ERASE)

In 2020, researchers working on a linked ARF/RHD data project published the End RHD in Australia: Study of Epidemiology (ERASE) study.17 ARF/RHD data were collected, linked and analysed from the five ARF/RHD disease registers,18 as well as data from hospital admissions, death databases, cardiac surgery databases, emergency department presentations, primary care presentations, and maternity notification data systems. The ERASE study provided the most comprehensive description of the epidemiology and management of ARF and RHD available in Australia.

   As well as being reported through a series of publications,19,20,21,22,23,24,25 results from the ERASE study are being used to guide health policy and clinical practice care to support people with ARF and RHD in Australia. For example, the results

  • provided evidence for the 3rd edition of the ARF/RHD guideline.
  • informed policy recommendations for the RHD Strategy Endgame Report.26
  • were used to project future costs associated with health interventions for ARF and RHD. 
  • are being used to provide meaningful information to Aboriginal and Torres Strait Islander communities about ARF and RHD risk, prevention, diagnosis, and management.

Resources

A series of slides from the ERASE study data have been developed for the health, research, and scientific community. These slides are available to use in reports and presentations. Interpretations have been included to help provide context and highlight important information. 

ERASE data: Maps (Select Read Only)

(Not all maps appear in the ERASE publications)

 

  • 1.  Sullivan E, Vaughan G, Li Z, et al. The high prevalence and impact of rheumatic heart disease in pregnancy in First Nations populations in a high-income setting: a prospective cohort study. BJOG: An International Journal of Obstetrics & Gynaecology. 2019;27(1):47-56. View Source
  • 2.  Hajar R. Rheumatic Fever and Rheumatic Heart Disease a Historical Perspective. Heart Views. 2016;17(3):120-126.
  • 3. English PC. Rheumatic fever in America and Britain. A Biological, Epidemiological, and Medical History. Rutgers University Press. New Jersey. 1999
  • 4.  Gordis L. The virtual disappearance of rheumatic fever in the United States: lessons in the rise and fall of disease. T. Duckett Jones memorial lecture. Circulation. 1985;72(6):1155-1162. View Source
  • 5.  Bland EF. Rheumatic fever: the way it was. Circulation. 1987;76(6):1190-1195.
  • 6.  Australian Institute of Health and Welfare. Acute rheumatic fever and rheumatic heart disease in Australia. Cat. no: CVD 86. Australian Institute of Health and Welfare, Canberra, 2019 View Source 
  • 7.  Zühlke L, Engel ME, Karthikeyan G, et al. Characteristics, complications, and gaps in evidence-based interventions in rheumatic heart disease: The Global Rheumatic Heart Disease Registry (the REMEDY study). European Heart Journal. 2015;36(18):1115-1122a. View Source 
  • 8.  Baker MG, Barnard LT, Kvalsvig A, et al. Increasing incidence of serious infectious diseases and inequalities in New Zealand: a national epidemiological study. The Lancet. 2012;379;(9821):1112-1119. View Source 
  • 9.  World Health Organization. Closing the gap in a generation: health equity through action on the social determinants of health. Final Report of the Commission on Social Determinants of Health. Geneva Switzerland, 2008. View Source
  • 10.  Carapetis JR, Zühlke L, Taubert K, Narula J. Continued challenge of rheumatic heart disease: The gap of understanding or the gap of implementation? Global Heart .2013;8(8):185-186. View Source
  • 11.  Australian Institute of Health and Welfare. Acute rheumatic fever and rheumatic heart disease in Australia. Cat. no: CVD 86. Australian Institute of Health and Welfare, Canberra, 2019
  • 12.  Australian Institute of Health and Welfare. Acute rheumatic fever and rheumatic heart disease in Australia. Cat. no: CVD 86. Australian Institute of Health and Welfare, Canberra, 2019
  • 13.  Australian Institute of Health and Welfare. Acute rheumatic fever and rheumatic heart disease in Australia. Cat. no: CVD 86. Australian Institute of Health and Welfare, Canberra, 2019
  • 14.  He VYF, Condon JR, Ralph AP, et al. Long-term outcomes from acute rheumatic fever and rheumatic heart disease: A data-linkage and survival analysis approach. Circulation. 2016;134:222-232. View Source
  • 15.  Colquhoun SM, Condon JR, Steer AC, et al. Disparity in mortality from Rheumatic Heart Disease in Indigenous Australians. Journal of the American Heart Association. 2015;4(7). View Source
  • 16.  Australian Institute of Health and Welfare 2019. Acute rheumatic fever and rheumatic heart disease—in brief. Cat. no. CVD 87. Canberra: AIHW.
  • 17.  Katzenellenbogen JM, Bond-Smith D, Cunneen R, et al. Contemporary Incidence and Prevalence of Rheumatic Fever and Rheumatic Heart Disease in Australia Using Linked Data: The Case for Policy Change. Journal of the American Health Association. 2020; Vol 9(19): e016851.
  • 18.  Bond-Smith D, Seth R, de Klerk N, et al. Development and evaluation of a prediction model for ascertaining rheumatic heart disease status in administrative data. Clinical Epidemiology. 2020;12: 717-730.
  • 19.  Bond-Smith D, Seth R, de Klerk N, et al. Development and evaluation of a prediction model for ascertaining rheumatic heart disease status in administrative data. Clinical Epidemiology. 2020;12: 717-730.
  • 20.  Katzenellenbogen JM, Bond-Smith D, Cunneen R, Dempsey K, Cannon J, Stacey I, Nedkoff L, Sanfilippo FM, Ralph AP, De Klerk N, Hung J, Geelhoed E, Williamson D, Wyber R, Bessarab D. The End Rheumatic Heart Disease in Australia Study of Epidemiology (ERASE) Project: data sources, case ascertainment and cohort profile. Clin Epidemiol 2019;11: 997-1010.
  • 21.  Cannon J, Bessarab DC, Wyber R, Katzenellenbogen JM. Public health and economic perspectives on acute rheumatic fever and rheumatic heart disease: can we afford ’business as usual’? Medical Journal of Australia. 2019;211(6): 250-252.
  • 22.  Katzenellenbogen JM, Bond-Smith D, Ralph A, et al. Priorities for improvement in management of Acute Rheumatic Fever and Rheumatic Heart Disease: analysis of cross-sectional continuous quality improvement data in Aboriginal primary health care centres in Australia. Australian Health Review 2019:44(2): 212-221.
  • 23.  Fitz-Gerald JA, Ongzalima CO, Ng A, et al. A validation study: how predictive is a diagnostic coding algorithm at identifying rheumatic heart disease in Western Australian hospital data? Heart Lung and Circulation. 2020:29(8): e194-e199.
  • 24.  Ongzalima CO, Greenland M, Vaughan G, et al.  Rheumatic Heart Disease in pregnancy: Profile of women admitted to a Western Australian Tertiary Obstetric Hospital. The Australian and New Zealand Journal of Obstetrics and Gynaecology. 2020;60(2): 302-308.
  • 25. Katzenellenbogen JM, Nedkoff L, Canon J, et al. Low positive predictive value of ICD-10 codes in relation to rheumatic heart disease: a challenge for global surveillance. International medical Journal. 2019;49(3): 400-403.
  • 26. Wyber R, Noonan K, Halkon C, et al. The RHD Endgame Strategy: The blueprint to eliminate rheumatic heart disease in Australia by 2031. Perth: The END RHD Centre of Research Excellence, Telethon Kids Institute, 2020.
Last Updated 
18 May 2022
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