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Screening for rheumatic heart disease: current approaches and controversies

Nature Reviews Cardiology volume 10, pages 49–58 (2013)Cite this article

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Abstract

Rheumatic heart disease (RHD) is a leading cause of cardiac disease among children in developing nations, and in indigenous populations of some industrialized countries. In endemic areas, RHD has long been a target of screening programmes that, historically, have relied on cardiac auscultation. The evolution of portable echocardiographic equipment has changed the face of screening for RHD over the past 5 years, with greatly improved sensitivity. However, concerns have been raised about the specificity of echocardiography, and the interpretation of minor abnormalities poses new challenges. The natural history of RHD in children with subclinical abnormalities detected by echocardiographic screening remains unknown, and long-term follow-up studies are needed to evaluate the significance of detecting these changes at an early stage. For a disease to be deemed suitable for screening from a public health perspective, it needs to fulfil a number of criteria. RHD meets some, but not all, of these criteria. If screening programmes are to identify additional cases of RHD, parallel improvements in the systems that deliver secondary prophylaxis are essential.

Key Points

  • Rheumatic heart disease (RHD) is preventable, yet continues to be a leading cause of cardiac disease in children in developing countries

  • For a disease to be deemed suitable for screening, it needs to fulfil a number of agreed criteria; RHD satisfies most, but not all, of those criteria

  • RHD has long been a target of screening programs that, historically, have relied on cardiac auscultation

  • The evolution of portable echocardiography has changed the face of screening for RHD, with greatly improved sensitivity; however, the interpretation of minor abnormalities poses new challenges

  • The natural history of minor valvular abnormalities detected by echocardiographic screening remains unknown

  • If screening programmes are to identify additional cases of RHD, parallel improvements in the systems that deliver secondary prophylaxis are essential

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Figure 1: Prevalence of rheumatic heart disease in children aged 5–14 years.
Figure 2: Echocardiographic images depict subclinical rheumatic heart disease in a 10-year-old boy with no cardiac murmur.

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References

  1. Carapetis, J. R., Steer, A. C., Mulholland, E. K. & Weber, M. The global burden of group A streptococcal diseases. Lancet Infect. Dis. 5, 685–694 (2005).

    Article  PubMed  Google Scholar 

  2. WHO. Prevention of rheumatic fever: Report of a WHO expert committee. WHO Technical Report Series, No. 342 (1966).

  3. Marijon, E. et al. Prevalence of rheumatic heart disease detected by echocardiographic screening. N. Engl. J. Med. 357, 470–476 (2007).

    Article  CAS  PubMed  Google Scholar 

  4. Carapetis, J. R. et al. Evaluation of a screening protocol using auscultation and portable echocardiography to detect asymptomatic rheumatic heart disease in Tongan schoolchildren. Nat. Clin. Pract. Cardiovasc. Med. 5, 411–417 (2008).

    Article  PubMed  Google Scholar 

  5. Bhaya, M., Panwar, S., Beniwal, R. & Panwar, R. B. High prevalence of rheumatic heart disease detected by echocardiography in school children. Echocardiography 27, 448–453 (2010).

    Article  PubMed  Google Scholar 

  6. Paar, J. A. et al. Prevalence of rheumatic heart disease in children and young adults in Nicaragua. Am. J. Cardiol. 105, 1809–1814 (2010).

    Article  PubMed  PubMed Central  Google Scholar 

  7. Webb, R. H. et al. Optimising echocardiographic screening for rheumatic heart disease in New Zealand: not all valve disease is rheumatic. Cardiol. Young 21, 436–443 (2011).

    Article  PubMed  Google Scholar 

  8. Saxena, A. et al. Prevalence and outcome of subclinical rheumatic heart disease in India: the RHEUMATIC (Rheumatic Heart Echo Utilisation and Monitoring Actuarial Trends in Indian Children) study. Heart 97, 2018–2022 (2011).

    Article  PubMed  Google Scholar 

  9. Beaton, A. et al. Echocardiography screening for rheumatic heart disease in Ugandan schoolchildren. Circulation 125, 3127–3132 (2012).

