Diagnosing rheumatic heart disease: where are we now and what are the challenges?

References

• Roth GA, Mensah GA, Johnson CO, et al. Global burden of cardiovascular diseases and risk factors, 1990-2019: update from the GBD 2019 study. J Am Coll Cardiol. 2020 Dec 22;76(25):2982–3021.
   PubMed Web of Science ®Google Scholar
• Watkins DA, Johnson CO, Colquhoun SM, et al. Global, regional, and national burden of rheumatic heart disease, 1990–2015. N Engl J Med. 2017;377(8):713–722.
   PubMed Web of Science ®Google Scholar
• Tormin J, Nascimento BR, Sable CA, et al. Cytokine gene functional polymorphisms and phenotypic expression as predictors of evolution from latent to clinical rheumatic heart disease. Cytokine. 2021 Feb;138:155370.
   PubMed Web of Science ®Google Scholar
• Cunningham MW. Rheumatic fever, autoimmunity, and molecular mimicry: the streptococcal connection. Int Rev Immunol. 2014 Jul-Aug;33(4):314–329.
   PubMed Web of Science ®Google Scholar
• Dougherty S, Beaton A, Nascimento BR, et al. Prevention and control of rheumatic heart disease: overcoming core challenges in resource-poor environments. Ann Pediatr Cardiol. 2018 Jan-Apr;11(1):68–78.
   PubMed Web of Science ®Google Scholar
• Islam AK, Majumder AA. Rheumatic fever and rheumatic heart disease in Bangladesh: a review. Indian Heart J. 2016 Jan-Feb;68(1):88–98.
   PubMedGoogle Scholar
• Noubiap JJ, Agbor VN, Bigna JJ, et al. Prevalence and progression of rheumatic heart disease: a global systematic review and meta-analysis of population-based echocardiographic studies. Sci Rep. 2019 Nov 19;9(1):17022.
   PubMedGoogle Scholar
• Ordunez P, Martinez R, Soliz P, et al. Rheumatic heart disease burden, trends, and inequalities in the Americas, 1990-2017: a population-based study. Lancet Glob Health. 2019 Oct;7(10):e1388–e1397.
   PubMed Web of Science ®Google Scholar
• Katzenellenbogen JM, Bond-Smith D, Seth RJ, et al. Contemporary incidence and prevalence of rheumatic fever and rheumatic heart disease in Australia using linked data: the case for policy change. J Am Heart Assoc. 2020 Oct 20;9(19):e016851.
   PubMed Web of Science ®Google Scholar
• Baker MG, Gurney J, Oliver J, et al. Risk factors for acute rheumatic fever: literature review and protocol for a case-control study in New Zealand. Int J Environ Res Public Health. 2019 Nov 15;16(22):4515.
   Web of Science ®Google Scholar
• Oliver J, Robertson O, Zhang J, et al. Ethnically disparate disease progression and outcomes among acute rheumatic fever patients in New Zealand, 1989-2015. Emerg Infect Dis. 2021 Jul;27(7):1893–1902.
   Web of Science ®Google Scholar
• Beaudoin A, Edison L, Introcaso CE, et al. Acute rheumatic fever and rheumatic heart disease among children–American Samoa, 2011-2012. MMWR Morb Mortal Wkly Rep. 2015 May 29;64(20):555–558.
   PubMed Web of Science ®Google Scholar
• Schaffer WL, Galloway JM, Roman MJ, et al. Prevalence and correlates of rheumatic heart disease in American Indians (the Strong Heart Study). Am J Cardiol. 2003 Jun 1;91(11):1379–1382.
   PubMed Web of Science ®Google Scholar
• Johnston JM, Day GE, Veazie MA, et al. Heart disease mortality among Alaska Native people, 1981-2007. 433021. 2011 Jan-Feb;126(1):73–83.
   Google Scholar
• Gewitz MH, Baltimore RS, Tani LY, et al. Revision of the Jones Criteria for the diagnosis of acute rheumatic fever in the era of Doppler echocardiography: a scientific statement from the American Heart Association. Circulation. 2015 May 19;131(20):1806–1818.
   PubMed Web of Science ®Google Scholar
• Karthikeyan G, Guilherme L. Acute rheumatic fever. Lancet. 2018 Jul 14;392(10142):161–174.
   PubMed Web of Science ®Google Scholar
• Carapetis JR, Beaton A, Cunningham MW, et al. Acute rheumatic fever and rheumatic heart disease. Nat Rev Dis Primers. 2016 Jan 14;2:15084.
