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

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.

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.