Abstract
Purpose of Review
Rheumatic heart disease (RHD) is a neglected disease of poverty, which presents challenges for patients, communities, and health systems. These effects are magnified in low resource countries, which bear the highest disease burden. When considering the impact of RHD, it is imperative that we widen our lens in order to better understand how RHD impacts the over 40 million people currently living with this preventable condition and their communities. We aimed to perform an updated literature review on the global impact of RHD, examining a broad range of aspects from disease burden to impact on healthcare system to socioeconomic implications.
Recent Findings
RHD accounts for 1.6% of all cardiovascular deaths, resulting in 306,000 deaths yearly, with a much higher contribution in low- and middle-income countries, where 82% of the deaths occurred in 2015. RHD can result in severe health adverse outcomes, markedly heart failure, arrhythmias, stroke and embolisms, and ultimately premature death. Thus, preventive, diagnostic and therapeutic interventions are required, although insufficiently available in undersourced settings. As examples, anticoagulation management is poor in endemic regions – and novel oral anticoagulants cannot be recommended – and less than 15% of those in need have access to interventional procedures and valve replacement in Africa.
Summary
RHD global impact remains high and unequally distributed, with a marked impact on lower resourced populations. This preventable disease negatively affects not only patients, but also the societies and health systems within which they live, presenting broad challenges and high costs along the pathway of prevention, diagnosis, and management.
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References
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Dick GFDGH. The etiology of scarlet fever. J Am Med Assoc. 1924;82(4):301–2.
Robertson KA, Volmink JA, Mayosi BM. Antibiotics for the primary prevention of acute rheumatic fever: a meta-analysis. BMC Cardiovasc Disord. 2005;5:11.
Gordis L. Effectiveness of comprehensive-care programs in preventing rheumatic fever. N Engl J Med. 1973;289:331–5.
Manyemba J, Mayosi BM. Penicillin for secondary prevention of rheumatic fever. Cochrane Database Syst Rev. 2002:CD002227.
Strasser T, Dondog N, El Kholy A, et al. The community control of rheumatic fever and rheumatic heart disease: report of a WHO international cooperative project. Bull World Health Organ. 1981;59:285–94.
• 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;76:2982–3021. This study reports the most up-to-date information on the global burden of cardiovascular disease worldwide, depicting current trends for Rheumatic Heart Disease.
Organization WH. Rheumatic fever and rheumatic heart disease. Executive Board, 141st Session: resolutions and decisions, annexes, summary records. Geneva, Switzerland, 2018.
Damasceno A, Mayosi BM, Sani M, et al. The causes, treatment, and outcome of acute heart failure in 1006 Africans from 9 countries: results of the sub-Saharan Africa survey of heart failure. Arch Intern Med. 2012;172:1386–94.
Watkins DA, Johnson CO, Colquhoun SM, et al. Global, regional, and national burden of rheumatic heart disease, 1990–2015. N Engl J Med. 2017;377:713–22.
Islam AK, Majumder AA. Rheumatic fever and rheumatic heart disease in Bangladesh: a review. Indian Heart J. 2016;68:88–98.
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;16.
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;9:e016851.
Wyber R, Noonan K, Halkon C, et al. Ending rheumatic heart disease in Australia: the evidence for a new approach. Med J Aust. 2020;213(Suppl 10):S3–31.
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;64:555–8.
Reeves BM, Kado J, Brook M. High prevalence of rheumatic heart disease in Fiji detected by echocardiography screening. J Paediatr Child Health. 2011;47:473–8.
Essop MR, Nkomo VT. Rheumatic and nonrheumatic valvular heart disease: epidemiology, management, and prevention in Africa. Circulation. 2005;112:3584–91.
Scheel A, Ssinabulya I, Aliku T, et al. Community study to uncover the full spectrum of rheumatic heart disease in Uganda. Heart. 2019;105:60–6.
Damasceno A, Mayosi BM, Sani M, et al. The causes, treatment, and outcome of acute heart failure in 1006 Africans from 9 countries. Arch Intern Med. 2012;172:1386–94.
Noubiap JJ, Agbor VN, Bigna JJ, Kaze AD, Nyaga UF, Mayosi BM. Prevalence and progression of rheumatic heart disease: a global systematic review and meta-analysis of population-based echocardiographic studies. Sci Rep. 2019;9:1–14.