   PubMed Web of Science ®Google Scholar
• 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). Eur Heart J. 2015 May 7;36(18):1115–22a.
   PubMed Web of Science ®Google Scholar
• 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. 1992;70(2):213–218.
   PubMedGoogle Scholar
• Anabwani GM, Bonhoeffer P. Prevalence of heart disease in school children in rural Kenya using colour-flow echocardiography. East Afr Med J. 1996 Apr;73(4):215–217.
   PubMed Web of Science ®Google Scholar
• Marijon E, Ou P, Celermajer DS, et al. Prevalence of rheumatic heart disease detected by echocardiographic screening. N Engl J Med. 2007 Aug 2;357(5):470–476.
   PubMed Web of Science ®Google Scholar
• Zühlke LJ, Engel ME, Nkepu S, et al. Evaluation of a focussed protocol for hand-held echocardiography and computer-assisted auscultation in detecting latent rheumatic heart disease in scholars. Cardiol Young. 2016 Aug;26(6):1097–1106.
   PubMed Web of Science ®Google Scholar
• Colquhoun SM, Kado JH, Remenyi B, et al. Echocardiographic screening in a resource poor setting: borderline rheumatic heart disease could be a normal variant. Int J Cardiol. 2014 May 1;173(2):284–289.
   PubMed Web of Science ®Google Scholar
• Essop MR, Mayosi BM. Echocardiographic detection of latent rheumatic heart disease: a Pandora’s box? Circulation. 2017 Dec 5;136(23):2245–2247.
   PubMed Web of Science ®Google Scholar
• Reményi B, Wilson N, Steer A, et al. World Heart Federation criteria for echocardiographic diagnosis of rheumatic heart disease–an evidence-based guideline. Nat Rev Cardiol. 2012;9(5):297–309.
   PubMed Web of Science ®Google Scholar
• Beaton A, Aliku T, Dewyer A, et al. Latent Rheumatic heart disease: identifying the children at highest risk of unfavorable outcome. Circulation. 2017 Dec 5;136(23):2233–2244.
   PubMed Web of Science ®Google Scholar
• Nascimento BR, Nunes MC, Lopes EL, et al. Rheumatic heart disease echocardiographic screening: approaching practical and affordable solutions. Heart. 2016 May;102(9):658–664.
   PubMed Web of Science ®Google Scholar
• Nascimento BR, Sable C, Nunes MCP, et al. Comparison Between different strategies of rheumatic heart disease echocardiographic screening in Brazil: data from the PROVAR (Rheumatic Valve Disease Screening Program) Study. J Am Heart Assoc. 2018 Feb 14;7(4). DOI:https://doi.org/10.1161/JAHA.117.008039.
   Google Scholar
• Colquhoun SM, Carapetis JR, Kado JH, et al. Pilot study of nurse-led rheumatic heart disease echocardiography screening in Fiji–a novel approach in a resource-poor setting. Cardiol Young. 2013 Aug;23(4):546–552.
   PubMed Web of Science ®Google Scholar
• Bechtlufft BM, Nascimento BR, Sable C, et al. Validation of a simplified score for predicting latent rheumatic heart disease progression using a prospective cohort of Brazilian schoolchildren. BMJ Open. 2020 May 10;10(5):e036827.
   PubMedGoogle Scholar
• Zühlke L, Engel ME, Lemmer CE, et al. The natural history of latent rheumatic heart disease in a 5 year follow-up study: a prospective observational study. BMC Cardiovasc Disord. 2016 Feb 19;16(1):46.
   PubMedGoogle Scholar
• Gutman SJ, Shemesh E, Marwick TH, et al. Echocardiographic screening to determine progression of latent rheumatic heart disease in endemic areas: a systematic review and meta-analysis. PLoS One. 2020;15(6):e0234196.
   PubMed Web of Science ®Google Scholar
• Beaton A, Okello E, Engelman D, et al. Determining the impact of Benzathine penicillin G prophylaxis in children with latent rheumatic heart disease (GOAL trial): study protocol for a randomized controlled trial. Am Heart J. 2019 Sep;215:95–105.
   PubMed Web of Science ®Google Scholar
• Kotit S, Said K, ElFaramawy A, et al. Prevalence and prognostic value of echocardiographic screening for rheumatic heart disease. Open Heart. 2017;4(2):e000702.