    Article  PubMed  Google Scholar 

  10. Kothari, S. S. Letter by Kothari regarding article, “Rheumatic heart disease screening by echocardiography: The inadequacy of World Health Organization criteria for optimizing the diagnosis of subclinical disease”. Circulation 121, e420; author reply e423 (2010).

    Article  PubMed  Google Scholar 

  11. DeGroff, C. G. Letter by DeGroff regarding article, “Rheumatic heart disease screening by echocardiography: The inadequacy of World Health Organization criteria for optimizing the diagnosis of subclinical disease”. Circulation 121, e421; author reply e423 (2010).

    Article  PubMed  Google Scholar 

  12. Steer, A. C. & Carapetis, J. R. Prevention and treatment of rheumatic heart disease in the developing world. Nat. Rev. Cardiol. 6, 689–698 (2009).

    Article  PubMed  Google Scholar 

  13. Marijon, E. et al. Echocardiographic screening for rheumatic heart disease. Bull. World Health Organ. 86, 84 (2008).

    Article  PubMed  PubMed Central  Google Scholar 

  14. Remenyi, B. et al. World Heart Federation criteria for echocardiographic diagnosis of rheumatic heart disease-an evidence-based guideline. Nat. Rev. Cardiol. 9, 297–309 (2012).

    Article  PubMed  PubMed Central  Google Scholar 

  15. Wilson, J. & Jungner, G. Principles and practice of screening for disease. WHO Public Health Papers, No. 34 (1968).

    Google Scholar 

  16. Council of Europe. Committee of Ministers. Recommendation No. R (94) 11 on Screening as a Tool of Preventive Medicine (1994).

  17. WHO. Global Burden of Disease (GBD) 2010 study [online], (2012).

  18. Parnaby, M. G. & Carapetis, J. R. Rheumatic fever in indigenous Australian children. J. Paediatr. Child Health 46, 527–533 (2010).

    Article  PubMed  Google Scholar 

  19. Longo-Mbenza, B. et al. Survey of rheumatic heart disease in school children of Kinshasa town. Int. J. Cardiol. 63, 287–294 (1998).

    Article  CAS  PubMed  Google Scholar 

  20. Rizvi, S. F. et al. Status of rheumatic heart disease in rural Pakistan. Heart 90, 394–399 (2004).

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  21. Sadiq, M. et al. Prevalence of rheumatic heart disease in school children of urban Lahore. Heart 95, 353–357 (2009).

    Article  CAS  PubMed  Google Scholar 

  22. Ba-Saddik, I. A. et al. Prevalence of rheumatic heart disease among school-children in Aden, Yemen. Ann. Trop. Paediatr. 31, 37–46 (2011).

    Article  CAS  PubMed  Google Scholar 

  23. Carapetis, J. R. Rheumatic heart disease in Asia. Circulation 118, 2748–2753 (2008).

    Article  PubMed  Google Scholar 

  24. Jackson, S. J., Steer, A. C. & Campbell, H. Systematic Review: estimation of global burden of non-suppurative sequelae of upper respiratory tract infection: rheumatic fever and post-streptococcal glomerulonephritis. Trop. Med. Int. Health 16, 2–11 (2011).

    Article  PubMed  Google Scholar 

  25. Bland, E. & Jones, T. Rheumatic fever and rheumatic heart disease. A twenty-year report on 1,000 patients followed since childhood. Circulation 4, 836–843 (1951).

    Article  CAS  PubMed  Google Scholar 

  26. Cohn, A. E. & Lingg, C. The natural history of rheumatic cardiac disease: a statistical study: I. Onset and duration of disease. JAMA 121, 1–8 (1943).

