Skip to main content
Download PDF

Screening for rheumatic heart disease: is a paradigm shift required?

Echo Research & Practice volume 4, pages R43–R52 (2017)Cite this article

Abstract

This focused review presents a critical appraisal of the World Heart Federation criteria for the echocardiographic diagnosis of rheumatic heart disease (RHD) and its performance in African RHD screening programmes. It identifies various logistical and methodological problems that negatively influence the current guideline’s performance. The authors explore novel RHD screening methodology that could address some of these shortcomings and if proven to be of merit, would require a paradigm shift in the approach to the echocardiographic diagnosis of subclinical RHD.

References

  1. Marijon E, Mirabel M, Celermajer DS, Jouven X 2012 Rheumatic heart disease. Lancet 379 953–964. (doi:10.1016/S0140-6736(11)61171-9)

    Article  PubMed  Google Scholar 

  2. Weinberg J, Beaton A, Aliku T, Lwabi P, Sable C 2017 Prevalence of rheumatic heart disease in African school-aged population: extrapolation from echocardiography screening using the 2012 World Heart Federation Guidelines. International Journal of Cardiology 202 238–239. (doi:10.1016/j.ijcard.2015.08.128)

    Article  Google Scholar 

  3. Watkins D, Zuhlke L, Engel M, Daniels R, Francis V, Shaboodien G, Kango M, Abul-Fadl A, Adeoye A, Ali S, et al. 2016 Seven key actions to eradicate rheumatic heart disease in Africa: the Addis Ababa communiqué. Cardiovascular Journal of Africa 27 184–187. (doi:10.5830/CVJA-2015-090)

    Article  PubMed  PubMed Central  Google Scholar 

  4. Stollerman G, Rusoff J 1952 Prophylaxis against group a streptococcal infections in rheumatic fever patients: use of new repository penicillin preparation. JAMA 150 1571–1575. (doi:10.1001/jama.1952.03680160021005)

    Article  CAS  Google Scholar 

  5. Tompkins DG, Boxerbaum B, Liebman J 1972 Long-term prognosis of rheumatic fever patients receiving regular intramuscular benzathine penicillin. Circulation 45 543–551. (doi:10.1161/01.CIR.45.3.543)

    Article  CAS  PubMed  Google Scholar 

  6. Majeed HA, Batnager S, Yousof AM, Khuffash F, Yusuf AR 1992 Acute rheumatic fever and the evolution of rheumatic heart disease: a prospective 12 year follow-up report. Journal of Clinical Epidemiology 45 871–875. (doi:10.1016/0895-4356(92)90070-4)

    Article  CAS  PubMed  Google Scholar 

  7. Roberts K, Colquhoun SM, Steer AC, Remenyi B 2013 Screening for rheumatic heart disease: current approaches and controversies. Nature Reviews Cardiology 10 49–58. (doi:10.1038/nrcardio.2012.157)

    Article  CAS  PubMed  Google Scholar 

  8. Oli K, Tekle-Haimanot R, Forsgren L, Ekstedt J 1992 Rheumatic heart disease prevalence among schoolchildren of an Ethiopian rural town. Cardiology 80 152–155. (doi:10.1159/000174993)

    Article  CAS  PubMed  Google Scholar 

  9. McLaren MJ, Hawkins DM, Koornhof HJ, Bloom KR, Bramwell-Jones DM, Cohen E, Gale GE, Kanarek K, Lachman AS, Lakier JB, et al. 1975 Epidemiology of rheumatic heart disease in black schoolchildren of Soweto, Johannesburg. BMJ 3 474–478. (doi:10.1136/bmj.3.5981.474)

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  10. Maharaj B, Dyer R, Leary WP, Arbuckle DD, Armstrong TG, Pudifin DJ 1987 Screening for rheumatic heart disease amongst black schoolchildren in Inanda, South Africa. Journal of Tropical Pediatrics 33 60–61. (doi:10.1093/tropej/33.1.60)

