The World Heart Federation (WHF) published the first evidence-based guidelines on the echocardiographic diagnosis of rheumatic heart disease (RHD) in 2012. These guidelines have since been applied internationally in research and clinical practice. Substantial research has assessed the utility of the 2012 WHF criteria, including its applicability in low-resource settings. This article summarises the evidence regarding the performance of the guidelines.A scoping review assessing the performance of the guidelines was performed. Cochrane, Embase, Medline, PubMed Lilacs, Sielo, and Portal BVS databases were searched for studies on the performance of the guidelines between January 2012-March 2023, and 4047 manuscripts met the search criteria, of which 34 were included. This included papers assessing the specificity, inter-rater reliability, application using hand-carried ultrasound, and modification of the criteria for simplicity. The review followed the PRISMA Extension for Scoping Reviews guideline.The WHF 2012 criteria were 100% specific for definite RHD when applied in low-prevalence populations. The criteria demonstrated substantial and moderate inter-rater reliability for detecting definite and borderline RHD, respectively. The inter-rater reliability for morphological features was lower than for valvular regurgitation. When applied to hand-carried ultrasound performed by an expert, modified versions of the criteria demonstrated a sensitivity and specificity range of 79-90% and 87-93% respectively for detecting any RHD, performing best for definite RHD. The sensitivity and the specificity were reduced when performed in task-sharing but remains moderately accurate.The WHF 2012 criteria provide clear guidance for the echocardiographic diagnosis of RHD that is reproducible and applicable to a range of echocardiographic technology. Furthermore, the criteria are highly specific and particularly accurate for detecting definite RHD. There are limitations in applying all aspects of the criteria in specific settings, including task-sharing. This summary of evidence can inform the updated version of the WHF guidelines to ensure improved applicability in all RHD endemic regions.

Transthoracic echocardiography is the gold standard for early detection of rheumatic heart disease (RHD) in asymptomatic children living in high-risk regions. Advances in technology allowing miniaturisation and increased portability of echocardiography devices have improved the accessibility of this vital diagnostic tool in RHD-endemic locations. Automation of image optimisation techniques and simplified RHD screening protocols permit use by non-experts after a brief period of training. While these changes are welcome advances in the battle to manage RHD, it is important that the sensitivity and specificity of RHD detection be maintained by all echocardiography users on any device to ensure accurate and timely diagnosis of RHD to facilitate initiation of appropriate therapy. This review of the evolution of echocardiography and its use in the detection of rheumatic valve disease may serve as a reminder of the key strengths and potential pitfalls of this increasingly relied-upon diagnostic test.

Rheumatic heart disease (RHD) remains prevalent within First Nations Australian communities. RHD is more common in females and peak prevalence corresponds with childbearing age. Significant valvular disease can complicate pregnancy. Current practice in Northern Australia is to refer pregnant women for echocardiography if there are signs or symptoms of possible cardiac pathology or a history of acute rheumatic fever (ARF) or RHD. It is not currently routine practice to offer echocardiographic screening for all pregnant women at high risk of RHD.This study aimed to assess the current referral practices for echocardiography and disease patterns in pregnant women in the Northern Territory, Australia-a region with a known high prevalence of RHD in the First Nations population.A retrospective analysis of all echocardiography referrals of pregnant women over a 4-year period was performed. Data included indication for echocardiography, clinical history, echocardiographic findings, and location of delivery. Comparisons were made using Fisher's exact and Mann-Whitney U tests.A total of 322 women underwent echocardiography during pregnancy: 195 First Nations and 127 non-Indigenous women (median age, 25 vs 30 years, respectively; p<0.01). Indications for echocardiography differed by ethnicity, with history of ARF or RHD being the most common indication in First Nations women, and incidental murmur the most common in non-Indigenous women. First Nations women were more likely to have abnormal echocardiograms (35.9% vs 11.0% in non-Indigenous women; p<0.01) or a history of ARF or RHD (39.4% vs 0.8%; p<0.01), but less likely to have documented cardiac symptoms as an indication for echocardiography (8.2% vs 20.5%; p<0.01). New cardiac diagnoses were made during pregnancy in 11 (5.6%) First Nations and two (1.6%) non-Indigenous women (p=0.02). Moderate or severe valve lesions were detected in 26 (13.3%) First Nations women (all previously diagnosed), and 11 (5.6%) had previous cardiac surgery. No severe valve lesions were identified in the non-Indigenous group. Interstate transfer to a tertiary centre with valve intervention services was required during pregnancy or the puerperium for 12 (6.2%) First Nations women and no non-Indigenous women.Amongst pregnant women in the Northern Territory who had an indication for echocardiography, First Nations women were more likely to have abnormal echocardiograms. This was mainly due to valvular disease secondary to RHD. Cardiac symptoms were infrequently recorded as an indication for echocardiography in First Nations women, suggesting possible underappreciation of symptoms. Having a low threshold for echocardiographic investigation, including consideration of universal screening during pregnancy, is important in a high RHD-burden setting such as ours. A better understanding of the true prevalence and spectrum of disease severity in this population would enable health services to invest in appropriate resources.

