Acute rheumatic fever (ARF) and its sequelae, Rheumatic heart disease (RHD), contribute significantly to the cardiovascular morbidity and mortality in developing countries. Generally considered a disease of poverty and poor socio-economic conditions, RHD affects the population at the most productive phase of their life. The diagnostic criteria for ARF have been constantly updated to improve the sensitivity. The diagnosis of ARF was entirely clinical however, recently echocardiographic evidence has been added as a major criterion. The disease seems to be on the decline in India, but recent studies using echocardiography have shown high prevalence of RHD among school children. The focus of management has been on prompt recognition and treatment of streptococcal pharyngitis and preventing recurrences of ARF with long-term antibiotic prophylaxis. However, emphasis should be placed on the appropriate management of patients with established RHD, in order to limit the RHD related mortality.
This is a preview of subscription content, access via your institution.
Buy single article
Instant access to the full article PDF.
Price excludes VAT (USA)
Tax calculation will be finalised during checkout.
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.
Ramakrishnan S, Kothari SS, Juneja R, Bhargava B, Saxena A, Bahl VK. Prevalence of rheumatic heart disease: has it declined in India? Natl Med J India. 2009;22:72–4.
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;249:438–42.
Jose VJ, Gomathi M. Declining prevalence of rheumatic heart disease in rural school children in India: 2001-2002. Indian Heart J. 2003;55:158–60.
Jai Vigyan Mission Mode Project. Community Control of Rheumatic Fever/ Rheumatic Heart Disease in India. Comprehensive Project Report. 2000–2010. Available at: https://www.icmr.nic.in/sites/default/files/reports/Jai Vigyan Mission Mode project.
Misra M, Mittal M, Singh R, et al. Prevalence of rheumatic heart disease in school-going children of eastern Uttar Pradesh. Indian Heart J. 2007;59:42–3.
Periwal K, Gupta B, Panwar R, Khatri P, Raja S, Gupta R. Prevalence of rheumatic heart disease in school children in Bikaner: an echocardiographic study. J Assoc Physicians India. 2006;54:279–82.
Negi PC, Kanwar A, Chauhan R, Asotra S, Thakur JS, Bhardwaj AK. Epidemiological trends of RF/RHD in school children of Shimla in North India. Indian J Med Res. 2013;137:1121–7.
Rama Kumari N, Bhaskara Raju I, Patnaik AN, et al. Prevalence of rheumatic and congenital heart disease in school children of Andhra Pradesh, South India. J Cardiovasc Dis Res 2013;4:11–4.
Saxena A, Ramakrishnan S, Roy 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. 2011;97:2018–22.
Bhaya M, Panwar S, Beniwal R, Panwar RB. High prevalence of rheumatic heart disease detected by echocardiography in school children. Echocardiography. 2010;27:448–53.
Nair B, Viswanathan S, Koshy AG, Gupta PN, Nair N, Thakkar A. Rheumatic heart disease in Kerala: a vanishing entity? An echo doppler study in 5–15-years-old school children. Int J Rheumatol. 2015. https://doi.org/10.1155/2015/930790.
Azevedo PM, Pereira RR, Guilherme L. Understanding rheumatic fever. Rheumatol Int. 2012;32:1113–20.
Guilherme L, Kalil J. Rheumatic fever and rheumatic heart disease: cellular mechanisms leading autoimmune reactivity and disease. J Clin Immunol. 2010;30:17–23.
Bryant PA, Robins-Browne R, Carapetis JR, Curtis N. Some of the people, some of the time: susceptibility to acute rheumatic fever. Circulation. 2009;119:742–53.
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;131:1806–18.
Veasy LG, Tani LY, Hill HR. Persistence of acute rheumatic fever in the intermountain area of the United States. J Pediatr. 1994;124:9–16.
Arora R, Subramanyam G, Khalilullah M, Gupta MP. Clinical profile of rheumatic fever and rheumatic heart disease: a study of 2,500 cases. Indian Heart J. 1981;33:264–9.
Tubridy-Clark M, Carapetis JR. Subclinical carditis in rheumatic fever: a systematic review. Int J Cardiol. 2007;119:54–8.
Sethi S, Kaushik K, Mohandas K, Sengupta C, Singh S, Sharma M. Anti-streptolysin O titers in normal healthy children of 5-15 years. Indian Pediatr. 2003;40:1068–71.