    Article  Google Scholar 

  27. Jaiyesimi, F. & Antia, A. U. Childhood rheumatic heart disease in Nigeria. Trop. Geogr. Med. 33, 8–13 (1981).

    CAS  PubMed  Google Scholar 

  28. Günther, G., Asmera, J. & Parry, E. Death from rheumatic heart disease in rural Ethiopia. Lancet 367, 391 (2006).

    Article  PubMed  Google Scholar 

  29. Kumar, R., Raizada, A., Aggarwal, A. K. & Ganguly, N. K. A community-based rheumatic fever/rheumatic heart disease cohort: twelve-year experience. Indian Heart J. 54, 54–58 (2002).

    PubMed  Google Scholar 

  30. Lloyd-Jones, D. et al. Heart disease and stroke statistics—2009 update: a report from the American Heart Association Statistics Committee and Stroke Statistics Subcommittee. Circulation 119, 480–486 (2009).

    Article  PubMed  Google Scholar 

  31. Quinn, R. W. Comprehensive review of morbidity and mortality trends for rheumatic fever, streptococcal disease, and scarlet fever: the decline of rheumatic fever. Rev. Infect. Dis. 11, 928–953 (1989).

    Article  CAS  PubMed  Google Scholar 

  32. Massell, B. F., Chute, C. G., Walker, A. M. & Kurland, G. S. Penicillin and the marked decrease in morbidity and mortality from rheumatic fever in the United States. N. Engl. J. Med. 318, 280–286 (1988).

    Article  CAS  PubMed  Google Scholar 

  33. Field, B. for the Australian Institute of Health and Welfare. Rheumatic Heart Disease: all but forgotten in Australia except amongst Aboriginal and Torres Strait Islander peoples. AIHW Bullentin No. 16, Cat. No. AUS 58 (2004).

  34. Colquhoun, S. et al. An analysis of mortality trends for rheumatic heart disease and rheumatic fever in indigenous and non-indigenous Australians [poster]. XVIII Lancefield International Symposium (2011).

  35. Carapetis, J. R. & Currie, B. J. Mortality due to acute rheumatic fever and rheumatic heart disease in the Northern Territory: a preventable cause of death in aboriginal people. Aust. N. Z. J. Public Health 23, 159–163 (1999).

    Article  CAS  PubMed  Google Scholar 

  36. Milne, R. J., Lennon, D., Stewart, J. M., Vander Hoorn, S. & Scuffham, P. A. Mortality and hospitalisation costs of rheumatic fever and rheumatic heart disease in New Zealand. J. Paediatr. Child Health 48, 692–697 (2012).

    Article  PubMed  Google Scholar 

  37. WHO. The global burden of disease: 2004 update [online]. (2004).

  38. Marijon, E., Mirabel, M., Celermajer, D. S. & Jouven, X. Rheumatic heart disease. Lancet 379, 953–964 (2012).

    Article  PubMed  Google Scholar 

  39. Carapetis, J. R. et al. Outcomes of cardiac valve replacement for rheumatic heart disease in aboriginal Australians. The Asia Pacific Heart Journal 8, 138–147 (1999).

    Article  Google Scholar 

  40. North, R. A. et al. Long-term survival and valve-related complications in young women with cardiac valve replacements. Circulation 99, 2669–2676 (1999).

    Article  CAS  PubMed  Google Scholar 

  41. [No authors listed] The natural history of rheumatic fever and rheumatic heart disease. Ten-year report of a cooperative clinical trial of ACTH, cortisone, and aspirin. Circulation 32, 457–476 (1965).

  42. Tompkins, D. G., Boxerbaum, B. & Liebman, J. Long-term prognosis of rheumatic fever patients receiving regular intramuscular benzathine penicillin. Circulation 45, 543–551 (1972).

    Article  CAS  PubMed  Google Scholar 

  43. Majeed, H. A. et al. The natural history of acute rheumatic fever in Kuwait: a prospective six year follow-up report. J. Chronic Dis. 39, 361–369 (1986).

    Article  CAS  PubMed  Google Scholar 

  44. Sanyal, S. K., Berry, A. M., Duggal, S., Hooja, V. & Ghosh, S. Sequelae of the initial attack of acute rheumatic fever in children from north India. A prospective 5-year follow-up study. Circulation 65, 375–379 (1982).