    Article  CAS  PubMed  Google Scholar 

  11. Anabwani GM, Amoa AB, Muita AK 1989 Epidemiology of rheumatic heart disease among primary school children in western Kenya. International Journal of Cardiology 23 249–252. (doi:10.1016/0167-5273(89)90254-4)

    Article  CAS  PubMed  Google Scholar 

  12. Longo-Mbenza B, Bayekula M, Ngiyulu R, Kintoki VE, Bikangi NF, Seghers KV, Lukoki LE, Mandundu MF, Manzanza M, Nlandu Y 1998 Survey of rheumatic heart disease in school children of Kinshasa town. International Journal of Cardiology 63 287–294. (doi:10.1016/S0167-5273(97)00311-2)

    Article  CAS  PubMed  Google Scholar 

  13. Marijon E, Ou P, Celermajer DS, Ferreira B, Mocumbi AO, Jani D, Paquet C, Jacob S, Sidi D, Jouven X 2007 Prevalence of rheumatic heart disease detected by echocardiographic screening. New England Journal of Medicine 357 470–476. (doi:10.1056/NEJMoa065085)

    Article  CAS  PubMed  Google Scholar 

  14. Anabwani GM, Bonhoeffer P 1996 Prevalence of heart disease in school children in rural Kenya using colour-flow echocardiography. East African Medical Journal 73 215–217.

    CAS  PubMed  Google Scholar 

  15. Danbauchi SS, Alhassan MA, David SO, Wammanda R, Oyati IA 2004 Spectrum of rheumatic heart disease in Zaria. Annals of African Medicine 3 17–21.

    Google Scholar 

  16. Lubega S, Aliku T, Lwabi P, Aliku T 2014 Echocardiographic pattern and severity of valve dysfunction in children with rheumatic heart disease seen at Uganda Heart Institute, Mulago hospital. African Health Sciences 14 617–625. (doi:10.4314/ahs.v14i3.17)

    Article  PubMed  PubMed Central  Google Scholar 

  17. Kane A, Mirabel M, Touré K, Périer MC, Fazaa S, Tafflet M, Karam N, Zourak I, Diagne D, Mbaye A, et al. 2013 Echocardiographic screening for rheumatic heart disease: age matters. International Journal of Cardiology 168 888–891. (doi:10.1016/j.ijcard.2012.10.090)

    Article  PubMed  Google Scholar 

  18. Beaton A, Okello E, Lwabi P, Mondo C, McCarter R, Sable C 2012 Echocardiography screening for rheumatic heart disease in Ugandan schoolchildren. Circulation 125 3127–3132. (doi:10.1161/CIRCULATIONAHA.112.092312)

    Article  PubMed  Google Scholar 

  19. Sadoh WE, Omuemu VO 2013 Prevalence of rheumatic heart disease among primary school pupils in Mid-Western Nigeria. East African Medical Journal 90 28–32.

    CAS  PubMed  Google Scholar 

  20. Sriha A, Abdelka K, El S, Abroug H, Ben A, Bouanene I, Bouanene I, Hassine F, Amara A, Bhiri S, et al. 2017 Rheumatic heart disease in a developing country: incidence and trend (Monastir; Tunisia: 2000–2013). International Journal of Cardiology 228 628–632. (doi:10.1016/j.ijcard.2016.11.249)

    Article  Google Scholar 

  21. Remenyi B, Wilson N, Steer A, Ferreira B, Kado J, Kumar K, Lawrenson J, Maguire G, Marijon E, Mirabel M, et al. 2012 World Heart Federation criteria for echocardiographic diagnosis of rheumatic heart disease–an evidence-based guideline. Nature Reviews Cardiology 9 297–309. (doi:10.1038/nrcardio.2012.7)

    Article  PubMed  PubMed Central  Google Scholar 

  22. Sims Sanyahumbi A, Sable CA, Beaton A, Chimalizeni Y, Guffey D, Hosseinipour M, Karlsten M, Kazembe PN, Kennedy N, Minard CG, et al. 2016 School and community screening shows Malawi, Africa, to have a high prevalence of latent rheumatic heart disease. Congenital Heart Disease 11 615–621. (doi:10.1111/chd.12353)

