Operational performance of the 2023 World Heart Federation screening criteria for rheumatic heart disease in a high-risk, resource-limited population

Abstract

BACKGROUND: Rheumatic heart disease (RHD) remains a major cause of morbidity and mortality in low-resource settings, underscoring the need for accurate and scalable screening approaches. The 2023 World Heart Federation (WHF) screening criteria were designed to facilitate task-shifting but real-world performance data are limited. We evaluated the diagnostic accuracy and operational performance of the 2023 WHF criteria in a large school-based screening programme in Uganda. METHODS: We conducted a cross-sectional diagnostic accuracy study among 11 198 children aged 5-17 years screened between September and November 2024 in Lira District, Northern Uganda. All screen-positive children (n=522) and a stratified random sample of screen-negative children (n=3301) underwent confirmatory echocardiography. The index test was nurse-performed handheld echocardiography using a standardised three-view protocol. The reference standard was comprehensive echocardiography interpreted by a blinded expert adjudication panel using full WHF criteria. Diagnostic accuracy metrics included sensitivity, specificity, positive and negative predictive values and likelihood ratios. Partial verification bias was addressed using multiple imputation. RESULTS: Among 3802 participants who had the reference standard test and no alternative cardiac diagnosis, 122 cases of RHD were identified. After accounting for partial verification bias, sensitivity was 60.0% (95% CI 46.6 to 73.5%) and specificity 96.3% (95% CI 96.0 to 96.7%). Positive and negative predictive values were 19.5% and 99.4%, respectively. Complete-case analysis overestimated sensitivity (80.3%) and underestimated specificity (89.0%). Missed cases were predominantly early-stage or aortic valve disease, with no advanced cases missed. Exploratory analysis using single-expert interpretation yielded comparable performance. CONCLUSIONS: In this large real-world evaluation, nurse-performed handheld echocardiographic screening using the 2023 WHF criteria demonstrated high specificity and negative predictive value, supporting its role as a scalable frontline triage strategy for RHD. After accounting for partial verification bias, sensitivity was moderate, with missed cases representing early-stage disease, highlighting opportunities to improve case detection through optimisation of screening workflows, training and technology.