Maria Carmo P Nunes1,2, Craig Sable3, Bruno R Nascimento1,2, Emilly Malveira de Lima4, Jose Luiz Padilha da Silva5, Adriana C Diamantino1, Kaciane K B Oliveira1, Emmy Okello6, Twalib Aliku6, Peter Lwabi6, Enrico Antonio Colosimo4, Antonio Luiz P Ribeiro1,2, Andrea Z Beaton7. 1. 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.). 2. Department of Internal Medicine, School of Medicine, Belo Horizonte, Minas Gerais, Brazil (M.C.P.N., B.R.N., A.L.P.R.). 3. Children's National Health System, Washington, DC (C.S.). 4. Statistical Department, Universidade Federal de Minas Gerais, Belo Horizonte, Minas Gerais, Brazil (E.M.d.L., E.A.C.). 5. Statistical Department, Universidade Federal do Paraná, Curitiba, Brazil (J.L.P.d.S.). 6. Uganda Heart Institute, Kampala (E.O., T.A., P.L.). 7. The Heart Institute, Cincinnati Children's Hospital Medical Center, OH (A.Z.B.).
Abstract
BACKGROUND: The 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. METHODS: This 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. RESULTS: Five 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). CONCLUSIONS: This 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.
BACKGROUND: The 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. METHODS: This 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. RESULTS: Five 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). CONCLUSIONS: This 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.
Authors: Dalton Bertolim Précoma; Gláucia Maria Moraes de Oliveira; Antonio Felipe Simão; Oscar Pereira Dutra; Otávio Rizzi Coelho; Maria Cristina de Oliveira Izar; Rui Manuel Dos Santos Póvoa; Isabela de Carlos Back Giuliano; Aristóteles Comte de Alencar Filho; Carlos Alberto Machado; Carlos Scherr; Francisco Antonio Helfenstein Fonseca; Raul Dias Dos Santos Filho; Tales de Carvalho; Álvaro Avezum; Roberto Esporcatte; Bruno Ramos Nascimento; David de Pádua Brasil; Gabriel Porto Soares; Paolo Blanco Villela; Roberto Muniz Ferreira; Wolney de Andrade Martins; Andrei C Sposito; Bruno Halpern; José Francisco Kerr Saraiva; Luiz Sergio Fernandes Carvalho; Marcos Antônio Tambascia; Otávio Rizzi Coelho-Filho; Adriana Bertolami; Harry Correa Filho; Hermes Toros Xavier; José Rocha Faria-Neto; Marcelo Chiara Bertolami; Viviane Zorzanelli Rocha Giraldez; Andrea Araújo Brandão; Audes Diógenes de Magalhães Feitosa; Celso Amodeo; Dilma do Socorro Moraes de Souza; Eduardo Costa Duarte Barbosa; Marcus Vinícius Bolívar Malachias; Weimar Kunz Sebba Barroso de Souza; Fernando Augusto Alves da Costa; Ivan Romero Rivera; Lucia Campos Pellanda; Maria Alayde Mendonça da Silva; Aloyzio Cechella Achutti; André Ribeiro Langowiski; Carla Janice Baister Lantieri; Jaqueline Ribeiro Scholz; Silvia Maria Cury Ismael; José Carlos Aidar Ayoub; Luiz César Nazário Scala; Mario Fritsch Neves; Paulo Cesar Brandão Veiga Jardim; Sandra Cristina Pereira Costa Fuchs; Thiago de Souza Veiga Jardim; Emilio Hideyuki Moriguchi; Jamil Cherem Schneider; Marcelo Heitor Vieira Assad; Sergio Emanuel Kaiser; Ana Maria Lottenberg; Carlos Daniel Magnoni; Marcio Hiroshi Miname; Roberta Soares Lara; Artur Haddad Herdy; Cláudio Gil Soares de Araújo; Mauricio Milani; Miguel Morita Fernandes da Silva; Ricardo Stein; Fernando Antonio Lucchese; Fernando Nobre; Hermilo Borba Griz; Lucélia Batista Neves Cunha Magalhães; Mario Henrique Elesbão de Borba; Mauro Ricardo Nunes Pontes; Ricardo Mourilhe-Rocha Journal: Arq Bras Cardiol Date: 2019-11-04 Impact factor: 2.000
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