Rheumatic Heart Disease (RHD) is the cardiac consequence of acute rheumatic fever (ARF), an inflammatory disease triggered by streptococcal pharyngitis. Although the prevalence of RHD has decreased in high-income countries, lack of social and economic development and poor primary prevention - mainly in in low- and middle-income countries - perpetuate an environment where RHD remains endemic. It is estimated that RHD continues to affect nearly 33 million people worldwide.
The main burden of RHD to public health systems consists of repeated hospital admissions and cardiac surgeries in the following decades after initial cardiac damage. If RHD is detected in its early stages, secondary prophylaxis (regular penicillin injections) can be initiated to prevent new episodes of ARF, avoiding further valve damage and progression of RHD. In high prevalence regions, RHD meets the traditional screening criteria defined by Wilson and Jungner,
The PROVAR study (
The regulatory process started in the end of 2013, and the study was approved by the institutional review boards of UFMG and CNHS, as well as the State Boards of Health and Education. Legal sectors then analyzed the proposal and approved it, and there was no extra cost for the government or patients. The Department of Education selected schools with the highest socioeconomic vulnerability, with a special interest in areas with limited access to healthcare. Prior to screening, an educational curriculum was implemented, including lectures and printed material, for students and their parents, teachers and school staff regarding the importance of streptococcal pharyngitis, ARF and RHD. Parents were asked to sign an informed consent form - a requirement of Brazilian research regulations. Non-physician personnel started echocardiographic screening in July 2014 after a 12-week hands-on training supervised by an expert cardiologist and online RHD education modules (WiRED International,
When abnormalities were detected during the screening echocardiogram, a medical student called the child's parents to schedule a follow-up standard exam, which was performed by an experienced pediatric cardiologist at the university's hospital. Borderline RHD cases had a clinical consult and a follow up echocardiogram scheduled within one year. For patients found to have definite RHD based on the screening and follow-up echocardiogram, in addition to the referral to a specialized outpatient clinic, penicillin prophylaxis was initiated and more frequent follow-up echocardiograms were recommended based on the observations of subsequent visits.
The PROVAR team has educated over 20,000 children on RHD, and has performed over 9,000 echocardiograms in 32 schools.
The accuracy of these non-physicians (nurses, technicians and medical students) for basic interpretation of simplified screening exams was tested and had good results: overall sensitivity of 83% and specificity of 85%.
There were several challenges during the implementation and conduction of the PROVAR study. During the initial ethics approval - which took almost 90 days in Brazil - some resistance from the Board of Education and Health was observed, mainly related to research procedures and the impact on the school routine. Some modifications to the consent forms were required, which took an extra 4 months. There was also questioning by the Board of Health, related to concerns about task-shifting (which could be questioned by medical and nursing councils) and the impact of screening on the population: referral strategies, availability of penicillin for all positive cases - considering the shortage observed in Brazil - and information for families with children with positive exams to avoid stigmatization.
During the initial steps of the field study, the PROVAR team faced several challenges. At first, the main challenges were related to the lack of involvement of school representatives with the program and lack of understanding possible benefits. Overall, there was low parental engagement with the project with the poorest attendance to educational sessions seen in the lowest socioeconomic status areas. The proportion of signed informed consents was low (about 35%: 5,996 out of 17,000 children)
There were also several challenges for follow-up visits. Phone contact with parents was often not possible because the children did not have the contact information, numbers changed frequently, and schools are not always authorized to disclose contact information. In addition, 35% of families failed to show up for scheduled appointments. This may presumably be, in part, due to patients being asymptomatic and families not being convinced of the importance of monitoring. Based on conversations with parents who have not returned for the follow-up, our team hypothesizes that financial constraints, living in distant neighborhoods and impossibility to miss a workday also contributed to the lack of compliance with recommendations for follow up in the study protocol. However, data about the reasons for the low compliance were not systematically collected. Some strategies, such as subsequent educational calls and flexible follow-up dates have been recently tested, with relative success. There is also a plan to provide follow-up echocardiograms in the schools, immediately after the screening process, to improve follow-up and referral rates. Additionally, educational and public awareness materials for different scenarios are under development, considering the questions posed by the families.
Fighting RHD is a challenge for Brazil. Understanding the burden of the disease and how it affects the health system is the primary objective of PROVAR. The PROVAR program will continue to screen for RHD in underserved areas, but we are now expanding our efforts to private schools, where students' socioeconomic conditions are much better. A much lower prevalence is anticipated in this "control" population.
Authorities should be prepared to effectively eradicate RHD. To do so, we believe ARF and RHD should be included in the list of notifiable diseases, since these conditions have a considerably high incidence in Brazil. Group A streptococcal infections (triggers for ARF) are communicable from patient to patient and transmission can be eliminated with eradication.
The next steps of PROVAR are related to the diagonal integration of RHD screening in primary care. This strategy is now being implemented in Montes Claros (MG, Brazil) (2 primary care centers have already been enrolled) and will be launched in Belo Horizonte and Nova Lima (MG - Brazil) in the following months, also including screening for other degenerative and congenital valve conditions. For this purpose, primary care physicians will also be trained in basic echocardiographic skills for routine evaluation of different age groups, utilizing the same telemedicine infrastructure for uploads and remote interpretation. We believe this integration is a crucial step for long-term sustainability of echocardiographic screening and for its integration into healthcare policies, and will allow assessment of the ideal screening strategies, including cost-effectiveness analysis. If RHD screening in these scenarios proves to be feasible and cost-effective, the final step would be to include it as a priority for the discussion of the Public Health System budget in the long run.