Rheumatic Heart Disease Control Programs, Registers, and Access to Care





Rheumatic Heart Disease Control Programs


Disease control is the “reduction in the incidence, prevalence, morbidity, or mortality of an infectious disease to a locally acceptable level.” Efforts to control acute rheumatic fever (ARF) and rheumatic heart disease (RHD) have been underway for nearly a century and are entering a new era with passing of the resolution on rheumatic fever and RHD at the 71st World Health Assembly. Indeed, a growing focus on the potential of RHD control programs has led to calls for elimination of the disease entirely. This chapter traces the development of RHD control initiatives and identifies common themes to inform development of new programs in the postresolution era. It is intended as a primer on key lessons in RHD control worldwide.


The terms control program and control initiatives are used interchangeably in this chapter and are broadly defined, spanning all activities intended to reduce the burden of group A Streptococcus (GAS), ARF, and RHD. Burden is considered to be both epidemiologic impact (on incidence and prevalence of disease) and human impact (on the lived experience of RHD and experience of people, families, and communities). Few of the RHD control programs described are discrete, planned, and continuously funded initiatives; almost all span years of mixed funding sources, research activities, and clinical care delivery. This approach can lead to fragmentation and is heavily reliant on individual “champions” to persevere with RHD activities. However, case studies—particularly from Sudan, Uganda, and Fiji—highlight how combinations of different activities can contribute to increasingly comprehensive and well-developed programs over a number of years. This overview is not exhaustive and is limited by underrepresentation of programs from low-resource settings in the published literature. Ensuring that results and outcomes from RHD control programs worldwide are evaluated, published, and acted upon is a shared global priority for improving service delivery and achieving equitable gains in reducing RHD.


1900 – 1950s


RHD was a leading cause of childhood morbidity and hospital admission in the United Kingdom and United States in the early part of the 20th century. This considerable disease burden prompted interest in improving clinical outcomes and exploring opportunities for prevention. In the absence of disease-altering therapies, early RHD control programs focused on research to understand etiology, case notification, and systems to improve medical management. Social determinants of health—including household crowding, lack of hygiene facilities and poverty—had been identified as risk factors for ARF. Strategies to address social determinants of health were a feature of this early period, and include efforts to improve housing for people at risk of ARF recurrences. For example, a 1930s program in Dublin, Ireland was established to identify children with symptoms of ARF in schools and refer them to hospital clinics for assessment. ARF education was provided for parents, and public health authorities were engaged to improve living conditions, particularly overcrowding, where necessary.


1950s–2000


The discovery of sulfonamides and penicillin in the 1940s and 1950s offered the first therapeutic opportunity to prevent ARF and progression of RHD. As evidence for primary and secondary prevention emerged, systems to deliver these disease-altering interventions were needed and RHD control programs assumed a new remit.


Registers to facilitate prophylaxis delivery began in the 1940s and 1950s in North America. In Toronto, Canada, a register was established in 1948 to facilitate care for children with congenital heart disease and RHD, including provision of prophylaxis. In the United States, the Maryland Register was developed in the mid-1950s to supply low cost oral penicillin and record medication adherence to prophylaxis. As the Maryland Register developed and became automated with punch cards, the role of the register expanded to include clinical review and advice to referring clinicians. Indeed, a system of community-based efforts to reduce ARF risk, supported by cardiology management of symptoms and prevention of recurrences, was a hallmark of this period.


Expansion of RHD registers and increasing access to disease-altering antibiotics spurred research interest, leading to a “cooperative investigative project” coordinated by the World Health Organization from 1972 to 1980. One of the goals of this formative program was “demonstration of the feasibility of community control of rheumatic heart disease in pilot programmes.” Nearly 3000 people were enrolled across seven sites with reasonable evidence of enhanced delivery of secondary prophylaxis over this time (see Table 12.1 ). One of the participating project sites in Delhi published an independent overview suggestive of increased engagement with RHD control and general support for register-based secondary prophylaxis.