    Article  Google Scholar 

  23. Engel ME, Haileamlak A, Zühlke L, Lemmer CE, Nkepu S, van de Wall M, Daniel W, Shung King M, Mayosi BM 2015 Prevalence of rheumatic heart disease in 4720 asymptomatic scholars from South Africa and Ethiopia. Heart 101 1389–1394. (doi:10.1136/heartjnl-2015-307444)

    Article  CAS  PubMed  Google Scholar 

  24. Godown J, Lu JC, Beaton A, Sable C, Mirembe G, Sanya R, Aliku T, Yu S, Lwabi P, Webb CL, et al. 2015 Handheld echocardiography versus auscultation for detection of rheumatic heart disease. Pediatrics 135 e939–e944. (doi:10.1542/peds.2014-2774)

    Article  PubMed  Google Scholar 

  25. Beaton A, Okello E, Aliku T, Lubega S, Lwabi P, Mondo C, McCarter R, Sable C 2014 Latent rheumatic heart disease: outcomes 2 years after echocardiographic detection. Pediatric Cardiology 35 1259–1267. (doi:10.1007/s00246-014-0925-3)

    Article  PubMed  Google Scholar 

  26. Rossi E, Felici AR, Banteyrga L 2014 Subclinical rheumatic heart disease in an Eritrean high-school population, detected by echocardiography. Journal of Heart Valve Disease 23 235–239.

    PubMed  Google Scholar 

  27. Yadeta D, Hailu A, Haileamlak A, Gedlu E, Guteta S, Tefera E, Tigabu Z, Tesfaye H, Daniel W, Mekonnen D, et al. 2017 Prevalence of rheumatic heart disease among school children in Ethiopia: a multisite echocardiography-based screening. International Journal of Cardiology 221 260–263. (doi:10.1016/j.ijcard.2016.06.232)

    Article  Google Scholar 

  28. Ngaïdé AA, Mbaye A, Kane A, Ndiaye MB, Jobe M, Bodian M, Dioum M, Sarr SA, Aw F, Mbakop PS, et al. 2015 Prevalence of rheumatic heart disease in Senegalese school children: a clinical and echocardiographic screening. Heart Asia 7 40–45.

    Article  PubMed  PubMed Central  Google Scholar 

  29. Beaton A, Lu JC, Aliku T, Dean P, Gaur L, Weinberg J, Godown J, Lwabi P, Mirembe G, Okello E, et al. 2015 The utility of handheld echocardiography for early rheumatic heart disease diagnosis: a field study. European Heart Journal 16 475–482.

    PubMed  PubMed Central  Google Scholar 

  30. Lu JC, Sable C, Ensing GJ, Webb C, Scheel J, Aliku T, Lwabi P, Godown J, Beaton A 2016 Simplified rheumatic heart disease screening criteria for handheld echocardiography. Journal of the American Society of Echocardiography 28 463–469. (doi:10.1016/j.echo.2015.01.001)

    Article  Google Scholar 

  31. Ploutz M, Lu JC, Scheel J, Webb C, Ensing GJ, Aliku T, Lwabi P, Sable C, Beaton A 2016 Handheld echocardiographic screening for rheumatic heart disease by non-experts. Heart 102 35–39. (doi:10.1136/heartjnl-2015-308236)

    Article  PubMed  Google Scholar 

  32. Sliwa K, Carrington M, Mayosi BM, Zigiriadis E, Mvungi R, Stewart S 2010 Incidence and characteristics of newly diagnosed rheumatic heart disease in urban African adults: insights from the heart of Soweto study. European Heart Journal 31 719–727. (doi:10.1093/eurheartj/ehp530)

