Over the past 5 years, the advent of echocardiographic screening for rheumatic heart disease (RHD) has revealed a higher RHD burden than previously thought. In light of this global experience, the development of new international echocardiographic guidelines that address the full spectrum of the rheumatic disease process is opportune. Systematic differences in the reporting of and diagnostic approach to RHD exist, reflecting differences in local experience and disease patterns. The World Heart Federation echocardiographic criteria for RHD have, therefore, been developed and are formulated on the basis of the best available evidence. Three categories are defined on the basis of assessment by 2D, continuous-wave, and color-Doppler echocardiography: ‘definite RHD’, ‘borderline RHD’, and ‘normal’. Four subcategories of ‘definite RHD’ and three subcategories of ‘borderline RHD’ exist, to reflect the various disease patterns. The morphological features of RHD and the criteria for pathological mitral and aortic regurgitation are also defined. The criteria are modified for those aged over 20 years on the basis of the available evidence. The standardized criteria aim to permit rapid and consistent identification of individuals with RHD without a clear history of acute rheumatic fever and hence allow enrollment into secondary prophylaxis programs. However, important unanswered questions remain about the importance of subclinical disease (borderline or definite RHD on echocardiography without a clinical pathological murmur), and about the practicalities of implementing screening programs. These standardized criteria will help enable new studies to be designed to evaluate the role of echocardiographic screening in RHD control.

This revision of the Jones criteria now brings them into closer alignment with other international guidelines for the diagnosis of acute rheumatic fever by defining high-risk populations, recognizing variability in clinical presentation in these high-risk populations, and including Doppler echocardiography as a tool to diagnose cardiac involvement.

In the 21(st) century, rheumatic fever (RF) and rheumatic heart disease (RHD) are neglected diseases of marginalized communities. Globally, RHD remains the most-common cardiovascular disease in young people aged <25 years. Although RF and RHD have been almost eradicated in areas with established economies, migration from low-income to high-income settings might be responsible for a new burden of RHD in high-income countries. The World Heart Federation (WHF) and its Working Group on RF and RHD unites global experts, combines their experience and enthusiasm, and provides a platform for RHD control. This paper is a declaration of the WHF institutional strategic goal--a 25% reduction in premature deaths from RF and RHD among individuals aged <25 years by the year 2025. The position statement affirms WHF commitments to five key strategic targets: comprehensive register-based control programmes, global access to benzathine penicillin G, identification and development of public figures as 'RHD champions', expansion of RHD training hubs, and support for vaccine development. In this paper, we also review existing barriers to RF and RHD control and identify the actions required to change the trajectory of control for these diseases. This approach provides the foundation for governments, civil society, patient advocates, clinicians, researchers, and funding agencies to develop partnerships and unify global efforts to control RF and RHD. The WHF plans to expand this position statement to an operational plan that will be founded on science, research, and quantifiable progress indicators to impact positively on the millions of people who are affected by RHD and its long-term sequelae.

Background— Echocardiographic screening for rheumatic heart disease (RHD) is becoming more widespread, but screening studies to date have used different echocardiographic definitions. The World Heart Federation has recently published new criteria for the echocardiographic diagnosis of RHD. We aimed to establish the prevalence of RHD in high-risk Indigenous Australian children using these criteria and to compare the findings with a group of Australian children at low risk for RHD. Methods and Results— Portable echocardiography was performed on high-risk Indigenous children aged 5 to15 years living in remote communities of northern Australia. A comparison group of low-risk, non-Indigenous children living in urban centers was also screened. Echocardiograms were reported in a standardized, blinded fashion. Of 3946 high-risk children, 34 met World Heart Federation criteria for definite RHD (prevalence, 8.6 per 1000 [95% confidence interval, 6.0–12.0]) and 66 for borderline RHD (prevalence, 16.7 per 1000 [95% confidence interval, 13.0–21.2]). Of 1053 low-risk children, none met the criteria for definite RHD, and 5 met the criteria for borderline RHD. High-risk children were more likely to have definite or borderline RHD than low-risk children (adjusted odds ratio, 5.7 [95% confidence interval, 2.3–14.1]; P <0.001). Conclusions— The prevalence of definite RHD in high-risk Indigenous Australian children approximates what we expected in our population, and no definite RHD was identified in the low-risk group. This study suggests that definite RHD, as defined by the World Heart Federation criteria, is likely to represent true disease. Borderline RHD was identified in children at both low and high risk, highlighting the need for longitudinal studies to evaluate the clinical significance of this finding.