• Karki P, Uranw S, Bastola S, et al. Effectiveness of systematic echocardiographic screening for rheumatic heart disease in Nepalese schoolchildren: a cluster randomized clinical trial. JAMA Cardiology. 2021;6:420–6. This study is the first large-scale trial testing the strategy of systematic echcardiographic screening on outcomes of individuals with Rheumatic Heart Disease, providing additional evidence to screening in clinical practice, noticeably in endemic settings.
•• 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;215:95–105. This is the protocol of the first randomized trial evaluating the effects of penicillin on clinical and echocardiographic outcomes of children with subclinical Rheumatic Heart Disease and high-risk features.
Kingue S, Ba SA, Balde D, et al. The VALVAFRIC study: a registry of rheumatic heart disease in Western and Central Africa. Arch Cardiovasc Dis. 2016;109:321–9.
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;36:1115–22.
Okello E, Wanzhu Z, Musoke C, et al. Cardiovascular complications in newly diagnosed rheumatic heart disease patients at Mulago Hospital, Uganda. Cardiovasc J Afr. 2013;24:82.
Zhang W, Mondo C, Okello E, et al. Presenting features of newly diagnosed rheumatic heart disease patients in Mulago Hospital: a pilot study: cardiovascular topics. Cardiovasc J Afr. 2013;24:28–33.
Watkins DA, Beaton AZ, Carapetis JR, et al. Rheumatic heart disease worldwide: JACC scientific expert panel. J Am Coll Cardiol. 2018;72:1397–416.
Wolf PA, Abbott RD, Kannel WB. Atrial fibrillation as an independent risk factor for stroke: the Framingham Study. Stroke. 1991;22:983–88.
Whitlock RP, Sun JC, Fremes SE, Rubens FD, Teoh KH. Antithrombotic and thrombolytic therapy for valvular disease: antithrombotic therapy and prevention of thrombosis: American College of Chest Physicians Evidence-Based Clinical Practice Guidelines. Chest. 2012;141:e576S-e600S.
January CT, Wann LS, Calkins H, et al. 2019 AHA/ACC/HRS focused update of the 2014 AHA/ACC/HRS guideline for the management of patients with atrial fibrillation: a report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Rhythm Society. J Am Coll Cardiol. 2019;74:104–32.
Semakula JR, Kisa G, Mouton JP, et al. Anticoagulation in sub-Saharan Africa: are direct oral anticoagulants the answer? A review of lessons learnt from warfarin. Br J Clin Pharmacol. 2021.
Ene G, Garcia Raso A, Gonzalez-Dominguez Weber A, Hidalgo-Vega A, Llamas P. Cost of vitamin K antagonist anticoagulant treatment in patients with metallic prosthetic valve in mitral position. SAGE Open Med. 2016;4:2050312116663123.
Guimaraes HP, Lopes RD, de Barros ESPGM, et al. Rivaroxaban in patients with atrial fibrillation and a bioprosthetic mitral valve. N Engl J Med. 2020;383:2117–26.
Karthikeyan G, Connolly SJ, Ntsekhe M, et al. The INVICTUS rheumatic heart disease research program: rationale, design and baseline characteristics of a randomized trial of rivaroxaban compared to vitamin K antagonists in rheumatic valvular disease and atrial fibrillation. Am Heart J. 2020;225:69–77.
Okello E, Longenecker CT, Beaton A, Kamya MR, Lwabi P. Rheumatic heart disease in Uganda: predictors of morbidity and mortality one year after presentation. BMC Cardiovasc Disord. 2017;17:1–10.
Yangni-Angate KH, Meneas C, Diby F, Diomande M, Adoubi A, Tanauh Y. Cardiac surgery in Africa: a thirty-five year experience on open heart surgery in Cote d’Ivoire. Cardiovascular Diagnosis and Therapy. 2016;6:S44.
Meneguz-Moreno RA, Costa JR Jr, Gomes NL, et al. Very long term follow-up after percutaneous balloon mitral valvuloplasty. JACC Cardiovasc Interv. 2018;11:1945–52.
Nunes MC, Nascimento BR, Lodi-Junqueira L, Tan TC, Athayde GR, Hung J. Update on percutaneous mitral commissurotomy. Heart. 2016;102:500–7.