   PubMed Web of Science ®Google Scholar
• Nunes MCP, Sable C, Nascimento BR, et al. Simplified echocardiography screening criteria for diagnosing and predicting progression of latent rheumatic heart disease. Circ Cardiovasc Imaging. 2019 Feb;12(2):e007928.
   PubMed Web of Science ®Google Scholar
• Remenyi B, ElGuindy A, Smith SC Jr., et al. Valvular aspects of rheumatic heart disease. Lancet. 2016 Mar 26;387(10025):1335–1346.
   PubMed Web of Science ®Google Scholar
• Leal M, Passos LSA, Guarçoni FV, et al. Rheumatic heart disease in the modern era: recent developments and current challenges. Rev Soc Bras Med Trop. 2019 Mar 14;52:e20180041.
   PubMed Web of Science ®Google Scholar
• Saxena A. Echocardiographic diagnosis of chronic rheumatic valvular lesions. Glob Heart. 2013 Sep;8(3):203–212.
   PubMedGoogle Scholar
• Passos LSA, Nunes MCP, Aikawa E. Rheumatic heart valve disease pathophysiology and underlying mechanisms. Front Cardiovasc Med. 2020;7:612716.
   PubMed Web of Science ®Google Scholar
• Peters F, Karthikeyan G, Abrams J, et al. Rheumatic heart disease: current status of diagnosis and therapy. Cardiovasc Diagn Ther. 2020 Apr;10(2):305–315.
   PubMed Web of Science ®Google Scholar
• Saxena A, Desai A, Narvencar K, et al. Echocardiographic prevalence of rheumatic heart disease in Indian school children using World Heart Federation criteria - A multi site extension of RHEUMATIC study (the e-RHEUMATIC study). Int J Cardiol. 2017 Dec 15;249:438–442.
   PubMed Web of Science ®Google Scholar
• Horton AGT, Remenyi B. Clinical evaluation and diagnosis of rheumatic heart disease. In: Doughtery SCJ, Zuhlke L, Wilson N, editors. Acute rheumatic fever and rheumatic heart disease. St. Louis, Missouri: Elsevier Inc; 2020. p. 69–106.
   Google Scholar
• Nunes MCP, Tan TC, Elmariah S, et al. Net atrioventricular compliance is an independent predictor of cardiovascular death in mitral stenosis. Heart. 2017 Dec;103(23):1891–1898.
   PubMed Web of Science ®Google Scholar
• Cannon J, Roberts K, Milne C, et al. Rheumatic heart disease severity, progression and outcomes: a multi-state model. J Am Heart Assoc. 2017 Mar 2;6(3). DOI:https://doi.org/10.1161/JAHA.116.003498.
   Web of Science ®Google Scholar
• Cawley PJ, Maki JH, Otto CM. Cardiovascular magnetic resonance imaging for valvular heart disease. Circulation. 2009;119(3):468–478.
   PubMed Web of Science ®Google Scholar
• Shriki J, Talkin B, Thomas IC, et al. Delayed gadolinium enhancement in the atrial wall: a novel finding in 3 patients with rheumatic heart disease. Tex Heart Inst J. 2011;38(1):56–60.
   PubMed Web of Science ®Google Scholar
• Meel R, Nethononda R, Libhaber E, et al. Assessment of myocardial fibrosis by late gadolinium enhancement imaging and biomarkers of collagen metabolism in chronic rheumatic mitral regurgitation. Cardiovasc J Afr. 2018 May/Jun 23;29(3):150–154.
   PubMed Web of Science ®Google Scholar
• Mutnuru PC, Singh SN, D’Souza J, et al. Cardiac MR Imaging in the evaluation of rheumatic valvular heart diseases. J Clin Diagn Res. 2016 Mar;10(3):Tc06–9.
   PubMed Web of Science ®Google Scholar
• Aremu OO, Samuels P, Jermy S, et al. Cardiovascular imaging modalities in the diagnosis and management of rheumatic heart disease. Int J Cardiol. 2021 Feb 15;325:176–185.
   PubMed Web of Science ®Google Scholar
• Otto CM, Nishimura RA, Bonow RO, et al. 2020 ACC/AHA guideline for the management of patients with valvular heart disease: executive summary: a report of the American College of Cardiology/American Heart Association joint committee on clinical practice guidelines. Circulation. 2021 Feb 2;143(5):e35–e71.