    Article  PubMed  Google Scholar 

  33. Bacquelin R, Tafflet M, Rouchon B, Guillot N, Marijon E, Jouven X, Mirabel M 2016 Echocardiography-based screening for rheumatic heart disease: what does borderline mean? International Journal of Cardiology 203 1003–1004. (doi:10.1016/j.ijcard.2015.11.110)

    Article  PubMed  Google Scholar 

  34. Grimaldi A, Ammirati E, Mirabel M, Marijon E 2017 Challenges of using ultrasounds for subclinical rheumatic heart disease screening. International Journal of Cardiology 167 3061. (doi:10.1016/j.ijcard.2012.11.083)

    Article  Google Scholar 

  35. Roberts AK, Colquhoun S, Steer A, Reményi B, Carapetis J 2013 Screening for rheumatic heart disease: current approaches and controversies. Nature Reviews Cardiology 10 49–58. (doi:10.1038/nrcardio.2012.157)

    Article  CAS  PubMed  Google Scholar 

  36. Anyangwe SCE, Mtonga C 2007 Inequities in the global health workforce: the greatest impediment to health in Sub-Saharan Africa. International Journal of Environmental Research and Public Health 4 93–100. (doi:10.3390/ijerph2007040002)

    Article  PubMed  PubMed Central  Google Scholar 

  37. Zühlke L, Engel ME, Lemmer CE, van de Wall M, Nkepu S, Meiring A, Bestawros M, Mayosi BM 2016 The natural history of latent rheumatic heart disease in a 5 year follow-up study: a prospective observational study. BMC Cardiovascular Disorders 16 46. (doi:10.1186/s12872-016-0225-3)

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  38. General Electric 2014 Technical Publications, Vscan, Version 1. Horten, Norway: GE Vingmed Ultrasound AS. (available at: http://www3.gehealthcare.com/~/media/documents/us-global/products/ultrasound/clinical%20product%20information/vscan/gehc-user-manual_vscan-1-2.pdf)

    Google Scholar 

  39. Engel ME, Nkepu S, Mayosi BM, Clinic TC, Hospital GS, Health C 2016 Evaluation of a focussed protocol for hand-held echocardiography and computer-assisted auscultation in detecting latent rheumatic heart disease in scholars. Cardiology in the Young 26 1097–1106. (doi:10.1017/S1047951115001857)

    Article  PubMed  Google Scholar 

  40. Mirabel M, Bacquelin R, Tafflet M, Robillard C, Huon B, Corsenac P, de Frémicourt I, Narayanan K, Meunier JM, Noël B, et al. 2014 Screening for rheumatic heart disease: evaluation of a focused cardiac ultrasound approach. Circulation: Cardiovascular Imaging 8 e002324.

    Google Scholar 

  41. Mirabel M, Celermajer DS, Ferreira B, Tafflet M, Perier MC, Karam N, Mocumbi AO, Jani DN, Sidi D, Jouven X, et al. 2012 Screening for rheumatic heart disease: evaluation of a simplified echocardiography-based approach. European Heart Journal: Cardiovascular Imaging 13 1024–1029. (doi:10.1093/ehjci/jes077)

    PubMed  Google Scholar 

  42. Herbst P 2015 Screening for asymptomatic rheumatic heart disease: Understanding the mechanisms key to the diagnostic criteria. SA Heart 12 134–144.

    Google Scholar 

  43. Marijon E, Celermajer DS, Tafflet M, El-Haou S, Jani DN, Ferreira B, Mocumbi A-O, Paquet C, Sidi D, Jouven X 2009 Rheumatic heart disease screening by echocardiography: the inadequacy of World Health Organization criteria for optimizing the diagnosis of subclinical disease. Circulation 120 663–668. (doi:10.1161/CIRCULATIONAHA.109.849190)

    Article  PubMed  Google Scholar 

  44. Woolf SH, Harris R 2012 The harms of screening: new attention to an old concern. JAMA 307 565–566.

    Article  PubMed  Google Scholar 

  45. Kostucki W, Vandenbossche JL, Friart A, Englert M 1986 Pulsed Doppler regurgitant flow patterns of normal valves. American Journal of Cardiology 58 309–313. (doi:10.1016/0002-9149(86)90068-8)