Bouleti C, Iung B, Laouenan C, et al. Late results of percutaneous mitral commissurotomy up to 20 years: development and validation of a risk score predicting late functional results from a series of 912 patients. Circulation. 2012;125:2119–27.
Nunes MC, Tan TC, Elmariah S, et al. The echo score revisited: impact of incorporating commissural morphology and leaflet displacement to the prediction of outcome for patients undergoing percutaneous mitral valvuloplasty. Circulation. 2014;129:886–95.
Watkins D, Zuhlke L, Engel M, et al. Seven key actions to eradicate rheumatic heart disease in Africa: the Addis Ababa communique. Cardiovasc J Afr. 2016;27:184–7.
Zhang W, Okello E, Nyakoojo W, Lwabi P, Mondo CK. Proportion of patients in the Uganda rheumatic heart disease registry with advanced disease requiring urgent surgical interventions. Afr Health Sci. 2015;15:1182–8.
Mirabel M, Grimaldi A, Freers J, Jouven X, Marijon E. Access to cardiac surgery in sub-Saharan Africa. The Lancet. 2015;385:606.
Antunes MJ, Finucane K, Kumar AS, Coutinho GF. Surgical management of rheumatic valvular heart disease. In: Carapetis J, Zühlke L, Wilson N, editors. Acute Rheumatic Fever and Rheumatic Heart Disease. Ireland: Elsevier; 2020. p 147–70.
Edwin F, Aniteye E, Tettey MM, Tamatey M, Frimpong-Boateng K. Outcome of left heart mechanical valve replacement in West African children-A 15-year retrospective study. J Cardiothorac Surg. 2011;6:1–8.
Milne O, Barthwal R, Agahari I, Ilton M, Kangaharan N. Management and outcomes of prosthetic valve thrombosis. An Australian case series from the northern territory. Heart Lung Circ. 2020;29:469–74.
Gündüz S, Kalçık M, Gürsoy MO, Güner A, Özkan M. Diagnosis, treatment & management of prosthetic valve thrombosis: the key considerations. Expert Rev Med Devices. 2020;17:209–21.
Nishimura RA, Otto CM, Bonow RO, et al. 2017 AHA/ACC focused update of the 2014 AHA/ACC guideline for the management of patients with valvular heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. J Am Coll Cardiol. 2017;70:252–89.
Eikelboom JW, Connolly SJ, Brueckmann M, et al. Dabigatran versus warfarin in patients with mechanical heart valves. N Engl J Med. 2013;369:1206–14.
Seckeler MD, Hoke TR. The worldwide epidemiology of acute rheumatic fever and rheumatic heart disease. Clin Epidemiol. 2011;3:67.
Thornburg KL, Jacobson SL, Giraud GD, Morton MJ. Hemodynamic changes in pregnancy. Semin Perinatol. 2000;24:11–4.
van Hagen IM, Thorne SA, Taha N, et al. Pregnancy outcomes in women with rheumatic mitral valve disease: results from the registry of pregnancy and cardiac disease. Circulation. 2018;137:806–16.
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;105:755–60.
Nascimento BR, Sable C, Nunes MCP, et al. Echocardiographic screening of pregnant women by non-physicians with remote interpretation in primary care. Family Practice. 2020.
Arioli F, Ammirati E. Mitral prosthetic valve thrombosis and cardiogenic shock in a limited resource setting in sub-Saharan Africa: a tailored treatment approach. Int J Cardiol. 2015;178:65–6.
Basu S, Aggarwal P, Kakani N, Kumar A. Low-dose maternal warfarin intake resulting in fetal warfarin syndrome: in search for a safe anticoagulant regimen during pregnancy. Birth Defects Res A. 2016;106:142–7.
D’Souza RD, Ostro J, Malinowski A, et al. 662: Anticoagulation for pregnant women with mechanical heart valves: a systematic review and meta-analysis. Am J Obstet Gynecol. 2016;214:S351.
D’Souza R, Ostro J, Shah PS, et al. Anticoagulation for pregnant women with mechanical heart valves: a systematic review and meta-analysis. Eur Heart J. 2017;38:1509–16.
Xu Z, Fan J, Luo X, et al. Anticoagulation regimens during pregnancy in patients with mechanical heart valves: a systematic review and meta-analysis. Can J Cardiol. 2016;32:1248. e1–1248. e9.