   PubMed Web of Science ®Google Scholar
• He VY, 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 Jul 19;134(3):222–232.
   PubMed Web of Science ®Google Scholar
• Kumar RK, Antunes MJ, Beaton A, et al. Contemporary diagnosis and management of rheumatic heart disease: implications for closing the gap: a scientific statement from the American Heart Association. Circulation. 2020 Nov 17;142(20):e337–e357.
   PubMed Web of Science ®Google Scholar
• Diker E, Aydogdu S, Ozdemir M, et al. Prevalence and predictors of atrial fibrillation in rheumatic valvular heart disease. Am J Cardiol. 1996 Jan 1;77(1):96–98.
   PubMed Web of Science ®Google Scholar
• Kim HJ, Cho GY, Kim YJ, et al. Development of atrial fibrillation in patients with rheumatic mitral valve disease in sinus rhythm. Int J Cardiovasc Imaging. 2015 Apr;31(4):735–742.
   PubMed Web of Science ®Google Scholar
• Negi PC, Sondhi S, Rana V, et al. Prevalence, risk determinants and consequences of atrial fibrillation in rheumatic heart disease: 6 years hospital based-Himachal Pradesh- Rheumatic Fever/Rheumatic Heart Disease (HP-RF/RHD) Registry. Indian Heart J. 2018 Dec;70(Suppl3):S68–s73.
   PubMedGoogle Scholar
• Gupta A, Bhatia R, Sharma G, et al. Predictors of Ischemic Stroke in Rheumatic Heart Disease. J Stroke Cerebrovasc Dis. 2015 Dec;24(12):2810–2815.
   PubMed Web of Science ®Google Scholar
• Negi PC, Mahajan K, Rana V, et al. Clinical characteristics, complications, and treatment practices in patients with RHD: 6-year results from HP-RHD registry. Glob Heart. 2018 Dec;13(4):267–274.e2.
   PubMed Web of Science ®Google Scholar
• Karthikeyan G, Ananthakrishnan R, Devasenapathy N, et al. Transient, subclinical atrial fibrillation and risk of systemic embolism in patients with rheumatic mitral stenosis in sinus rhythm. Am J Cardiol. 2014 Sep 15;114(6):869–874.
   PubMed Web of Science ®Google Scholar
• Vasconcelos M, Vasconcelos L, Ribeiro V, et al. Incidence and predictors of stroke in patients with rheumatic heart disease. Heart. 2021 May;107(9):748–754.
   PubMed Web of Science ®Google Scholar
• Beaton A, Okello E, Scheel A, et al. Impact of heart disease on maternal, fetal and neonatal outcomes in a low-resource setting. Heart. 2019 May;105(10):755–760.
   PubMed Web of Science ®Google Scholar
• Robertson KA, Volmink JA, Mayosi BM. Antibiotics for the primary prevention of acute rheumatic fever: a meta-analysis. BMC Cardiovasc Disord. 2005 May 31;5(1):11.
   PubMedGoogle Scholar
• Katzenellenbogen JM, Ralph AP, Wyber R, et al. Rheumatic heart disease: infectious disease origin, chronic care approach. BMC Health Serv Res. 2017 Nov 29;17(1):793.
   PubMedGoogle Scholar
• de Dassel JL, de Klerk N, Carapetis JR, et al. How many doses make a difference? An analysis of secondary prevention of rheumatic fever and rheumatic heart disease. J Am Heart Assoc. 2018 Dec 18;7(24):e010223.
   PubMed Web of Science ®Google Scholar
• Manyemba J, Mayosi BM. Penicillin for secondary prevention of rheumatic fever. Cochrane Database Syst Rev. 2002;2002(3):Cd002227.
   Google Scholar
• Tompkins DG, Boxerbaum B, Liebman J. Long-term prognosis of rheumatic fever patients receiving regular intramuscular benzathine penicillin. Circulation. 1972 Mar;45(3):543–551.
   PubMed Web of Science ®Google Scholar
• Wyber R, Taubert K, Marko S, et al. Benzathine penicillin G for the management of RHD: concerns about quality and access, and opportunities for intervention and improvement. Glob Heart. 2013 Sep;8(3):227–234.
   PubMedGoogle Scholar
• Chamberlain-Salaun J, Mills J, Kevat PM, et al. Sharing success - understanding barriers and enablers to secondary prophylaxis delivery for rheumatic fever and rheumatic heart disease. BMC Cardiovasc Disord. 2016 Aug 31;16(1):166.
