• Maria Carmo P Nunes, Craig Sable, Bruno R Nascimento, Lima Emilly Malveira de EM Statistical Department, Universidade Federal de Minas Gerais, Belo Horizonte, Minas Gerais, Brazil (E.M.d.L., E.A.C.)., Jose Luiz Padilha da Silva, Adriana C Diamantino, Kaciane K B Oliveira, Emmy Okello, Twalib Aliku, Peter Lwabi, Enrico Antonio Colosimo, Ribeiro Antonio Luiz P ALP Serviço de Cardiologia e Cirurgia Cardiovascular e Centro de Telessaúde do Hospital das Clínicas da UFMG, Belo Horizonte, Minas Gerais, Brazil , and Andrea Z Beaton.
• Serviço de Cardiologia e Cirurgia Cardiovascular e Centro de Telessaúde do Hospital das Clínicas da UFMG, Belo Horizonte, Minas Gerais, Brazil (M.C.P.N., B.R.N., A.C.D., K.K.B.O., A.L.P.R.).
• Circ Cardiovasc Imaging. 2019 Feb 1; 12 (2): e007928.

BackgroundThe 2012 World Heart Federation Criteria are the current gold standard for the diagnosis of latent rheumatic heart disease (RHD). Because data and experience using these criteria have grown, there is opportunity to simplify and develop outcome prediction tools. We aimed to develop a simple echocardiographic score applicable for RHD screening with potential to predict disease progression.MethodsThis study included 3 cohorts used for score derivation (n=9501), score validation (n=7312), and assessment of outcomes prediction (n=227). In the derivation cohort, variables independently associated with definite RHD were assigned point values proportional to their regression coefficients. The sum of these values was stratified into low (0-6), intermediate (7-9), and high (≥10) risk.ResultsFive components were selected for score development, including mitral valve anterior leaflet thickening, excessive leaflet tip motion, and regurgitation jet length ≥2 cm, and aortic valve focal thickening and any regurgitation. The score showed optimal discrimination and calibration for RHD diagnosis in the derivation and validation cohorts (C statistic, 0.998 and 0.994, respectively), with good discrimination for predicting disease progression (C statistic, 0.811). Progression-free survival rate in the low-risk children at 1-, 2-, and 3-year follow-up was 100%, 100%, and 93%, respectively, compared with 90%, 60%, and 47% in high-risk group. The point-based score was strongly associated with disease progression (hazard ratio, 1.270; 95% CI, 1.188-1.358; P<0.001).ConclusionsThis simplified score, based on components of the World Heart Federation criteria, is highly accurate to recognize definite RHD and provides the first tool for risk stratification, assigning children with latent RHD to low, intermediate, or high risk based on echocardiographic features at diagnosis.

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