French KA, Poppas A. Rheumatic heart disease in pregnancy: global challenges and clear opportunities. Am Heart Assoc. 2018.
Voleti S, Okello E, Murali M, et al. The personal and clinical impact of screen-detected maternal rheumatic heart disease in Uganda: a prospective follow up study. BMC Pregnancy Childbirth. 2020;20:611.
Chang AY, Nabbaale J, Nalubwama H, et al. Motivations of women in Uganda living with rheumatic heart disease: a mixed methods study of experiences in stigma, childbearing, anticoagulation, and contraception. PloS One. 2018;13:e0194030.
PREVENTION of rheumatic fever and bacterial endocarditis through control of streptococcal infections. Pediatrics. 1955;15:642–6.
WHO. Rheumatic fever and rheumatic heart disease. In: Organization WH, editor. Geneva, 2001.
WHF. Diagnosis and mangement of acute rheumatic fever and rheumatic heart disease. In: Federation WH, editor. Geneva, Switzerland, 2008.
Australia R. Australian guideline for prevention, diagnosis and mangement of acute rheumatic fever and rheumatic heart disease. In: Zealand NHFoAaCSoAaN, editor. Darwin, Australia, 2020.
Wyber R, Taubert K, Marko S, Kaplan EL. Benzathine Penicillin G for the management of RHD: concerns about quality and access, and opportunities for intervention and improvement. Glob Heart. 2013;8:227–34.
Taubert K, Marko S. Access to essential medicines: illuminating disparities in the global supply of benzathine penicillin G in the context of rheumatic fever/rheumatic heart disease. J Am Coll Cardiol. 2013;61:e-2004.
de Dassel JL, de Klerk N, Carapetis JR, Ralph AP. How many doses make a difference? An Analysis of secondary prevention of rheumatic fever and rheumatic heart disease. J Am Heart Assoc. 2018;7:e010223.
Kevat PM, Reeves BM, Ruben AR, Gunnarsson R. Adherence to secondary prophylaxis for acute rheumatic fever and rheumatic heart disease: a systematic review. Curr Cardiol Rev. 2017;13:155–66.
Read C, Mitchell AG, de Dassel JL, et al. Qualitative evaluation of a complex intervention to improve rheumatic heart disease secondary prophylaxis. J Am Heart Assoc. 2018;7.
Ralph AP, de Dassel JL, Kirby A, et al. Improving delivery of secondary prophylaxis for rheumatic heart disease in a high-burden setting: outcome of a stepped-wedge, community, randomized trial. J Am Heart Assoc. 2018;7.
Balbaa A, ElGuindy A, Pericak D, Yacoub MH, Schwalm JD. An evaluation of secondary prophylaxis for rheumatic heart disease in rural Egypt. Glob Cardiol Sci Pract. 2015;2015:40.
Thompson SB, Brown CH, Edwards AM, Lindo JL. Low adherence to secondary prophylaxis among clients diagnosed with rheumatic fever. Jamaica Pathog Glob Health. 2014;108:229–34.
Engelman D, Ah Kee M, Mataika RL, et al. Secondary prevention for screening detected rheumatic heart disease: opportunities to improve adherence. Trans R Soc Trop Med Hyg. 2017;111:154–62.
Mincham CM, Toussaint S, Mak DB, Plant AJ. Patient views on the management of rheumatic fever and rheumatic heart disease in the Kimberley: a qualitative study. Aust J Rural Health. 2003;11:260–5.
Huck DM, Nalubwama H, Longenecker CT, Frank SH, Okello E, Webel AR. A qualitative examination of secondary prophylaxis in rheumatic heart disease: factors influencing adherence to secondary prophylaxis in Uganda. Glob Heart. 2015;10:63–69 e1.
Barker H, Oetzel JG, Scott N, Morley M, Carr PEA, Oetzel KB. Enablers and barriers to secondary prophylaxis for rheumatic fever among Maori aged 14–21 in New Zealand: a framework method study. Int J Equity Health. 2017;16:201.
Kumar R, Raizada A, Aggarwal AK, Ganguly NK. A community-based rheumatic fever/rheumatic heart disease cohort: twelve-year experience. Indian Heart J. 2002;54:54–8.
