- Email: [email protected]
The challenge of silent rheumatic heart disease
Comment
The challenge of silent rheumatic heart disease Over the past 10 years, the number of studies on the burden of rheumatic heart disease in developing countries has substantially increased.1 These studies range from echocardiographic screening surveys of the prevalence of rheumatic heart disease in school children,2 to studies of incidence and progression,3,4 to investigations of the pathogenesis of the disease.5 The systematic review and metaanalysis of prevalence studies of rheumatic heart disease by Martina Rothenbühler and colleagues6 in The Lancet Global Health is a timely summary of a decade of renewed focus by the international community on a neglected disease of poverty. Rothenbühler and colleagues6 sought to assess the effect of different screening methods (ie, auscultation vs echocardiography) on estimated prevalence of rheumatic heart disease among children and adolescents in endemic regions of the world. Of the 37 populations included, 17 were from Asia, nine Africa, seven Oceania, three Latin America, and one Europe. The pooled prevalence of rheumatic heart disease detected by cardiac auscultation was 2·9 per 1000 people (95% CI 1·7–5·0) and by echocardiography it was 12·9 per 1000 people (8·9–18·6), with substantial heterogeneity between individual reports for both screening tests (I²=99·0% and I²=94·9%, respectively). The previously reported7 association between social inequality expressed by the Gini coefficient and prevalence of rheumatic heart disease was also confirmed by Rothenbühler and colleagues (p=0·0002).6 The prevalence of clinically silent rheumatic heart disease (21·1 per 1000 people, 95% CI 14·1–31·4) was about seven to eight times higher than that of clinically manifest disease (2·7 per 1000 people, 1·6–4·4). As has been reported by others,8,9 prevalence progressively increased with advancing age, from 4·7 per 1000 people (95% CI 0·0–11·2) in 5-year-old children, to 21·0 per 1000 people (6·8–35·1) in 16-yearold adolescents, and the estimated overall incidence was 1·6 per 1000 people (95% CI 0·8–2·3) and was constant across age categories (range 2·5, 95% CI 1·3–3·7 in 5-yearold children to 1·7, 0·0–5·1 in 15-year-old adolescents). Unlike others who have noted a female preponderance in rheumatic heart disease prevalence,3 no sex-related differences in prevalence of rheumatic heart disease were found (p=0·829). www.thelancet.com/lancetgh Vol 2 December 2014
Findings from the last major systematic review of the global prevalence of echocardiographic rheumatic heart disease showed that the highest calculated regional prevalence was in sub-Saharan Africa (5·7 cases per 1000 people), indigenous people in the Pacific islands, Australia, and New Zealand (3·5 cases per 1000 people), and south central Asia (2·2 cases per 1000 people).10 By contrast, the additional studies included in the present review show that southeast Asia is the new hot spot for echocardiographic rheumatic heart disease (28·0 cases per 1000 people, 95% CI 16·6–49·9), followed by Oceania (14·0 per 1000 people, 7·7–25·5), and Africa (7·9 per 1000 people, 2·9–21·4). The dramatic revival of rheumatic heart disease in the former Soviet republics of central Asia since the fall of the Berlin Wall in 198911 is consistent with the location of the epicentre of rheumatic heart disease in Asia reported in the present systematic review.6 The changing pattern of rheumatic heart disease in different regions of the world emphasises the importance of improving social conditions and access to primary health care for the control of rheumatic heart disease. The pooled prevalence of clinical rheumatic heart disease (ie, presence of pathological murmur) seemed to be comparable whether it was measured by auscultation only (2·9 per 1000 people, 95% CI 1·7–5·0) or by echocardiography and auscultation (2·7 per 1000 people, 1·6–4·4), which is remarkable. Consensus is emerging that this group of people with asymptomatic but clinically evident rheumatic heart disease should be considered for secondary antibiotic prophylaxis with penicillin to prevent progression to symptomatic disease, although there is no randomised evidence to support this recommendation.12 However, the challenge is how to manage treatment in children and adolescents with clinically silent rheumatic heart disease, whose prevalence was substantially higher than the cases of clinically manifest rheumatic heart disease in this systematic review. There is limited information on the natural history and virtually no evidence upon which to base the management of children and adolescents with asymptomatic subclinical rheumatic heart disease that is detected on active surveillance.12 The time has come to launch large-scale prospective studies of the natural history of latent rheumatic heart disease and trials to