Table 12.1

Selected 20th Century Control Programs for Rheumatic Heart Disease.












































































































Program Reporting Dates a Register for Secondary Prevention Health Worker Education Community Education or Engagement Primary Prevention Clinical Guidelines Concurrent Research Activities Notifications/Disease Surveillances Community Screening Notes Reported Outcomes
WHO study “community control of RF/RHD”—community level projects in the following countries:
Egypt, Cyprus, Jamaica, Lagos, India, Iran, Mongolia.
1972–1980 Yes Yes Yes Not addressed Not addressed Yes Yes Yes—auscultation Observational study.
No control or comparison data.
In aggregated analysis across seven study sites with 50% response rate, delivery of secondary prophylaxis injections improved from 38.3% of people receiving 10-12 scheduled injections in year 1 to 76% receiving 10-12 scheduled injections in year 6. Increasing number of injections each year associated with reduced hospital admissions. Many practical difficulties noted in delivering the program.
One positive report from project site.
WHO Global Programme in the following countries:
Mali, Zambia, Zimbabwe, Bolivia, El Salvador, Jamaica, Egypt, Iraq, Pakistan, Sudan, India, Sri Lanka, Thailand, China, Philippines, Tonga.
1984–2001 Yes Yes Yes Yes Not addressed Yes Not addressed Yes—auscultation Over 200,000 children screened through auscultation.
Observational study.
No control or comparison data.
Reports of improved adherence with secondary prophylaxis but baseline data or temporal changes not clearly reported.
Martinique and Guadeloupe 1981–1992 Yes Yes Yes Yes [including treatment of skin infections] Not addressed Yes Not addressed Yes—pharyngeal swabbing Term used to describe the disease was changed to help public education campaign.
Full time pediatrician dedicated to RHD in each island.
Observational study.
No control or comparison data.
Reduced total ARF incidence (78% reduction in Martinique, 74% reduction in Guadeloupe).
Reports of reduction in need for open heart surgery
Costa Rica 1985–1990 Yes Yes Not addressed Yes [changed clinical criteria for treatment of GAS pharyngitis] Yes [changed clinical criteria for treatment of GAS pharyngitis] Not addressed Not addressed Not addressed Observational study.
No control or comparison data.
Reduced total ARF incidence (7.8/100,000 in 1985 to 1/100,000 in 1990)
Jamaica 1985–1995 Yes Yes Yes Yes Yes Yes Yes Not addressed Description of different clinical presentations at beginning and end of reporting period presented but no clear epidemiologic data.
Pinar del Rio, Cuba 1986–1996 Yes Yes Yes Yes Not addressed Yes Yes No Observational study.
No control or comparison data.
Reduced total ARF incidence (28.4/100,000 to 2.7/100,000)
Reduced proportion of recurrent ARF (5/100,000 to 0.9/100,000)
Improved adherence to SP from ( n = 52) 50% “regular” adherence in 1986 to ( n = 193) 93.8% regular SP in 1996
Minas Gerais, Brazil 1988 2000 Yes Yes Yes Not addressed Yes Yes Not addressed No Financial support for people with RHD and their families. Dedicated ARF clinic.
Observational study.
No control or comparison data.
Statistically significant decline in recurrences, severity of carditis, hospitalization, surgery and deaths over the time period 1977/1978 – 1988–2000. Declines attributed to improved adherence though this was not quantified.
∗Changes in adherence rates to secondary prophylaxis not provided.

ARF , acute rheumatic fever; RHD , rheumatic heart disease; GAS , group A streptococcus; SP , secondary prophylaxis

a Many programs continued to operate beyond specified reporting dates.



Evidence for primary prevention and primary care programs grew in the 1960s and 1970s. A number of studies of comprehensive care programs in Baltimore suggested that primary care services may reduce ARF incidence. Similarly, an ARF program in Costa Rica in the 1970s focused on improving primary prevention through changes to clinical guidelines: healthcare workers could treat sore throats without microbiological confirmation of GAS infection and with injectable penicillin instead of oral medication. This focus on primary prevention and increased use of benzathine penicillin G (BPG) seemed to accelerate the decline in ARF incidence in Costa Rica, although the introduction of a nationalized healthcare system and other social policies makes it difficult to identify the impact of individual interventions. By 1978, a detailed WHO Memorandum called for community RHD control projects, incorporating both primary and secondary prevention of ARF.


The success of early RHD control programs led to the creation of the WHO Global Programme for the Prevention of RF/RHD in Sixteen Developing Countries in 1984. Supported by external development funding, this program focused on secondary prevention, with elements of active case finding, health worker training, and health education. Growing interest in incorporating primary prevention elements emerged at a 1994 expert meeting. By 1999, all 16 countries had completed a pilot phase and over 15 million school children had been auscultated to screen for RHD. An unpublished review of this program suggested that it was highly efficient to run, costing less that $US 1 million, while raising awareness and generating new data about the burden of disease.


