背景:我们研究的目的是评估新的美国心脏病学会(ACC)/美国心脏协会(AHA)指南的性能,关于心脏性猝死(SCD)的预防,与欧洲心脏病学会(肥厚型心肌病[HCM]Risk-SCD)的既定风险评分相比,在一个大型地中海HCM患者队列中。
方法:784例HCM患者的临床和影像学特征(首次评估时的平均年龄52±16岁,67.2%的男性)进行了回顾性分析。敏感性,特异性,根据ACC/AHA指南2020,SCD存在≥1个危险因素以及HCMRisk-SCD≥6%和HCMRisk-SCD≥4%的SCD事件的阴性预测值在随访期间进行评估.
结果:随访期间,47例(6%)患者发生SCD事件。根据新的ACC/AHA指南,SCD存在≥1个主要危险因素的敏感性为96%(95%CI85.5-99.5%),中度特异性为59%(95%CI55-62.2%),阴性预测值为99.5%(95%CI98.2-99.9%)。相反,HCM-风险SCD≥6%的敏感性相对较低(32%,95%CI19.1-47.1%)和95%的高特异性(95%CI93.1-96.4%),然而,HCM-Risk-SCD≥4%的敏感性为60%(95%CI44-74%),特异性为83.9%(95%CI80-85.6%)。与用于SCD预测的ACC/AHA算法相比,HCM风险-SCD截止值均显示出较低的阴性预测值,但准确性较高。
结论:新的ACC/AHA提出的算法可识别出大多数患有SCD事件的患者,其成本为大量除颤器植入。HCM-Risk-SCD表现出更高的特异性,而其敏感性和阴性预测值适中。
BACKGROUND: The aim of our study was to assess the performance of the new American College of Cardiology (ACC)/American Heart Association (AHA)
Guidelines, with respect to sudden cardiac death (SCD) prevention, in comparison with the established risk score of the European Society of Cardiology (hypertrophic cardiomyopathy [HCM] Risk-SCD), in a large Mediterranean cohort of HCM patients.
METHODS: The clinical and imaging characteristics of 784 HCM patients (mean age at first evaluation 52 ± 16 years, 67.2% males) were analyzed retrospectively. The sensitivity, specificity, and negative predictive value for SCD events of the presence of ≥1 risk factor for SCD according to the ACC/AHA
Guidelines 2020 and of the HCM Risk-SCD≥6% and HCM Risk-SCD≥4% were estimated during follow-up.
RESULTS: During follow-up, 47 (6%) patients suffered an SCD event. The presence of ≥1 major risk factor for SCD according to the new ACC/AHA
Guidelines had 96% sensitivity (95% CI 85.5-99.5%) with modest specificity of 59% (95% CI 55-62.2%) and negative predictive value of 99.5% (95% CI 98.2-99.9%). On the contrary, HCM- Risk-SCD≥6% had a relatively low sensitivity (32%, 95% CI 19.1-47.1%) and high specificity of 95% (95% CI 93.1-96.4%), whereas, HCM-Risk-SCD≥4% had sensitivity of 60% (95% CI 44-74%) and specificity of 83.9% (95% CI 80-85.6%). Both the HCM Risk-SCD cut-off values demonstrated lower negative predictive value but higher accuracy than the ACC/AHA algorithm for SCD prediction.
CONCLUSIONS: The novel ACC/AHA proposed algorithm identifies most of the patients with an SCD event with the cost of numerous defibrillator implantations. HCM-Risk-SCD demonstrated higher specificity, whereas its sensitivity and negative predictive value are modest.