See Articles page e717
e677
Comment
assess the efficacy of penicillin prophylaxis to prevent progression of disease. The most important message of this systematic review and meta-analysis6 is that rheumatic heart disease remains a major public health problem in developing countries. The disease causes the highest number of disability-adjusted life-years of all listed cardiovascular diseases among 10–14-year-olds (516·6 per 100 000 people, 95% CI 425·3–647·0) and the second highest number among children aged 5–9 years (362·0 per 100 000 people, 294·6–462·0).13 WHO has set a target of reducing premature mortality from rheumatic heart disease and other noncommunicable disease by 25% by the year 2025.14 This target can be achieved and exceeded through the establishment of national rheumatic heart disease programmes that implement a comprehensive strategy for primary and secondary prevention of the disease, such as the Stop Rheumatic Heart Disease A.S.A.P. Programme, which has been launched in Africa.1
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Bongani M Mayosi Department of Medicine, Groote Schuur Hospital and University of Cape Town, Cape Town 7925, South Africa [email protected] Copyright © Mayosi. Open Access article distributed under the terms of CC BY.
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Mayosi BM, Gamra H, Dangou J-M, Kasonde J. Rheumatic heart disease in Africa: the Mosi-o-Tunya call to action. Lancet Glob Health 2014; 2: e438–39. Marijon E, Ou P, Celermajer DS, et al. Prevalence of rheumatic heart disease detected by echocardiographic screening. N Engl J Med 2007; 357: 470–76. Sliwa K, Carrington M, Mayosi BM, Zigiriadis E, Mvungi R, Stewart S. Incidence and characteristics of newly diagnosed rheumatic heart disease in Urban African adults: insights from the Heart of Soweto Study. Eur Heart J 2010; 31: 719–27. Lawrence JG, Carapetis JR, Griffiths K, Edwards K, Condon JR. Acute rheumatic fever and rheumatic heart disease: incidence and progression in the Northern Territory of Australia, 1997 to 2010. Circulation 2013; 128: 492–501. Engel ME, Stander R, Vogel J, Adeyemo AA, Mayosi BM. Genetic susceptibility to acute rheumatic fever: a systematic review and meta-analysis of twin studies. PLoS One 2011; 6: e25326. Rothenbühler M, O’Sullivan CJ, Stortecky S, et al. Active surveillance for rheumatic heart disease in endemic regions: a systematic review and metaanalysis of prevalence among children and adolescents. Lancet Glob Health; 2014; 2: e717–726. Brown A, McDonald MI, Calma T. Rheumatic fever and social justice. Med J Aust 2007; 186: 557–58. McLaren MJ, Hawkins DM, Koornhof HJ, et al. Epidemiology of rheumatic heart disease in black school children of Soweto, Johannesburg. Br Med J 1975; 3: 474–78. Kane A, Mirabel M, Touré K, et al. Echocardiographic screening for rheumatic heart disease: age matters. Int J Cardiol 2013; 168: 888–91. Carapetis JR, Steer AC, Mulholland EK, Weber M. The global burden of group A streptococcal diseases. Lancet Infect Dis 2005; 5: 685–94. Omurzakova NA, Yamano Y, Saatova GM, et al. High incidence of rheumatic fever and rheumatic heart disease in the republics of Central Asia. Int J Rheum Dis 2009; 12: 79–83. Zuhlke L, Mayosi BM. Echocardiographic screening for subclinical rheumatic heart disease remains a research tool pending studies of impact on prognosis. Curr Cardiol Rep 2013; 15: 343. Murray CJL, Vos T, Lozano R, et al. Disability-adjusted life years (DALYs) for 291 diseases and injuries in 21 regions, 1990–2010: a systematic analysis for the Global Burden of Disease Study 2010. Lancet 2012; 380: 2197–223. WHO. Global action plan for the prevention and control of NCDs 2013–2020. Geneva: World Health Organization, 2013.