In some WHO regions, this work was amplified by guidelines for program standardization and implementation. Alongside this momentum, RHD control programs emerged organically in a number of locations (outlined in Table 12.1 ), including landmark programs in Cuba and the French Caribbean. Collectively, these formative control initiatives formed a template of how RHD control programs have been conceived and delivered worldwide.


A number of the RHD programs seeded by the WHO Global Programme have endured and transitioned to new funding models. For example, an ARF/RHD National Control Program began in Jamaica in 1985 as part of the WHO Global Programme following preliminary burden of disease assessments. A pilot phase was initiated in two regions, expanding in 1991 to provide coverage to the whole country through the Ministry of Health. The program focused on primary prevention and development of an RHD register. A secondary prophylaxis program is ongoing in Jamaica and notifications of ARF are provided to the Ministry of Health National Surveillance Unit. Despite these efforts, a significant burden of RHD persists and cardiac surgery for RHD is frequently necessary.


Outside the auspices of the WHO Global Programme, the Division of Pediatric Cardiology at the Federal University of Minas Gerais in Brazil established the Prevention Program for Rheumatic Fever in 1986. Since 1988, the program has been delivered by the Reference Centre for Rheumatic Fever focusing on secondary prophylaxis and encompassing an innovative package of service delivery and support services including standardized clinical protocols, transport provided to biannual clinical review, centralized appointment scheduling, free access to BPG, and accommodation for parents of inpatients. Statistically significant improvements in recurrence rates and severity and surgical demand have been recorded as the program began, though secondary prophylaxis adherence is not provided in published reports. Evidence from echocardiography screening studies demonstrates a persistently high burden of RHD in Minas Gerais. This may be indicative of an ongoing need for primary prevention activities to reduce disease incidence in similar settings.


RHD control programs in the 1970s and 1980s generally began as pragmatic responses to the high burden of RHD. Hospital records and data collection of that period were not usually intended for rigorous epidemiologic analysis or research outcomes. Observational data published in a number of papers indicated some evidence of impact—particularly in reducing the incidence of ARF and, perhaps, reduced progression of RHD. However, it was not possible to assess whether these changes were causally associated with the activities of control programs. Indeed, the incidence of ARF began to fall before the advent of antibiotics, a change widely attributed to reduction of overcrowding and improved living standards. This decline continued throughout the latter half of the 20th century, even in places without a control program, including Slovakia, Denmark and Sweden. Conversely, in the United States rates of ARF declined more dramatically at about the time penicillin became widely used for the management of pharyngitis. The contribution of control program activities to changes in RHD epidemiology was, and remains, empirically unproven. However, the etiologic complexity of GAS, ARF, and RHD meant that multimodal interventions would be needed for many people, over many years, to detect changes in critical endpoints. This would be ethically and financially challenging, particularly given that biologically plausible recommendations for reducing the burden of disease have already been adopted by WHO.


At the end of the century, it was largely accepted that RHD control programs could accelerate the decline in incidence and severity of ARF and RHD by improving access to disease-altering penicillin. Emerging evidence of cost-effectiveness of RHD control programs strengthened this assessment. Therefore, in settings with a high burden of RHD, control programs were considered an appropriate intervention to improve outcomes of people already living with disease and to reduce the incidence of new cases. This rationale remains important, particularly in places where persistent social and economic disparity contributes to ongoing disease.


2000–2005


By the turn of the millennium, new cases of ARF and RHD were rare in most high-income countries. In association with this decline, register-based control programs had largely ceased throughout the United States and were even closing in parts of New Zealand. New global health priorities emerged, including a marked increase in noncommunicable cardiovascular disease burden and HIV. In 2001, the WHO Global Programme in RHD disbanded in the face of competing health priorities. At around this period, WHO held a technical meeting on RHD, producing a document that summarized the lessons of RHD control programs of the 20th century. This WHO Technical Report Series identified six main components of national RHD control programs: secondary prevention, primary prevention, health education activities, training of healthcare providers, epidemiological surveillance, and community involvement. However, the discontinuation of the WHO Global Programme meant that these recommendations were not systematically adopted into practice or evaluated. Relatively few RHD control programs were initiated in the early 2000s.