Rheumatic heart disease (RHD) is an important and preventable cause of morbidity and mortality among children and young adults in low-income and middle-income countries, as well as among certain at-risk populations living in high-income countries. The 2012 World Heart Federation echocardiographic criteria provided a standardized approach for the identification of RHD and facilitated an improvement in early case detection. The 2012 criteria were used to define disease burden in numerous epidemiological studies, but researchers and clinicians have since highlighted limitations that have prompted a revision. In this updated version of the guidelines, we incorporate evidence from a scoping review, an expert panel and end-user feedback and present an approach for active case finding for RHD, including the use of screening and confirmatory criteria. These guidelines also introduce a new stage-based classification for RHD to identify the risk of disease progression. They describe the latest evidence and recommendations on population-based echocardiographic active case finding and risk stratification. Secondary antibiotic prophylaxis, echocardiography equipment and task sharing for RHD active case finding are also discussed. These World Heart Federation 2023 guidelines provide a concise and updated resource for clinical and research applications in RHD-endemic regions.

INTRODUCTION: Rheumatic heart disease (RHD) causes significant morbidity and mortality in young people from disadvantaged populations. Early detection through echocardiography screening can facilitate early access to treatment. Large-scale implementation of screening could be feasible with the combination of inexpensive standalone ultrasound transducers and upskilling non-expert practitioners to perform abbreviated echocardiography. METHODS AND ANALYSIS: A prospective cross-sectional study will evaluate an abbreviated echocardiography screening protocol for the detection of latent (asymptomatic) RHD in high-risk populations. The study will evaluate the diagnostic accuracy of health worker conducted single parasternal long axis view with a sweep using handheld devices (SPLASH) (Philips Lumify S4-1 phased array transducer). Each participant will have at least one reference test performed on the same day by an expert echocardiographer. Diagnosis of RHD will be determined by a panel of three experts, using 2012 World Heart Federation criteria. Sensitivity and specificity of the index test will be calculated with 95% CIs, to determine diagnostic accuracy of a screen-and-refer approach to echocardiography screening for RHD. Remote review of SPLASH images obtained by health workers will facilitate evaluation of the sensitivity and specificity of an alternative approach, using external review of health worker obtained SPLASH images to decide onward referral. ETHICS AND DISSEMINATION: Ethics approval was obtained from the Human Research Ethics Committee of the Northern Territory Department of Health and Menzies School of Health Research, for the project to be carried out in Timor-Leste (HREC 2019-3399), and in Australia, following review by the Aboriginal Ethics subcommittee (HREC 2019-334). Ethical and technical approval was granted in Timor-Leste, by the Institute National of Health Research Ethics and Technical Committee (1073-MS-INS/GDE/VII/2019). Study results will be disseminated in the communities involved in the study, and through peer-reviewed publications and conference abstracts. TRIAL REGISTRATION NUMBER: The Australia New Zealand Clinical Trials Registry (ACTRN12620000122954).