    Article  CAS  PubMed  Google Scholar 

  45. Feinstein, A. R. et al. Rheumatic fever in children and adolescents. A long-term epidemiologic study of subsequent prophylaxis, streptococcal infections, and clinical sequelae. Vii. Cardiac changes and sequelae. Ann. Intern. Med. 60 (Suppl. 5), 87–123 (1964).

    Article  Google Scholar 

  46. Majeed, H. A., Batnager, S., Yousof, A. M., Khuffash, F. & Yusuf, A. R. Acute rheumatic fever and the evolution of rheumatic heart disease: a prospective 12 year follow-up report. J. Clin. Epidemiol. 45, 871–875 (1992).

    Article  CAS  PubMed  Google Scholar 

  47. Carapetis, J. R., Currie, B. J. & Mathews, J. D. Cumulative incidence of rheumatic fever in an endemic region: a guide to the susceptibility of the population? Epidemiol. Infect. 124, 239–244 (2000).

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  48. Sliwa, K. et al. Incidence and characteristics of newly diagnosed rheumatic heart disease in urban African adults: insights from the heart of Soweto study. Eur. Heart J. 31, 719–727 (2010).

    Article  PubMed  Google Scholar 

  49. Jones, T. D. Diagnosis of rheumatic fever. JAMA 126, 481–484 (1944).

    Article  Google Scholar 

  50. Dajani, A. S. et al. Guidelines for the diagnosis of rheumatic fever. Jones Criteria, 1992 update. Special Writing Group of the Committee on Rheumatic Fever, Endocarditis, and Kawasaki Disease of the Council on Cardiovascular Disease in the Young of the American Heart Association. JAMA 268, 2069–2073 (1992).

    Article  Google Scholar 

  51. Carapetis, J. R. et al. An Australian guideline for rheumatic fever and rheumatic heart disease: an abridged outline. Med. J. Aust. 186, 581–586 (2007).

    PubMed  Google Scholar 

  52. Stollerman, G. H., Rusoff, J. H. & Hirschfeld, I. Prophylaxis against group A streptococci in rheumatic fever; the use of single monthly injections of benzathine penicillin G. N. Engl. J. Med. 252, 787–792 (1955).

    Article  CAS  PubMed  Google Scholar 

  53. Padmavati, S., Sharma, K. B. & Jayaram, O. Epidemiology and prophylaxis of rheumatic fever in Delhi—a five year follow-up. Singapore Med. J. 14, 457–461 (1973).

    CAS  PubMed  Google Scholar 

  54. Feinstein, A. R. et al. A controlled study of three methods of prophylaxis against streptococcal infection in a population of rheumatic children. II. Results of the first three years of the study, including methods for evaluating the maintenance of oral prophylaxis. N. Engl. J. Med. 260, 697–702 (1959).

    Article  CAS  PubMed  Google Scholar 

  55. Wood, H. F., Feinstein, A. R., Taranta, A., Epstein, J. A. & Simpson, R. Rheumatic fever in children and adolescents: a long-term epidemiologic study of subsequent prophylaxis, streptococcal infections, and clinical sequelae: III. Comparative effectiveness of three prophylaxis regiments in preventing streptococcal infection and rheumatic recurrences. Ann. Intern. Med. 60, 31–46 (1964).

    Article  Google Scholar 

  56. Feinstein, A. R., Stern, E. K. & Spagnuolo, M. The prognosis of acute rheumatic fever. Am. Heart J. 68, 817–834 (1964).

    Article  CAS  PubMed  Google Scholar 

  57. Lue, H. C. et al. Clinical and epidemiological features of rheumatic fever and rheumatic heart disease in Taiwan and the Far East. Indian Heart J. 35, 139–146 (1983).

    CAS  PubMed  Google Scholar 

  58. Manyemba, J. & Mayosi, B. M. Penicillin for secondary prevention of rheumatic fever. Cochrane Database of Systematic Reviews 2002, Issue 3. Art No.: CD002227. http://dx.doi.org/10.1002/14651858.CD002227.