2005–2018


Clinician-led research in low-resource settings in the early 2000s highlighted the persistent burden of RHD and very poor clinical outcomes. This work indicated an ongoing unmet need for RHD control activities in many regions of the world, including in Fiji ( Box 12.1 ) and Australia and New Zealand ( Box 12.2 ) . In 2005, the First All Africa Workshop on ARF and RHD and the resulting Drakensberg Declaration became a call to action. The Drakensberg Declaration promulgated the A.S.A.P (Awareness, Surveillance, Advocacy, Prevention) approach and called for “establishment of national primary and secondary prevention programmes for RF and RHD” in conjunction with international partners.



Box 12.1

Rheumatic Heart Disease Control in Fiji


Other rheumatic heart disease (RHD) control initiatives have evolved outside the auspices of the formative WHO program and can provide instructive lessons about priorities and sustainability. For example, RHD control activities have been underway in Fiji for over 50 years spanning different projects and funding agencies. The first Rheumatic Fever Control Program in Fiji was established in 1964 by the Fiji Medical Department to tackle the growing concern about the increasing numbers of young “cardiac cripples” resulting from acute rheumatic fever (ARF). ARF control included making “ Acute rheumatism ” a notifiable disease, regional ARF registers based at divisional hospitals and the primary care level responsible for delivery of secondary prevention, and ensuring timely attendance at specialist clinics. In 1967, concerns about overdiagnosis led to all suspected cases of ARF being referred to the Consultant Physician for confirmation and provision of medical literature about ARF.


Within 5 years of establishing the control program, there were reports that the incidence rates of both ARF and RHD were showing a declining trend, though these were based on hospital admissions, and there was evidence of underreporting. This downward trend from 35 – 37/100,000 in 1960s seemed to be confirmed up to 1984 with a reported incidence for ARF of 9.6/100,000, with the greatest decrease noted among the ethnic Indians (who previously represented over 80% of admissions) and among women.


By the turn of the century, there were growing concerns from the medical fraternity about the number of young people presenting for the first time with ARF but already with established RHD and heart failure and occasionally atrial fibrillation [Tikoduadua, personal communication Kado, 2018]. This gave impetus to the formation of a revamped Fiji RHD Control Program in 2005 with 3-year funding support from the World Heart Federation (WHF). The program was register-based (stand-alone, health services research-based) and focused on improving secondary prophylaxis, standardizing diagnosis and management of ARF/RHD, improving health worker knowledge and providing support to the community. The program established the role of the National Coordinator for RHD whose activities were governed by a Technical Advisory Committee.


In 2006, the Fiji Group A Streptococcal Project (GrASP) was established to determine the burden of GAS disease in Fiji as a fore-runner to establish a potential vaccine trial site. Studies from this project highlighted the high burden of underdiagnosis and the estimated cost of illness of ARF and RHD. GrASP also established the normal ranges of GAS antibody tests for Fiji, burden of subclinical RHD diagnosed by echocardiographic screening and explored task-shifting the role of echocardiography to briefly trained, less-skilled cadres of health workers, and development of a standard curriculum for training. The project also quantified the poor adherence to secondary prophylaxis and clinical outcomes of screening detected RHD in Fiji.


Funding for the RHD program lapsed but a concept paper prepared by the Fiji RHD Control Program formed the basis for the development of the Acute Rheumatic Fever and Rheumatic Heart Disease Policy of the Fiji Ministry of Health in 2014. This policy and the ground work of the GrASP team helped leverage funding from partners Cure Kids and the New Zealand Ministry of Foreign Affairs and Trade and ushered in the third iteration of the Fiji RHD Prevention and Control Program in 2015.


Aligned to the Fiji RF and RHD Policy with global guidance from the published Tools for Implementing RHD Control Programmes (TIPs) resource, the program aims to be integrated rather than siloed and has four major components:



  • 1.

    National register (RF information system [RFIS]) linked to the national patient information system (PATIS): This is both a patient-care and public health register custom-built to allow national, divisional, health clinic and hospital staff to effectively monitor and manage the national register-based prevention program with the explicit focus of improving secondary prophylaxis adherence and clinical management and to facilitate timely reporting.


  • 2.

    Clinical care for patients: to help clinical care providers deliver effective comprehensive ARF/RHD related services through development of best practice guidelines for ARF and sore throat management [under development], clinical pathways, and capacity building for all cadres of health staff including preservice training modules for delivery in training institutions. This includes the employment of divisional RHD coordinators and liaison nurses at all health centers.


  • 3.

