• Research Review
• Journal Summary

Authors: G Karthikeyan and BM Mayosi

Reference: Circulation. 2009;120:709-713. http://circ.ahajournals.org/cgi/content/abstract/CIRCULATIONAHA.108.836510v1
Link to PDF: http://circ.ahajournals.org/cgi/reprint/CIRCULATIONAHA.108.836510?ijkey=z7GXTp4XpzMkHam&keytype=ref

Summarized by: Catherine Coleman, Editor in Chief, ProCor

Rheumatic heart disease (RHD) is a global problem, affecting more than 16 million people worldwide and resulting in more than 200,000 deaths each year. RHD is a "disease of poverty," and thus primarily destroys health in less developed countries, particularly Africa.

Some developed countries are experiencing a resurgence of RHD, and I can't help but cynically wonder if this is what it will take to help focus attention on a disease that is both devastating and neglected.

Ganesan Karthikeyan, from the All India Institute of Medical Sciences, New Delhi, India, and Bongani Mayosi, Department of Medicine, University of Cape Town, South Africa (corresponding author; email bongani.mayosi@uct.ac.za) are the authors of a special report published in Circulation. "Is primary prevention of rheumatic fever the missing link in the control of rheumatic heart disease in Africa?" offers a concise and challenging assessment of current and emerging strategies to control RHD, and presents a powerful argument for the syndromic treatment of suspected streptococcal sore throat as a primary prevention strategy which could be successfully implemented in resource-poor settings globally.

Equally important, throughout the report they remind us that the underlying and fundamental cause of RHD is poor living conditions, and advocate that "over and above the preventive strategies, living conditions and health care must improve substantially in order to reduce disease burden..."

This Special Report is exceptionally worthy of reading, discussing, and sharing but is not freely available at present. Following is a summary of key points. We will promptly provide additional information if the publisher opens access to the report.
---------------------

The report summarizes a wide range of "rationales and strategies" for preventing rheumatic fever and RHD, then poses and discusses key questions that are essential to clinical and policy decision making: Why is secondary prevention essential but not sufficient for preventing RHD? Is primary prevention an effective strategy, and is it a feasible strategy in Africa? Can clinical (non-bacteriologic) diagnosis of streptococcal sore throat promote primary prevention within Africa's health systems?

Primary versus secondary treatment
As no proven treatment can alter the natural history of rheumatic fever, prevention is key to reducing its burden. Proven preventive strategies, both primary and secondary, have existed for more than 50 years. Primary prevention detects sore throats caused by symptomatic group A streptococcal (GAS) infection in susceptible individuals (mainly children) and treats it with oral or parenteral penicillin. Secondary prophylaxis periodically administers penicillin to people who have had previous episodes of rheumatic fever or who have RHD, in order to prevent recurrent GAS sore throat. Current approaches to RHD control rely almost entirely on secondary prophylaxis, as recommended by WHO and others. While secondary prevention reduces the risk of recurrences of rheumatic fever, it has not been shown to reduce development of chronic RHD or RHD-caused mortality.

The Special Report addresses a range of concerns about adopting primary antibiotic prophylaxis to prevent RF and RHD as a public health intervention in the community and presents diverse analyses of affordability, logistical feasibility, issues such as health-seeking behavior in resource-poor countries, etc. By primary antibiotic prohylaxis, the authors are referring to the syndromic treatment of symptomatic sore throat that is suspected to be due to bacterial rather than viral infection on clinical grounds (they are NOT referring to screening of asymptomatic children for strep throat). In reviewing affordability issues, the authors note that available cost estimates are based on findings from developed countries and may not be applicable to Africa, where efficient, effective integration of primary prevention into existing systems could eliminate many of the assumed barriers.

Secondary prevention cannot adequately prevent RHD, according to the authors, because in poor countries, "susceptible patients have already suffered substantial valve damage from unrecognized episodes of RF by the time they come to clinical attention. Often patients present for the first time with symptoms due to the hemodynamic consequences of the resulting valve disease. The subsequent clinical course of these patients is largely determined by the natural history of the valve lesions. Although secondary prophylaxis reduces recurrent RF episodes, there is no evidence to suggest that such treatment alters natural history once significant valve damage has occurred. Moreover, the early episodes of acute RF in children in Africa often follow a fulminant course, which may require surgery in the acute phase of illness."

