Crown Princess Victoria Children's Hospital, Department of Biomedical and Clinical Sciences, Department of Pediatrics, Linköping University, 581 85, Linköping, Sweden.
Department of Pediatrics, Institute of Clinical Sciences, Sahlgrenska Academy, University of Gothenburg, Gothenburg, Sweden.
Pediatr Cardiol. 2021 Mar;42(3):492-500. doi: 10.1007/s00246-020-02506-9. Epub 2021 Jan 30.
An ECG risk-score has been described that predicts high risk of subsequent cardiac arrest in young patients with hypertrophic cardiomyopathy (HCM). Myocardial fibrosis measured by cardiac magnetic resonance (CMR) late gadolinium enhancement (LGE) also affects prognosis. We assessed whether an ECG risk-score could be used as an indicator of myocardial fibrosis or perfusion deficit on CMR in HCM. In total 42 individuals (7-31 years); 26 HCM patients, seven genotype-positive, phenotype-negative individuals at risk of HCM (first-degree relatives) and nine healthy volunteers, underwent CMR to identify, and grade extent of, myocardial fibrosis and perfusion defect. 12-lead ECG was used for calculating the ECG risk-score (grading 0-14p). High-risk ECG (risk-score > 5p) occurred only in the HCM group (9/26), and the proportion was significantly higher vs mutation carriers combined with healthy volunteers (0/16, p = 0.008). Extent of LGE correlated to the ECG-score (R = 0.47, p = 0.001) in sarcomeric mutations. In low-risk ECG-score patients (0-2p), median percent of myocardium showing LGE (LGE%LVM) were: 0% [interquartile range, IQR, 0-0%], in intermediate-risk (3-5p): 5.4% [IQR 0-13.5%] and in high-risk (6-14p): 10.9% [IQR 4.2-12.3%]. ECG-score > 2p had a sensitivity and specificity of 79% and 84% to detect positive LGE on CMR and 77% vs. 75% to detect perfusion defects in sarcomeric mutations carriers. In patients with myocardial fibrosis as identified by LGE, median ECG risk-score was 8p [range 3-10p]. In conclusions, ECG risk-score > 2 p could be used as a cut-off for screening of myocardial fibrosis. Thus ECG risk-score is an inexpensive complementary tool in risk stratification of HCM in the young.
一种心电图风险评分可预测肥厚型心肌病（HCM）年轻患者发生心搏骤停的高危风险。心脏磁共振（CMR）检测的心肌纤维化（通过心脏磁共振延迟钆增强[LGE]测量）也会影响预后。我们评估心电图风险评分是否可用于指示 HCM 患者的 CMR 上的心肌纤维化或灌注不足。共有 42 人（7-31 岁）；26 名 HCM 患者，7 名有 HCM 风险的基因型阳性、表型阴性个体（一级亲属）和 9 名健康志愿者，接受 CMR 检查以确定并分级心肌纤维化和灌注缺陷的程度。12 导联心电图用于计算心电图风险评分（0-14 分）。高危心电图（风险评分>5 分）仅发生在 HCM 组（26 例中的 9 例），与突变携带者和健康志愿者的比例（0/16，p=0.008）相比显著更高。LGE 的程度与肌节突变的心电图评分相关（R=0.47，p=0.001）。在低风险心电图评分患者（0-2 分）中，LGE 占左心室心肌质量百分比中位数（LGE%LVM）为：0%[四分位间距，IQR，0-0%]，中风险（3-5 分）：5.4%[IQR 0-13.5%]和高风险（6-14 分）：10.9%[IQR 4.2-12.3%]。心电图评分>2 分对检测 CMR 上阳性 LGE 的灵敏度和特异性分别为 79%和 84%，对肌节突变携带者检测灌注缺陷的灵敏度和特异性分别为 77%和 75%。在通过 LGE 确定的心肌纤维化患者中，中位数心电图风险评分 8 分[范围 3-10 分]。总之，心电图评分>2 分可作为筛选心肌纤维化的切点。因此，心电图风险评分是年轻患者 HCM 危险分层的一种廉价的辅助工具。