   PubMedGoogle Scholar
• Musoke C, Mondo CK, Okello E, et al. Benzathine penicillin adherence for secondary prophylaxis among patients affected with rheumatic heart disease attending Mulago Hospital. Cardiovasc J Afr. 2013 May;24(4):124–129.
   PubMed Web of Science ®Google Scholar
• Barker H, Oetzel JG, Scott N, et al. Enablers and barriers to secondary prophylaxis for rheumatic fever among Māori aged 14-21 in New Zealand: a framework method study. Int J Equity Health. 2017 Nov 17;16(1):201.
   PubMedGoogle Scholar
• Huck DM, Nalubwama H, Longenecker CT, et al. A qualitative examination of secondary prophylaxis in rheumatic heart disease: factors influencing adherence to secondary prophylaxis in Uganda. Glob Heart. 2015 Mar;10(1):63–69.e1.
   PubMed Web of Science ®Google Scholar
• Balbaa A, ElGuindy A, Pericak D, et al. An evaluation of secondary prophylaxis for rheumatic heart disease in rural Egypt. Glob Cardiol Sci Pract. 2015;2015(3):40.
   PubMedGoogle Scholar
• Kaplan EL, Zaher SR. Benzathine Penicillin Formulations. Pediatr Infect Dis J. 2004;23(6):592–593.
   PubMed Web of Science ®Google Scholar
• Broderick MP, Hansen CJ, Russell KL, et al. Serum penicillin G levels are lower than expected in adults within two weeks of administration of 1.2 million units. PLoS One. 2011;6(10):e25308.
   PubMed Web of Science ®Google Scholar
• Inadvertent use of Bicillin C-R to treat syphilis infection–Los Angeles, California, 1999-2004. MMWR Morb Mortal Wkly Rep. 2005 Mar 11;54(9):217–219.
   PubMedGoogle Scholar
• Carapetis JR, Zühlke LJ. Global research priorities in rheumatic fever and rheumatic heart disease. Ann Pediatr Cardiol. 2011 Jan;4(1):4–12.
   PubMedGoogle Scholar
• Maguire GP, Carapetis JR, Walsh WF, et al. The future of acute rheumatic fever and rheumatic heart disease in Australia. Med J Aust. 2012 Aug 6;197(3):133–134.
   PubMed Web of Science ®Google Scholar
• Dale JB, Fischetti VA, Carapetis JR, et al. Group A streptococcal vaccines: paving a path for accelerated development. Vaccine. 2013 Apr 18;31(Suppl 2):B216–22.
   PubMed Web of Science ®Google Scholar
• Guerino MT, Postol E, Demarchi LM, et al. HLA class II transgenic mice develop a safe and long lasting immune response against StreptInCor, an anti-group A streptococcus vaccine candidate. Vaccine. 2011 Oct 26;29(46):8250–8256.
   PubMed Web of Science ®Google Scholar
• Rivera-Hernandez T, Carnathan DG, Jones S, et al. An experimental group a streptococcus vaccine that reduces pharyngitis and tonsillitis in a nonhuman primate model. mBio. 2019 Apr 30;10(2). DOI:https://doi.org/10.1128/mBio.00693-19.
   Google Scholar
• Vekemans J, Gouvea-Reis F, Kim JH, et al. The path to Group A streptococcus vaccines: World Health Organization Research and Development Technology Roadmap and preferred product characteristics. Clin Infect Dis. 2019 Aug 16;69(5):877–883.
   PubMed Web of Science ®Google Scholar
• Osowicki J, Vekemans J, Kaslow DC, et al. WHO/IVI global stakeholder consultation on group A Streptococcus vaccine development: report from a meeting held on 12-13 December 2016. Vaccine. 2018 Jun 7;36(24):3397–3405.
   PubMed Web of Science ®Google Scholar
• Raynes JM, Young PG, Proft T, et al. Protein adhesins as vaccine antigens for Group A Streptococcus. Pathog Dis. 2018 Mar 1;76(2). DOI:https://doi.org/10.1093/femspd/fty016.
   Google Scholar
• Heart University Cincinnati, Ohio: Cincinnati Children’s Hospital Medical Center; [cited 2021 Jun 10]; Free, web-based global education resource and training tool with a focus on acquired and congenital heart disease created by top experts in the field. Available from https://www.heartuniversity.org/
   Google Scholar