    Article  CAS  PubMed  Google Scholar 

  46. Berger M, Hecht SR, Van Tosh A, Lingam U 1989 Pulsed and continuous wave Doppler echocardiographic assessment of valvular regurgitation in normal subjects. Journal of the American College of Cardiology 13 1540–1545. (doi:10.1016/0735-1097(89)90345-8)

    Article  CAS  PubMed  Google Scholar 

  47. Choong CY, Abascal VM, Weyman J, Levine RA, Gentile F, Thomas JD, Weyman AE 1989 Prevalence of valvular regurgitation by Doppler echocardiography in patients with structurally normal hearts by two-dimensional echocardiography. American Heart Journal 117 636–642. (doi:10.1016/0002-8703(89)90739-4)

    Article  CAS  PubMed  Google Scholar 

  48. Yoshida K, Yoshikawa J, Shakudo M, Akasaka T, Jyo Y, Takao S, Shiratori K, Koizumi K, Okumachi F, Kato H, et al. 1988 Color Doppler evaluation of valvular regurgitation in normal subjects. Circulation 78 840–847. (doi:10.1161/01.CIR.78.4.840)

    Article  CAS  PubMed  Google Scholar 

  49. Wilson NJ, Neutze JM 1995 Echocardiographic diagnosis of subclinical carditis in acute rheumatic fever. International Journal of Cardiology 50 1–6. (doi:10.1016/0167-5273(95)02325-Q)

    Article  CAS  PubMed  Google Scholar 

  50. Minich LL, Tani LY, Pagotto LT, Shaddy RE, Veasy LG 1997 Doppler echocardiography distinguishes between physiologic and pathologic ‘silent’ mitral regurgitation in patients with rheumatic fever. Clinical Cardiology 20 924–926. (doi:10.1002/clc.4960201105)

    Article  CAS  PubMed  Google Scholar 

  51. WHO 2001 Rheumatic fever and rheumatic heart disease. World Health Organization Technical Report Series 923 1–122. (available at: http://www.who.int/cardiovascular_diseases/publications/trs923/en/)

    Google Scholar 

  52. Webb RH, Gentles TL, Stirling JW, Lee M, O’Donnell C, Wilson NJ 2015 Valvular regurgitation using portable echocardiography in a healthy student population: implications for rheumatic heart disease screening. Journal of the American Society of Echocardiography 28 981–988. (doi:10.1016/j.echo.2015.03.012)

    Article  PubMed  Google Scholar 

  53. Webb RH, Wilson NJ, Lennon DR, Wilson EM, Nicholson RW, Gentles TL, O’Donnell CP, Stirling JW, Zeng I, Trenholme AA 2011 Optimising echocardiographic screening for rheumatic heart disease in New Zealand: not all valve disease is rheumatic. Cardiology in the Young 21 436–443. (doi:10.1017/S1047951111000266)

    Article  PubMed  Google Scholar 

  54. Roberts K, Maguire G, Brown A, Atkinson D, Reményi B, Wheaton G, Kelly A, Kumar RK, Su JY, Carapetis JR 2014 Echocardiographic screening for rheumatic heart disease in high and low risk Australian children. Circulation 129 1953–1961. (doi:10.1161/CIRCULATIONAHA.113.003495)

    Article  PubMed  Google Scholar 

  55. Colquhoun SM, Kado JH, Remenyi B, Wilson NJ, Carapetis JR, Steer AC 2014 Echocardiographic screening in a resource poor setting: borderline rheumatic heart disease could be a normal variant. International Journal of Cardiology 173 284–289. (doi:10.1016/j.ijcard.2014.03.004)

    Article  CAS  PubMed  Google Scholar 

  56. Saxena A, Zühlke L, Wilson N 2013 Echocardiographic screening for rheumatic heart disease. Global Heart 8 197–202. (doi:10.1016/j.gheart.2013.08.004)