    Early case detection: to develop a model for RHD early case detection for the Ministry of Health that includes improved surveillance and notification of newly diagnosed cases of ARF and RHD; pilot of utility and cost effectiveness of school health nurses conducting RHD screening in primary schools and expanded notification to include both incident cases of ARF and newly diagnosed RHD.


  • 4.

    Primary prevention, health promotion, and advocacy: to improve community awareness and engagement in ARF and RHD and destigmatize ARF/RHD.



The new control program has focused on involvement of people living with ARF/RHD, and this is reflected in the terms of reference for the RHD Technical Advisory Committee with representation of people living with RHD, health center-based RHD working groups and the popular peer-support groups, and the parallel parent/career support meetings. An off-shoot of the early peer support group formation has been the identification of RHD Champions. In particular, a young woman diagnosed with RHD through school screening participated in the Fiji Hibiscus Pageant to raise the profile of RHD. She has become the face of RHD in Fiji and has represented people living with RHD (PLWRHD) in three international forums, including a side-event organized by the WHF at the 71st World Health Assembly (WHA) in Geneva in 2018.


Advocacy work by the program and a technical advisory committee has seen Fiji’s senior health executives engage in both local and international spheres relating to RHD. Fiji played a key role in supporting the New Zealand-led RHD resolution that was endorsed at the 71st WHA.


There are some indications that awareness of ARF and RHD and adherence to secondary prophylaxis has improved. Assessment is ongoing, and the impact of these interventions will form part of the program evaluation. Interim lessons from the Fiji experience include the need to deliver care through primary care clinics, to address both primary and secondary prevention and the important role of PLWRHD to amplify program outcomes.



Box 12.2

Rheumatic Heart Disease Control in Australia and New Zealand


Alongside the renaissance of RHD control programs in endemic low-resource settings, RHD control has continued to evolve in a small number of high-income countries with a high burden of ARF/RHD in Indigenous communities. The development of RHD control programs in Australia and New Zealand is somewhat anomalous to global experience and provides some unique insights.


Research and programmatic initiatives began early in New Zealand, including an early control program in 1974, political engagement with the Maori Women’s Welfare League, and local clinical guidelines in 1984. Throughout New Zealand, RHD registers evolved organically, some with an epidemiologic surveillance focus and others addressing clinical management and program delivery. For example, the Auckland Rheumatic Fever register was developed in 1981 to support delivery of monthly secondary prophylaxis injections. The program evolved to be a nurse-led service with in–home injection delivery, very high rates of adherence and ARF recurrence rates of less than 5%. Although significant progress was achieved in secondary prophylaxis delivery, the incidence of first episode of ARF remained high in Māori and Pacific Island people in New Zealand. Between 1998 and 2001, a large randomized clinical trial of 22,000 school children in South Auckland was conducted to explore improving primary prevention through nurse-led, school-based sore throat services. Results from this study did not meet statistical significance but a subsequent meta-analysis suggested that ARF incidence could potentially be reduced by school-based sore throat programs.


The increasing disparity in ARF incidence for Maori and Pacific Island people in New Zealand made ARF a political issue in 2011. In 2012, the government of New Zealand set a Better Public Service Target to reduce the incidence of ARF by two thirds by 2017. Implemented through the Rheumatic Fever Prevention Program, this included the largest school-based sore throat program ever developed—a peak intensity it included 251 schools, covering 53,998 children in high-risk communities with regular access to throat swabs and oral antibiotics. By December 2017, the rate of ARF had fallen by 23%, but this increased subsequently. Detailed evaluation has not found a statistically significant association in areas participating in the school sore throat program, although it did reach statistical significance in the highest-incidence region.


Other initiatives in New Zealand included focused efforts to improve housing quality for people at high risk of ARF and considerable investment in public education and health literacy campaigns. Overall, reduction in ARF rates in New Zealand was likely to be multifactorial, including the impact of widespread community education, changes in health seeking behavior and primary care management of ARF.


In Australia, a high burden of ARF in urban children was documented in the 1930s – 1950s. This early study did not report the Indigenous ethnicity of participants but did demonstrate that incidence of ARF in low-income locations was triple that of high-income locations. New cases in nonIndigenous people became rare in the latter half of the 20th century but systematic case record reviews in new locations identified a very high burden of RHD for Aboriginal and Torres Strait Islander people in the 1980s. These audits provided the basis for an RHD register and control program in the Northern Territory in 1997. RHD control efforts expanded in a number of Australian states with increasing register coverage, and ARF became notifiable in a number of settings. Evaluation of register data in some locations suggested a reduction in rates of ARF. National clinical guidelines for the management of ARF and RHD were first developed in 2006 and updated in 2012.


