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.).
Department of Internal Medicine, School of Medicine, Belo Horizonte, Minas Gerais, Brazil (M.C.P.N., B.R.N., A.L.P.R.).
Circ Cardiovasc Imaging. 2019 Feb;12(2):e007928. doi: 10.1161/CIRCIMAGING.118.007928.
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.
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.
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).
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.
2012 年世界心脏联盟标准是目前诊断潜伏性风湿性心脏病（RHD）的金标准。由于使用这些标准的数据和经验不断增加，因此有机会简化和开发预后预测工具。我们旨在开发一种简单的超声心动图评分，适用于 RHD 筛查，并具有预测疾病进展的潜力。
本研究包括用于评分推导（n=9501）、评分验证（n=7312）和结局预测评估（n=227）的 3 个队列。在推导队列中，与明确 RHD 独立相关的变量按其回归系数的比例分配分值。这些值的总和分层为低（0-6）、中（7-9）和高（≥10）风险。
选择了 5 个成分用于评分开发，包括二尖瓣前叶增厚、瓣叶尖端过度运动和反流射流长度≥2cm，以及主动脉瓣局灶性增厚和任何反流。该评分在推导和验证队列中显示出最佳的 RHD 诊断区分度和校准度（C 统计量分别为 0.998 和 0.994），对预测疾病进展也有较好的区分度（C 统计量为 0.811）。在低危儿童中，1 年、2 年和 3 年随访时无进展生存率分别为 100%、100%和 93%，而高危组分别为 90%、60%和 47%。基于分数的评分与疾病进展密切相关（危险比，1.270；95%CI，1.188-1.358；P<0.001）。
这个简化的评分，基于世界心脏联盟标准的组成部分，高度准确地识别明确的 RHD，并为风险分层提供了第一个工具，根据诊断时的超声心动图特征将潜伏性 RHD 患儿分为低危、中危或高危。