www.thelancet.com/lancetgh Vol 2 December 2014
The challenge of silent rheumatic heart disease Over the past 10 years, the number of studies on the burden of rheumatic heart disease in developing countries has substantially increased.1 These studies range from echocardiographic screening surveys of the prevalence of rheumatic heart disease in school children,2 to studies of incidence and progression,3,4 to investigations of the pathogenesis of the disease.5 The systematic review and metaanalysis of prevalence studies of rheumatic heart disease by Martina Rothenbühler and colleagues6 in The Lancet Global Health is a timely summary of a decade of renewed focus by the international community on a neglected disease of poverty. Rothenbühler and colleagues6 sought to assess the effect of different screening methods (ie, auscultation vs echocardiography) on estimated prevalence of rheumatic heart disease among children and adolescents in endemic regions of the world. Of the 37 populations included, 17 were from Asia, nine Africa, seven Oceania, three Latin America, and one Europe. The pooled prevalence of rheumatic heart disease detected by cardiac auscultation was 2·9 per 1000 people (95% CI 1·7–5·0) and by echocardiography it was 12·9 per 1000 people (8·9–18·6), with substantial heterogeneity between individual reports for both screening tests (I²=99·0% and I²=94·9%, respectively). The previously reported7 association between social inequality expressed by the Gini coefficient and prevalence of rheumatic heart disease was also confirmed by Rothenbühler and colleagues (p=0·0002).6 The prevalence of clinically silent rheumatic heart disease (21·1 per 1000 people, 95% CI 14·1–31·4) was about seven to eight times higher than that of clinically manifest disease (2·7 per 1000 people, 1·6–4·4). As has been reported by others,8,9 prevalence progressively increased with advancing age, from 4·7 per 1000 people (95% CI 0·0–11·2) in 5-year-old children, to 21·0 per 1000 people (6·8–35·1) in 16-yearold adolescents, and the estimated overall incidence was 1·6 per 1000 people (95% CI 0·8–2·3) and was constant across age categories (range 2·5, 95% CI 1·3–3·7 in 5-yearold children to 1·7, 0·0–5·1 in 15-year-old adolescents). Unlike others who have noted a female preponderance in rheumatic heart disease prevalence,3 no sex-related differences in prevalence of rheumatic heart disease were found (p=0·829). www.thelancet.com/lancetgh Vol 2 December 2014
Findings from the last major systematic review of the global prevalence of echocardiographic rheumatic heart disease showed that the highest calculated regional prevalence was in sub-Saharan Africa (5·7 cases per 1000 people), indigenous people in the Pacific islands, Australia, and New Zealand (3·5 cases per 1000 people), and south central Asia (2·2 cases per 1000 people).10 By contrast, the additional studies included in the present review show that southeast Asia is the new hot spot for echocardiographic rheumatic heart disease (28·0 cases per 1000 people, 95% CI 16·6–49·9), followed by Oceania (14·0 per 1000 people, 7·7–25·5), and Africa (7·9 per 1000 people, 2·9–21·4). The dramatic revival of rheumatic heart disease in the former Soviet republics of central Asia since the fall of the Berlin Wall in 198911 is consistent with the location of the epicentre of rheumatic heart disease in Asia reported in the present systematic review.6 The changing pattern of rheumatic heart disease in different regions of the world emphasises the importance of improving social conditions and access to primary health care for the control of rheumatic heart disease. The pooled prevalence of clinical rheumatic heart disease (ie, presence of pathological murmur) seemed to be comparable whether it was measured by auscultation only (2·9 per 1000 people, 95% CI 1·7–5·0) or by echocardiography and auscultation (2·7 per 1000 people, 1·6–4·4), which is remarkable. Consensus is emerging that this group of people with asymptomatic but clinically evident rheumatic heart disease should be considered for secondary antibiotic prophylaxis with penicillin to prevent progression to symptomatic disease, although there is no randomised evidence to support this recommendation.12 However, the challenge is how to manage treatment in children and adolescents with clinically silent rheumatic heart disease, whose prevalence was substantially higher than the cases of clinically manifest rheumatic heart disease in this systematic review. There is limited information on the natural history and virtually no evidence upon which to base the management of children and adolescents with asymptomatic subclinical rheumatic heart disease that is detected on active surveillance.12 The time has come to launch large-scale prospective studies of the natural history of latent rheumatic heart disease and trials to
