【摘要】 目的：探讨心脏磁共振成像（CMR）左心室应变参数对左心室射血分数（LVEF）正常且钆延迟增强百分比（LGE%）较低的“表型良性”肥厚型心肌病（HCM）患者预后的预测价值。方法：回顾性连续纳入2019～2024年云南省第一人民医院100例LVEF≥55%、LGE%<10%的HCM患者，同期纳入35名健康受试者建立CMR特征追踪应变参考值。以由心脏性猝死（SCD）、可中止的SCD、预防性植入埋藏式心脏复律除颤器组成的复合事件为随访终点，对比分析发生和未发生终点事件的HCM患者的基线特征。采用多因素Cox比例风险回归模型及LASSO回归分析评估包括左心室应变参数在内的CMR参数对终点事件的预测价值，并采用受试者工作特征（ROC）曲线确定最佳截断值及其预测效能。比较健康受试者、心肌质量指数（MMI）正常和增高的HCM患者的左心室应变参数，并采用Pearson相关性分析评估MMI与左心室应变参数的相关性。结果：中位随访24.0(18.0,26.0)个月期间，23例（23.0%）患者发生终点事件。与未发生终点事件的患者相比，发生终点事件的患者年龄更大，MMI更高，且左心室整体纵向应变（GLS）、整体环向应变（GCS）、整体径向应变（GRS）均显著受损（绝对值更小）（P均<0.05）。多因素Cox回归分析显示，校正年龄、HCM家族史、MMI、室间隔最大厚度及LGE%等混杂因素后，左心室GCS是终点事件的唯一独立预测因子（HR=1.185,95%CI:1.071～1.283,P=0.002）。ROC曲线分析显示，当左心室GCS绝对值<16.9%时，预测终点事件的灵敏度为79.2%，特异度为80.3%。与健康受试者相比，MMI正常的HCM患者左心室GLS和GRS已显著受损，MMI增高的HCM患者左心室应变受损进一步加剧，特别是左心室GCS绝对值显著下降。Pearson相关性分析显示，MMI与左心室GLS、左心室GCS的绝对值均呈显著线性负相关（r分别为-0.43和-0.35,P均<0.001）。结论：对于LVEF正常且LGE%较低的HCM患者，CMR衍生的左心室GCS是其预后的独立预测指标，且左心室GCS绝对值与MMI呈线性负相关，因此可考虑用来识别该类患者中的高危个体。更多还原

【Abstract】 Objectives: To evaluate the prognostic value of left ventricular strain parameters derived from cardiac magnetic resonance imaging(CMR) for patients with "phenotypically benign" hypertrophic cardiomyopathy(HCM) who have preserved left ventricular ejection fraction(LVEF) and low late gadolinium enhancement percentage(LGE%).Methods: We retrospectively enrolled 100 consecutive HCM patients(LVEF ≥ 55%, LGE% < 10%) and 35 healthy volunteers scanned during 2019-2024 to establish CMR-feature-tracking reference values. The adjudicated composite endpoint comprised sudden cardiac death(SCD), aborted SCD, or prophylactic implantable cardioverter-defibrillator implantation. A multivariate Cox proportional hazards regression model and LASSO regression analysis were employed to evaluate the predictive value of CMR parameters, including left ventricular strain parameters, for the endpoint events. The optimal cutoff value and its predictive power were determined using the receiver operating characteristic(ROC) curve. This study also compared left ventriculular strain parameters in healthy subjects, HCM patients with normal or elevated myocardial mass index(MMI), and assessed the correlation between MMI and left ventricular strain parameters using Pearson correlation analysis.Results: During follow-up of 24.0(18.0, 26.0) months, 23 patients(23.0%) experienced the endpoint events. Compared with patients who did not experience the endpoint events, patients who did were older, had higher MMI, and significantly impaired left ventricular global longitudinal strain(GLS), global circumferential strain(GCS), and global radial strain(GRS)(with smaller absolute values)(all P<0.05). Multivariate Cox regression analysis revealed that after adjusting for confounding factors such as age, family history of HCM, MMI, maximum ventricular septal thickness, and LGE%, left ventricular GCS was the only independent predictor of the endpoint events(HR=1.185, 95%CI: 1.071-1.283, P=0.002). ROC curve analysis showed that when the absolute value of left ventricular GCS was <16.9%, the sensitivity for predicting the endpoint events was 79.2%, and the specificity was 80.3%. Compared with healthy subjects, HCM patients with normal MMI exhibited significantly impaired left ventricular GLS and GRS, while those with elevated MMI had further aggravated left ventricular strain impairment, particularly a significant decrease in the absolute value of left ventricular GCS. Pearson correlation analysis demonstrated a significant linear negative correlation between MMI and the absolute values of left ventricular GLS(r=-0.43) and left ventricular GCS(r=-0.35)(both P<0.001).Conclusions: For HCM patients with preserved LVEF and low LGE%, the left ventricular GCS derived from CMR might serve as an independent prognostic predictor, and the absolute value of left ventricular GCS shows a linear negative correlation with the MMI. Therefore, it can be considered for identifying high-risk individuals within this patient population.更多还原