  59. WHO. WHO Expert Consultation on Rheumatic Fever and Rheumatic Heart Disease (Geneva, 29 October–1 November 2001). WHO Technical Report Series 923 (2004).

  60. Markowitz, M. & Lue, H. C. Allergic reactions in rheumatic fever patients on long-term benzathine penicillin G: the role of skin testing for penicillin allergy. Pediatrics 97, 981–983 (1996).

    CAS  PubMed  Google Scholar 

  61. Lue, H. C., Chen, C. L. & Wei, H. Some problems in long-term prevention of streptococcal infection among children with rheumatic heart disease in Taiwan. Jpn Heart J. 17, 550–559 (1976).

    Article  CAS  PubMed  Google Scholar 

  62. Shah, B. et al. (2012). Rheumatic heart disease: progress and challenges in India. Indian J. Pediatr. http://dx.doi.org/10.1007/s12098-012-0853-2.

  63. International Rheumatic Fever Study Group. Allergic reactions to long-term benzathine penicillin prophylaxis for rheumatic fever. Lancet 337, 1308–1310 (1991).

  64. Carapetis, J. R. & Zühlke, L. J. Global research priorities in rheumatic fever and rheumatic heart disease. Ann. Pediatr. Cardiol. 4, 4–12 (2011).

    Article  PubMed  PubMed Central  Google Scholar 

  65. Pelajo, C. F., Lopez-Benitez, J. M., Torres, J. M. & de Oliveira, S. K. Adherence to secondary prophylaxis and disease recurrence in 536 Brazilian children with rheumatic fever. Pediatr. Rheumatol. Online J. 8, 22 (2010).

    Article  PubMed  PubMed Central  Google Scholar 

  66. Stewart, T., McDonald, R. & Currie, B. Acute rheumatic fever: adherence to secondary prophylaxis and follow up of Indigenous patients in the Katherine region of the Northern Territory. Aust. J. Rural Health 15, 234–240 (2007).

    Article  PubMed  Google Scholar 

  67. Nordet, P. WHO programme for the prevention of rheumatic fever/rheumatic heart disease in 16 developing countries: report from Phase I (1986–90). WHO Cardiovascular Diseases Unit and principal investigators. Bull. World Health Organ. 70, 213–218 (1992).

    Google Scholar 

  68. Steer, A. C. et al. High prevalence of rheumatic heart disease by clinical and echocardiographic screening among children in Fiji. J. Heart Valve Dis. 18, 327–335 (2009).

    PubMed  Google Scholar 

  69. Reeves, B. M., Kado, J. & Brook, M. High prevalence of rheumatic heart disease in Fiji detected by echocardiography screening. J. Paediatr. Child Health 47, 473–478 (2011).

    Article  PubMed  Google Scholar 

  70. Mirabel, M. et al. Screening for rheumatic heart disease: evaluation of a simplified echocardiography-based approach. Eur. Heart J. Cardiovasc. Imaging http://dx.doi.org/10.1093/ehjci/jes077.

  71. Marijon, E. et al. Rheumatic heart disease screening by echocardiography: the inadequacy of World Health Organization criteria for optimizing the diagnosis of subclinical disease. Circulation 120, 663–668 (2009).

    Article  PubMed  Google Scholar 

  72. Woolf, S. H. & Harris, R. The harms of screening: new attention to an old concern. JAMA 307, 565–566 (2012).

    Article  PubMed  Google Scholar 

  73. Peckham, C. S. & Dezateux, C. Issues underlying the evaluation of screening programmes. Br. Med. Bull. 54, 767–778 (1998).

    Article  CAS  PubMed  Google Scholar 

  74. Carapetis, J., Parr, J. & Cherian, T. Standardization of epidemiologic protocols for surveillance of post-streptococcal sequaelae: acute rhematic fever, rheumatic heart disease and acute post-streptococal glomerulonephritis. National Institute of Allergy and Infectious Diseases [online], (2006).