Preventing the first episode of rheumatic carditis thus is essential in order to prevent rheumatic valve disease. The effectiveness of primary prevention is demonstrated with numerous examples, with the strongest evidence coming from Costa Rica and Cuba, which have virtually eliminated rheumatic fever utilizing primary prevention programs.

The route to primary prevention: Clinical diagnosis
The authors propose that "Clinical algorithms for diagnosing bacterial pharyngitis, although far less accurate than throat swab culture, can conceivably substantially reduce the number of patients receiving unnecessary antibiotic therapy. Several clinical decision rules are available for the diagnosis of streptococcal sore throat. In order to be an effective replacement for bacteriologic diagnosis, the decision rule must be easy to apply and must correctly diagnose most cases of streptococcal sore throat while keeping overtreatment of viral sore throats to a minimum." Diagnostic accuracy of clinical decision rules and their success in identifying children in need of treatment while reducing unnecessary antibiotic treatment are impressive. A variety of algorithms are explored, with the authors concluding that "...the systematic implementation of a primary prevention program incorporating any of these algorithms would represent a major improvement over the existing situation in Africa," and they further recommend tailoring decision rules to specific populations to maximize their performance characteristics.

Delivering primary prevention: The logistics of implementation
Costa Rica and Cuba provide inspiring models of successful primary prevention programs. In Costa Rica, a program launched in the 1970s eliminated throat swab culture to confirm streptococcal sore throats, and diagnosis was made entirely on clinical grounds; sore throats were treated with a single intramuscular injection of penzathine penicillin; and an educational campaign informed physicians ,nurses, health technicians, and medical students about the need to treat streptococcal sore throats. Over the next 20 years, RF incidence declined dramatically, to one per 100,000. A similar campaign in the Pinar del Rio province of Cuba detected and treated sore throats, increased community awareness, and provided secondary prevention; again RF incidence fell dramatically. Both programs were integrated into existing healthcare systems, so minimal cost was incurred.

Addressing Africa's RHD burden
The report urges that existing policies and systems take on RHD prevention, and urge expansion of the Awareness Surveillance Advocacy Prevention (ASAP) Program. "Treatment of sore throat is already part of the Integrated Management of Childhood Illness program in Africa, and penicillin is on the essential drug list of the WHO," the authors note. "Therefore, our call is for the implementation of existing policy within existing health systems, which we believe is within the reach of most African countries. The Awareness Surveillance Advocacy Prevention (ASAP) Program is an ongoing prevention program being implemented under the auspices of the Pan African Society of Cardiology (www.pascar.co.za) with the support of the national Departments of Health of South Africa, Ghana, and Egypt and the World Heart Federation. In addition to the objectives of raising awareness about the disease in the community and among physicians, ASAP aims to implement primary prevention strategies in tandem with secondary prevention within the existing primary healthcare system. Pilot programs will be developed at selected sentinel/ demonstration sites in the participating countries, which will ultimately serve as the basis for the establishment of national programs for the control of RF/RHD in these countries."

They conclude, "We believe that a preventive program that relies almost exclusively on secondary prevention, such as the one advocated by the WHO, is unlikely to reduce the burden of RF and RHD in Africa. A strategy consisting of educating health personnel to recognize bacterial sore throat using simple clinical algorithms (instead of relying on a bacteriologic diagnosis), followed by a single injection of benzathine penicillin for the treatment of suspected cases, has been shown to be effective. The implementation of such a strategy through the existing healthcare infrastructure may be efficient and cost-effective and has the potential to reduce the burden of RF/RHD. The recently devised ASAP program is the first step toward implementing such a comprehensive preventive strategy in Africa. The initial effort and expense in integrating primary prevention into national RHD programs can be expected to be more than offset by the reduction in the number of patients with severe valvular disease who will subsequently require expensive tertiary care. Finally, it cannot be overemphasized that RF/RHD is a disease of poverty. Therefore, over and above the preventive strategies, living conditions and access to healthcare must improve substantially in order to reduce disease burden in sub-Saharan Africa."

Read a series of briefings issued during Rheumatic Fever Week in South Africa (3-7 August 2009), including a description of the ASAP program, on ProCor's site: www.procor.org/news/news_show.htm?doc_id=984447.