See Articles page e717
e677
Comment
assess the efficacy of penicillin prophylaxis to prevent progression of disease. The most important message of this systematic review and meta-analysis6 is that rheumatic heart disease remains a major public health problem in developing countries. The disease causes the highest number of disability-adjusted life-years of all listed cardiovascular diseases among 10–14-year-olds (516·6 per 100 000 people, 95% CI 425·3–647·0) and the second highest number among children aged 5–9 years (362·0 per 100 000 people, 294·6–462·0).13 WHO has set a target of reducing premature mortality from rheumatic heart disease and other noncommunicable disease by 25% by the year 2025.14 This target can be achieved and exceeded through the establishment of national rheumatic heart disease programmes that implement a comprehensive strategy for primary and secondary prevention of the disease, such as the Stop Rheumatic Heart Disease A.S.A.P. Programme, which has been launched in Africa.1
1
2 3
4
5
6
7 8
9 10 11
12
Bongani M Mayosi Department of Medicine, Groote Schuur Hospital and University of Cape Town, Cape Town 7925, South Africa [email protected] Copyright © Mayosi. Open Access article distributed under the terms of CC BY.
e678
13
14
Mayosi BM, Gamra H, Dangou J-M, Kasonde J. Rheumatic heart disease in Africa: the Mosi-o-Tunya call to action. Lancet Glob Health 2014; 2: e438–39. Marijon E, Ou P, Celermajer DS, et al. Prevalence of rheumatic heart disease detected by echocardiographic screening. N Engl J Med 2007; 357: 470–76. Sliwa K, Carrington M, Mayosi BM, Zigiriadis E, Mvungi R, Stewart S. Incidence and characteristics of newly diagnosed rheumatic heart disease in Urban African adults: insights from the Heart of Soweto Study. Eur Heart J 2010; 31: 719–27. Lawrence JG, Carapetis JR, Griffiths K, Edwards K, Condon JR. Acute rheumatic fever and rheumatic heart disease: incidence and progression in the Northern Territory of Australia, 1997 to 2010. Circulation 2013; 128: 492–501. Engel ME, Stander R, Vogel J, Adeyemo AA, Mayosi BM. Genetic susceptibility to acute rheumatic fever: a systematic review and meta-analysis of twin studies. PLoS One 2011; 6: e25326. Rothenbühler M, O’Sullivan CJ, Stortecky S, et al. Active surveillance for rheumatic heart disease in endemic regions: a systematic review and metaanalysis of prevalence among children and adolescents. Lancet Glob Health; 2014; 2: e717–726. Brown A, McDonald MI, Calma T. Rheumatic fever and social justice. Med J Aust 2007; 186: 557–58. McLaren MJ, Hawkins DM, Koornhof HJ, et al. Epidemiology of rheumatic heart disease in black school children of Soweto, Johannesburg. Br Med J 1975; 3: 474–78. Kane A, Mirabel M, Touré K, et al. Echocardiographic screening for rheumatic heart disease: age matters. Int J Cardiol 2013; 168: 888–91. Carapetis JR, Steer AC, Mulholland EK, Weber M. The global burden of group A streptococcal diseases. Lancet Infect Dis 2005; 5: 685–94. Omurzakova NA, Yamano Y, Saatova GM, et al. High incidence of rheumatic fever and rheumatic heart disease in the republics of Central Asia. Int J Rheum Dis 2009; 12: 79–83. Zuhlke L, Mayosi BM. Echocardiographic screening for subclinical rheumatic heart disease remains a research tool pending studies of impact on prognosis. Curr Cardiol Rep 2013; 15: 343. Murray CJL, Vos T, Lozano R, et al. Disability-adjusted life years (DALYs) for 291 diseases and injuries in 21 regions, 1990–2010: a systematic analysis for the Global Burden of Disease Study 2010. Lancet 2012; 380: 2197–223. WHO. Global action plan for the prevention and control of NCDs 2013–2020. Geneva: World Health Organization, 2013.
www.thelancet.com/lancetgh Vol 2 December 2014