Interest and understanding about RHD in Australia was spurred by echocardiography screening studies. In 2009, Australian government support amplified this work by providing funding for a national coordinating unit (RHDAustralia) and support to jurisdictional control programs in a number of states. An extensive review of this program in 2017 identified a number of successes, particularly improved adherence to secondary prophylaxis and epidemiologic data. The review identified future opportunities around a strengthened role for primary care, improved community education, improving automation and strengthening capacity for primary and primordial intervention. A national research initiative is now underway in Australia to develop an evidence-based strategy to eliminate RHD (the “RHD Endgame Strategy”). Community models of care, primary prevention, and comprehensive primary healthcare are likely to be part of this strategy.


The resources of Australia and New Zealand and the sharp ethnic disparities in ARF/RHD make control programs different from the rest of the world. However, the evolution in both countries to pivot from register-based secondary prophylaxis to primary prevention and an increased focused on community-based care has some parallels with international experience.



In many places, the initiation of new RHD control programs was further catalyzed by echocardiographic screening. Auscultation screening for RHD had been a part of some formative RHD control initiatives and had been endorsed in the 2001 WHO technical guidelines. In 2007, a landmark study found almost 10 times as many cases of RHD detectable on echocardiographic screening of school children in Cambodia and Mozambique than were detectable by auscultation screening alone. Echocardiography screening was rapidly and widely adopted as a new approach to addressing RHD and many thousands of young people were screened around the world over the next few years. Outstanding questions remain about the clinical utility of echocardiography screening, and whether it satisfies accepted public health criteria for diseases warranting population-based screening (see Chapter 9 ), but the increasingly accessible modality has been critical to growing awareness of the need for action on RHD.


Other events in the 2000s challenged established thinking about best practice in control programs. Longstanding debate about the most appropriate balance of primary and secondary prevention was revisited on the basis of new empiric data about primary prevention focused programs. Additionally, slight growth in cardiac surgical capacity in low-resource settings necessitated improved systems for preoperative triage and waiting list management. In parallel, new challenges in RHD control emerged including widespread stockouts of BPG, which stymied the options for effective control programs. In the absence of global clinical guidelines for RHD control, local guideline development took increasing precedence in some places. Changes in funding sources and the role of nongovernment organizations evolved, necessitating increased collaboration and a practical role for the World Heart Federation (WHF). These technical and programmatic evolutions have driven a new era of systematic national and international efforts to tackle RHD.


Contemporary RHD control programs are necessarily more complex and comprehensive than programmatic examples of the 20th century. This is well demonstrated by the experience of Sudan where a historic secondary prevention program provided the foundation for a modern RHD program with expanded scope. The Ministry of Health in Sudan participated in the WHO Global Programme on RHD from 1986 to 2000. Over 13,000 children underwent auscultation screening for RHD. The program focused on delivery of secondary prophylaxis, reportedly achieving prophylaxis coverage of 72%. In the absence of ongoing funding, the initiative ended in the year 2000. A Sudanese review of the program identified three key outstanding needs from the WHO program: increased primary prevention alongside secondary prevention, increased advocacy by nongovernment organizations, public and patients, and increased cooperation with regional partners.


In 2012 a new RHD control program began as a partnership between the Sudan Heart Society and Sudan Ministry of Health. The new program includes primary prevention, secondary prevention, and awareness raising. A National RHD Awareness Day is held in July each year to raise awareness of the burden of RHD. New clinical guidelines were developed in 2012 and updated in 2017. A National Registry for RHD was developed, initially enrolling patients into tertiary clinics with echocardiography facilities. Over 2 years, 370 patients were added to the register, the vast majority of whom had severe RHD. Echocardiography screening has been used to better understand the burden and distribution of RHD and amplify key messages about RHD. Secondary prophylaxis and access to ongoing care is limited. Adherence to secondary prophylaxis is low and compounded by shortages of BPG. Only a third of people receive ongoing follow-up and less than one in 10 people who need cardiac surgery can access it. Multiple sources of funding support the RHD control program in Sudan, including from WHO Sudan Country Office, Sudanese Children’s Heart Society and the Sudanese American Medical Association.