Saini N, Kumar D, Swarnim S, Bhatt D, Kishore S. Comparison of antistreptolysin O and anti-deoxyribonucleic B titers in healthy children to those with acute pharyngitis, acute rheumatic fever, and rheumatic heart disease aged 5-15 years. Ann Pediatr Cardiol. 2019;12:195–200.
Pichichero ME, Marsocci SM, Murphy ML, Hoeger W, Green JL, Sorrento A. Incidence of streptococcal carriers in private pediatric practice. Arch Pediatr Adolesc Med. 1999;153:624–8.
Ramakrishnan S. Echocardiography in acute rheumatic fever. Ann Pediatr Card. 2009;2:61–4.
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;9:297–309.
Karthikeyan G, Guilherme L. Acute rheumatic fever. Lancet. 2018;392:161–74.
Van Asselt GJ, Mouton RP. Detection of penicillin tolerance in Streptococcus pyogenes. J Med Microbiol. 1993;38:197–202.
Hashkes PJ, Tauber T, Somekh E, et al. Naproxen as an alternative to aspirin for the treatment of arthritis of rheumatic fever: a randomized trial. J Pediatr. 2003;143:399–401.
Treatement of acute rheumatic fever in children a co-operative clinical trial of A.C.T.H., cortisone, and aspirin; a joint report by the Rheumatic Fever Working Party of the Medical Research Council of Great Britain and the Subcommittee of Principal Investigators of the American Council on Rheumatic Fever and Congenital Heart Disease, American Heart Association. Br Med J. 1955; 1:555–74.
Mehta A, Saxena A, Juneja R, Ramakrishnan S, Gupta S, Kothari SS. Characteristics and outcomes of Indian children enrolled in a rheumatic heart disease registry. Int J Cardiol. 2016;222:1136–40.
Enriquez-Sarano M, Basmadjian AJ, Rossi A, Bailey KR, Seward JB, Tajik AJ. Progression of mitral regurgitation: a prospective Doppler echocardiographic study. J Am Coll Cardiol. 1999;34:1137–44.
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;36:1115–22a.
Roy SB, Bhatia ML, Lazaro EJ, Ramalingaswami V. Juvenile mitral stenosis in India. Lancet. 1963;282:1193–6.
Goswami KC, Rao MB, Dev V, Shrivastava S. Juvenile tricuspid stenosis and rheumatic tricuspid valve disease: an echocardiographic study. Int J Cardiol. 1999;72:83–6.
International Rheumatic Fever Study Group. Allergic reactions to long-term benzathine penicillin prophylaxis for rheumatic fever. Lancet. 1991;337:1308–10.
Nishimura RA, Otto CM, Bonow RO, et al. AHA/ACC guideline for the management of patients with valvular heart disease: executive summary: a report of the American College of Cardiology/American Heart Association task force on practice guidelines. Circulation. 2014;129:2440–92.
Kumar AS, Talwar S, Saxena A, Singh R, Velayoudam D. Results of mitral valve repair in rheumatic mitral regurgitation. Interact Cardiovasc Thorac Surg. 2006;5:356–61.
Skoularigis J, Sinovich V, Joubert G, Sareli P. Evaluation of the long-term results of mitral valve repair in 254 young patients with rheumatic mitral regurgitation. Circulation. 1994;90:(II)167–74.
Gometza B. al-Halees Z, Shahid M, Hatle LK, Duran CM. Surgery for rheumatic mitral regurgitation in patients below twenty years of age. An analysis of failures. J Heart Valve Dis. 1996;5:294–301.
Kothari SS, Ramakrishnan S, Kumar CK, Juneja R, Yadav R. Intermediate-term results of percutaneous transvenous mitral commissurotomy in children less than 12 years of age. Catheter Cardiovasc Interv. 2005;64:487–90.
Kothari SS, Ramakrishnan S, Juneja R, Yadav R. Percutaneous transvenous mitral commissurotomy in patients with severe mitral stenosis and acute rheumatic fever. Pediatr Cardiol. 2006;27:347–50.
Conflict of Interest
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
About this article
Cite this article
Arvind, B., Ramakrishnan, S. Rheumatic Fever and Rheumatic Heart Disease in Children. Indian J Pediatr 87, 305–311 (2020). https://doi.org/10.1007/s12098-019-03128-7