Harrington Z, Thomas DP, Currie BJ, Bulkanhawuy J. Challenging perceptions of non-compliance with rheumatic fever prophylaxis in a remote Aboriginal community. Med J Aust. 2006;184:514–7.
World Health Organization. Report of the Commission on Macroeconomics and health. 2002.
Watkins D, Zuhlke L, Engel M, et al. Seven key actions to eradicate rheumatic heart disease in Africa: the Addis Ababa communiqué: cardiovascular topics. Cardiovasc J Afr. 2016;27:184–7.
Oyebamiji O. The household economic impact of rheumatic heart disease (RHD) in South Africa. University of Cape Town, 2018.
Hellebo AG, Zuhlke LJ, Watkins DA, Alaba O. Health system costs of rheumatic heart disease care in South Africa. BMC Public Health. 2021;21:1–11.
Mocumbi AO. Out-of-pocket costs in rheumatic heart disease care: a major barrier to equity in cardiovascular health. Indian Heart J. 2021;73:141.
Arvind B, Saxena A, Kazi DS, Bolger AF. Out-of-pocket expenditure for administration of benzathine penicillin G injections for secondary prophylaxis in patients with rheumatic heart disease: a registry-based data from a tertiary care center in Northern India. Indian Heart J. 2021;73:169–73.
Haynes E, Mitchell A, Enkel S, Wyber R, Bessarab D. Voices behind the statistics: a systematic literature review of the lived experience of rheumatic heart disease. Int J Environ Res Public Health. 2020;17:1347.
Monib D, Wahdan I, Hasab A, Zaher S. Health related quality of life of rheumatic heart disease patients in Alexandria: an intervention approach. Journal of High Institute of Public Health. 2013;43:112–20.
Riaz A, Hanif MI, Khan IH, Hanif A, Mughal S, Anwer A. Quality of life in patients with rheumatic heart disease. J Pakistan Med Assoc. 2018;68:370–5.
Bradley-Hewitt T, Dantin A, Ploutz M, et al. The impact of echocardiographic screening for rheumatic heart disease on patient quality of life. J Pediatr. 2016;175:123–9.
Zühlke L, Perkins S, Cembi S. Rheumatic heart disease patient event: Cape Town hosts 4th annual listen to my heart rheumatic heart disease for patients at the South African heart association meeting in 2017. Oxford University Press, 2018.
Macleod CK, Bright P, Steer AC, Kim J, Mabey D, Parks T. Neglecting the neglected: the objective evidence of underfunding in rheumatic heart disease. Trans R Soc Trop Med Hyg. 2019;113:287–90.
World Health Organization. 71st World Health Assembly adopts resolution calling for greater action on rheumatic heart disease. Seventy-First World Health Assembly World Health Organization, Geneva, Switzerland 2018. https://www.who.int/about/governance/world-health-assembly/seventy-first.
World Health Organization. Rheumatic fever and rheumatic heart disease. Report by the Director General. Seventy-First World Health Assembly World Health Organization, Geneva, Switzerland 2018. https://apps.who.int/iris/handle/10665/276479.
Beaton A, Kamalembo FB, Dale J, et al. The American Heart Association’s call to action for reducing the global burden of rheumatic heart disease: a policy statement from the American Heart Association. Circulation. 2020;142:e358–68.
Mayosi BM, Gamra H, Dangou JM, Kasonde J. 2nd All-Africa Workshop on Rheumatic Fever and Rheumatic Heart Disease participants. Rheumatic heart disease in Africa: the Mosi-o-Tunya call to action. Lancet Glob Health. 2014;2:e438–9.
Dale JB, Walker MJ. Update on group A streptococcal vaccine development. Curr Opin Infect Dis. 2020;33:244–250.
Funding
This study has no specific funding. Dr. Nascimento was supported in part by CNPq (Bolsa de produtividade em pesquisa, 312382/2019–7), by the Edwards Lifesciences Foundation (Improving the Prevention and Detection of Heart Valve Disease Across the Lifespan, 2021), and by FAPEMIG (grant APQ-000627–20).
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Rwebembera, J., Beaton, A.Z., de Loizaga, S.R. et al. The Global Impact of Rheumatic Heart Disease. Curr Cardiol Rep 23, 160 (2021). https://doi.org/10.1007/s11886-021-01592-2
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DOI: https://doi.org/10.1007/s11886-021-01592-2