Zambia was also part of the WHO global program for RHD control from 1986, including large-scale auscultation screening. In 2014, a new “BeatRHD Zambia” initiative began as part of a “multifaceted public–private effort” with a particular focus on primary prevention. The new program raises awareness about sore throats to influence health seeking behavior. This has included a partnership with the Ministries of Health and Education to deliver education in nearly 50 schools and through an RHD Week. Research initiatives have demonstrated a high burden of pharyngitis. Service responses have included training for health professionals on administering BPG injections safely and management of anaphylaxis. People living with RHD have been involved in delivering some activities and trainings associated with the program.


In Nepal, a program of RHD prevention and control was initiated in 2007 as a partnership between the Nepal Heart Foundation and the Government of Nepal. This comprehensive program focuses on early detection and registration of ARF/RHD patients and establishing centers for safe administration of BPG. An overarching goal is to develop a national strategy for ARF/RHD prevention and control with an RHD control toolkit. A national computer register is centrally maintained, in addition to paper registers at hospitals and health centers. The program includes primary and secondary prophylaxis with a major focus on staff training and addressing provider fears about adverse drug reactions to BPG.


In Uganda, an RHD register was initiated in 2010 as a partnership between the Uganda Heart Institute and the Ministry of Health. Echocardiography screening in 2011 provided new evidence about the burden of disease and spurred an active research collaboration with international partners. With ongoing research and philanthropic funding, the RHD control program in Uganda has expanded from the capital Kampala to three new sites. This allows increased access to secondary prophylaxis clinics, echocardiography services, and referrals for surgical evaluation. Ongoing research efforts have elucidated determinants of secondary prophylaxis adherence, offered new insights into the treatment cascade for patients retained in ongoing care, and explored the role of peer support groups. Mortality for people with RHD in the control program is high (1 year mortality rate of 17.8%) with optimal care available to only about a third of patients.


In Tunisia, an RHD control program was established in 1978. The program entailed core elements of primary prevention, secondary prevention, and making ARF a notifiable disease. Economic development supported improvements in the social determinants of health over this time. By 2015, the incidence of ARF had fallen from a peak of 900 cases of ARF annually in the late 1970s to 9. This reduction in new cases has paralleled a reduction in new cases of RHD.


Contemporary recommendations for RHD control programs


In 2013, the WHF reemphasized the importance of control programs by identifying them as one of five targets to achieve the goal of a 25% reduction in premature deaths from ARF and RHD in people under 25 years by the 2025. Specifically, the WHF sought to “ensure that 90% of countries with endemic RHD have integrated and comprehensive control programmes by 2025.” However, the constituent parts of a “comprehensive” RHD control program were not defined.


A review of RHD control programs attempted to address this gap by analyzing WHO, WHF, and published evaluations of existing initiatives. This review informed development of a conceptual framework for describing comprehensive RHD control programs. The conceptual framework included the traditional program elements (primary and secondary prevention, community education and health worker training) alongside emerging issues in RHD control (the role of echocardiography screening, access to BPG, clinical guidelines, government engagement and advocacy). This framework is reproduced in Fig. 12.1 and underpins the RHD Action resource, Tools for Implementing RHD Control Programmes (TIPS). TIPs, in turn, articulates with the RHD Action Needs Assessment Tool.




Fig. 12.1


Conceptual Framework for Rheumatic Heart Disease Control Programs .

A conceptual framework for comprehensive rheumatic heart disease control programs. Components are arranged in approximate order of priority, working from left to right, bottom to top, in each row.

RF , rheumatic fever; RHD , rheumatic heart disease; BPG , benzathine penicillin G.


The TIPs framework helps codify a “typical” pathway in developing RHD control programs that has evolved in recent decades. A generalized trajectory often includes the following elements:




  • Concerned clinicians identify that the burden of RHD is high in a given location and often become champions for tackling the condition. This sometimes occurs in conjunction with visiting surgical teams or humanitarian outreach programs.



  • Clinicians and collaborators work to document the burden of disease through clinical record audits or active echocardiography screening.



  • The process of identifying cases of RHD catalyzes the creation of an RHD register that can be used to facilitate improved delivery of secondary prophylaxis.



  • Register development, prophylaxis delivery, community, and health worker education generally begin in a relatively defined geographic region, the location of which is often influenced by disease champions.



  • Governments or other funding agencies become engaged in RHD control and may provide some resourcing for scale up to new areas.



  • Research activities engage more people in the nascent RHD control program and can provide additional funding sources.