    Google Scholar 

  75. Bhaya, M., Beniwal, R., Panwar, S. & Panwar, R. B. Two years of follow-up validates the echocardiographic criteria for the diagnosis and screening of rheumatic heart disease in asymptomatic populations. Echocardiography 28, 929–933 (2011).

    Article  PubMed  Google Scholar 

  76. Anabwani, G. M. & Bonhoeffer, P. Prevalence of heart disease in school children in rural Kenya using colour-flow echocardiography. East Afr. Med. J. 73, 215–217 (1996).

    CAS  PubMed  Google Scholar 

  77. Freedman, B. Equipoise and the ethics of clinical research. N. Engl. J. Med. 317, 141–145 (1987).

    Article  CAS  PubMed  Google Scholar 

  78. Michaud, C. & Narula, J. in Rheumatic Fever (eds Narula, J., Virmani, R., Reddy, K. S. & Tandon, R.) 485–497 (American Registry of Pathology, Washington, DC, 1999).

    Google Scholar 

  79. Strasser, T. Cost-effective control of rheumatic fever in the community. Health Policy 5, 159–164 (1985).

    Article  CAS  PubMed  Google Scholar 

  80. Michaud, C., Gutierrez, J., Cruz, C. & Pearson, T. The World Bank Health Sector Priorities Review. Rheumatic Heart Disease 1–31 (World Bank, Washington, DC, 1991).

    Google Scholar 

  81. North, D. A., Heynes, R. A., Lennon, D. R. & Neutze, J. Analysis of costs of acute rheumatic fever and rheumatic heart disease in Auckland. N. Z. Med. J. 106, 400–403 (1993).

    CAS  PubMed  Google Scholar 

  82. Sliwa, K. & Zilla, P. Rheumatic heart disease: the tip of the iceberg. Circulation 125, 3060–3062 (2012).

    Article  PubMed  Google Scholar 

  83. Colquhoun, S. M., Carapetis, J. R., Kado, J. H. & Steer, A. C. Rheumatic heart disease and its control in the Pacific. Expert Rev. Cardiovasc. Ther. 7, 1517–1524 (2009).

    Article  PubMed  Google Scholar 

  84. Viali, S., Saena, P. & Futi, V. Rheumatic Fever Programme in Samoa. N. Z. Med. J. 124, 26–35 (2011).

    PubMed  Google Scholar 

  85. Viali, S. Rheumatic fever and rheumatic heart disease in Samoa. Pac. Health Dialog. 13, 31–38 (2006).

    PubMed  Google Scholar 

  86. Colquhoun, S. M. et al. Pilot study of nurse-led rheumatic heart disease echocardiography screening in Fiji—a novel approach in a resource-poor setting. Cardiol. Young 8, 1–7 (2012).

    Google Scholar 

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  1. Menzies School of Health Research, Charles Darwin University, John Mathews Building (Building 58), Royal Darwin Hospital Campus, Rocklands Drive, Casuarina, Darwin, 0810, NT, Australia

    Kathryn Roberts, Samantha Colquhoun & Bo Reményi

  2. Centre for International Child Health, The Royal Children's Hospital, University of Melbourne, 50 Flemington Road, Parkville, Melbourne, 3052, VIC, Australia

    Andrew Steer

  3. Telethon Institute for Child Health Research, Centre for Child Health Research, University of Western Australia, 100 Roberts Road, Subiaco, Perth, 6008, WA, Australia

    Jonathan Carapetis

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K. Roberts researched data for the article. K. Roberts, S. Colquhoun, A. Steer, B. Reményi, and J. Carapetis contributed substantially to the discussion of content. The article was written by K. Roberts. All authors reviewed/edited the manuscript prior to submission.

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Roberts, K., Colquhoun, S., Steer, A. et al. Screening for rheumatic heart disease: current approaches and controversies. Nat Rev Cardiol 10, 49–58 (2013). https://doi.org/10.1038/nrcardio.2012.157

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