    Article  PubMed  Google Scholar 

  57. Ring L, Rana BS, Ho SY, Wells FC 2013 The prevalence and impact of deep clefts in the mitral leaflets in MV prolapse. European Heart Journal: Cardiovascular Imaging 14 595–602. (doi:10.1093/ehjci/jes310)

    PubMed  Google Scholar 

  58. La Canna G, Arendar I, Maisano F, Monaco F, Collu E, Benussi S, De Bonis M, Castiglioni A, Alfieri O 2017 Real-time three-dimensional transesophageal echocardiography for assessment of mitral valve functional anatomy in patients with prolapse-related regurgitation. American Journal of Cardiology 107 1365–1374. (doi:10.1016/j.amjcard.2010.12.048)

    Article  Google Scholar 

  59. Victor S, Nayak VM 1994 Definition and function of commissures, slits and scallops of the mitral valve: analysis in 100 hearts. Asia Pacific Journal of Thoracic and Cardiovascular Surgery 3 10–16. (doi:10.1016/1324-2881(94)90050-7)

    Article  Google Scholar 

  60. Zuhlke L, Mirabel M, Marijon E 2013 Congenital heart disease and rheumatic heart disease in Africa: recent advances and current priorities. Heart 99 1554–1561. (doi:10.1136/heartjnl-2013-303896)

    Article  PubMed  Google Scholar 

  61. Zühlke L, Mayosi BM 2013 Echocardiographic screening for subclinical rheumatic heart disease remains a research tool pending studies of impact on prognosis. Current Cardiology Reports 15 343.

    Article  PubMed  Google Scholar 

  62. Rémond M, Atkinson D, White A, Brown A, Carapetis J, Remenyi B, Roberts K, Maguire G 2016 Are minor echocardiographic changes associated with an increased risk of acute rheumatic fever or progression to rheumatic heart disease? International Journal of Cardiology 198 117–122. (doi:10.1016/j.ijcard.2015.07.005)

    Article  Google Scholar 

  63. Bertaina G, Rouchon B, Huon B, Guillot N, Robillard C, Noel B, Nadra M, Tribouilloy C, Marijon E, Jouven X, et al. 2017 Outcomes of borderline rheumatic heart disease: a prospective cohort study. International Journal of Cardiology 228 661–665. (doi:10.1016/j.ijcard.2016.11.234)

    Article  PubMed  Google Scholar 

  64. Zoghbi WA, Adams D, Bonow RO, Enriquez-Sarano M, Foster E, Grayburn PA, Hahn RT, Han Y, Hung J, Lang RM, et al. 2017 Recommendations for noninvasive evaluation of native valvular regurgitation. Journal of the American Society of Echocardiography 30 303–371. (doi:10.1016/j.echo.2017.01.007)

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Division of Cardiology, Tygerberg Hospital and University of Stellenbosch, Cape Town, South Africa

    L. D. Hunter, A. J. K. Pecoraro, A. F. Doubell & P. G. Herbst

  2. King’s College Hospital NHS Trust, London, UK

    M. Monaghan

  3. Department of Echocardiography, Barts Heart Centre, St Bartholomew’s Hospital, London, UK

    G. Lloyd

Authors
  1. L. D. Hunter
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. M. Monaghan
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. G. Lloyd
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. A. J. K. Pecoraro
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. A. F. Doubell
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. P. G. Herbst
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to L. D. Hunter.

Rights and permissions

Open Access This article is distributed under the terms of the Creative Commons Attribution 4.0 International License (https://doi.org/creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made.

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Hunter, L.D., Monaghan, M., Lloyd, G. et al. Screening for rheumatic heart disease: is a paradigm shift required?. Echo Res Pract 4, R43–R52 (2017). https://doi.org/10.1530/ERP-17-0037

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1530/ERP-17-0037

Key Words

Echo Research & Practice

ISSN: 2055-0464

Contact us