Therefore, the TIPs document is structured so the first five boxes reflect and support these priority areas, in addition to providing a foundation for future development. These first five priority areas are addressed in more detail in Table 12.2 .



Table 12.2

Priority Activities for New Rheumatic Heart Disease Control Programs.


















Burden of disease data Some measure of ARF or RHD incidence, recurrence, or prevalence has been needed in almost all RHD control initiatives. This information can be used to established baseline burden (to assess the impact of subsequent intervention), engage governments and funding agencies to support control programs, and predict demand. Determining burden of RHD through echocardiography screening projects has catalyzed RHD control programs in many countries in recent years. Good use of routinely collected data or focused audits is also appropriate.
Government engagement and advocacy Governments generally control access to health resources and are critical stakeholders in the success of RHD control programs. Ensuring that governments are aware of the local burden of RHD and are taking action to ensure the disease is included in National Action Plans for noncommunicable and health benefits packages is an important role for control programs.
Community education Almost all RHD control programs have included some element of community education or awareness raising around GAS, ARF, or RHD. Ensuring that people at risk of ARF present for medical evaluation of relevant symptoms is a critical element of disease control—particularly to enable primary prevention of new cases. A further discussion on raising awareness of GAS, RHD, and RHD is included in Chapter 15 .
RHD register RHD registers have an essential role in supporting prophylaxis delivery, facilitating ongoing care delivery for people living with RHD and program evaluation. They may also be used for research, for managing surgical waiting lists and for providing focused education support to people with a history of ARF or living with RHD. Many registers also provide a focus point for healthcare workers seeking advice on how to manage RHD through a lead clinician.
Medical management of ARF and RHD RHD control initiatives have a humanitarian imperative to support care delivery for people already living with ARF and RHD, alongside activities to prevent new cases of disease. In some places this means facilitating access to surgical services but even in the absence of surgical capacity, medical care can include accurate diagnosis, management of complications and comorbidities.

ARF , acute rheumatic fever; RHD , rheumatic heart disease; GAS , group A streptococcus


Longitudinal narrative review of RHD control activities also identifies common programmatic risks. In particular:




  • Individual champions who are passionate about RHD control often spearhead program development and are critical to engaging others. Programs can falter if these lynchpin individuals are no longer available. This risk can be mitigated by the formation of an RHD Committee to provide a mechanism for engaging and educating a larger number of RHD champions.



  • The protracted causal path of RHD means that activity needs to be sustained for a decade before changes in disease outcomes can be demonstrated. Impetus for program delivery may fade or be crowded out by competing priorities before an impact on disease metrics has been achieved. Using interim measures in primary and secondary prevention, health-seeking behavior can help minimize this risk and demonstrate early evidence of efficacy.



  • Monitoring and evaluation are not embedded into the design of RHD control programs, which makes it difficult to demonstrate success or identify areas for improvement.



  • Almost all measurable successes in RHD control have occurred in relatively small, defined geographic/administrative areas. Effective systems for scale up and expansion have not yet been defined, reflecting a shared challenge with other health interventions in low-resource settings.



  • Medically and technically advanced approaches (echocardiography screening and cardiac surgical services) may be valued more highly than “basic” innovations in primary healthcare delivery. There is reasonable evidence for focusing on primary healthcare, community education, community health workers, clinical guidelines, and penicillin delivery as first priorities in RHD control.



The 2018 WHO resolution on ARF and RHD represents a new era in RHD control. The resolution calls for support in “ identifying disease burden and, where appropriate, in developing and implementing rheumatic heart disease programmes and strengthening health systems in order to improve disease surveillance, increase the availability and training of the community and primary health care workforce, and ensure reliable access to affordable prevention, diagnostic and treatment tools .” Additionally, increased rigor in monitoring and evaluation will be needed so that best practice can be identified and refined. New systems, leadership and institutions will be needed to meet this need. As technical support capacity grows some interim key lessons can be extracted from existing program experience, outlined in Box 12.3 . These pragmatic suggestions closely parallel the six recommended control program elements of the 2001 WHO Technical Report on ARF and RHD (secondary prevention, primary prevention, health education, health worker training, epidemiologic surveillance, and community involvement). The challenge now, as it was then, is to support countries to implement these elements in high quality, scalable and replicable ways.


Feb 2, 2021 | Posted by in RHEUMATOLOGY | Comments Off on Rheumatic Heart Disease Control Programs, Registers, and Access to Care
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