目的:左心房(LA)肌病,以LA增大和机械功能障碍为特征,与肥厚型心肌病(HCM)的不良预后相关,而肌节突变对LA肌病的影响尚不清楚。我们旨在评估LA肌病与肌节突变之间的关联,并探索LA菌株在突变预测中的增量效用。
方法:共有105名连续的HCM患者(平均年龄47.8±11.9岁,71%的男性)进行了HCM相关基因筛查和心脏MRI的回顾性研究。洛杉矶卷,储层射血分数和应变指数,导管,和增压泵阶段分别进行了研究。
结果:50例突变阳性患者显示出较高的LA最大体积指数(59.4±28.2vs43.8±18.1mL/m2,p=0.001),下水库(21.3±7.9vs26.2±6.6%,p<0.001),和增压泵应变(12.1±5.4vs17.1±5.0%,p<0.001),但导管应变相似(9.2±4.5vs9.1±4.5%,p=0.909)与突变阴性患者相比。在多变量逻辑回归中,LA增压泵应变与肌节突变(比值比=0.86,95%置信区间:0.77-0.96,p=0.010)相关,与最大壁厚无关。晚期钆增强,洛杉矶卷。此外,LA增压泵菌株显示出增加的MayoII评分的突变预测值(AUC0.798vs0.709,p=0.024)。
结论:在HCM中,突变阳性患者的LA扩大更为严重,储液器和增压泵功能更差。LA增压泵菌株是MayoII评分中添加的肌节突变预测的重要因素。
结论:肌节突变与左心房机械功能障碍的独立关联为心房肌病的发病机制提供了新的见解,有助于了解突变阳性患者房颤和卒中的不良预后。
结论:•在肥厚型心肌病患者中,左心房(LA)储液器和增压泵功能,但不是管道功能,与突变阴性患者相比,突变阳性患者明显受损。•通过MRI衍生的特征跟踪测量的LA增压泵应变对于预测肌节突变具有高增量值增加到MayoII评分是可行的。
OBJECTIVE: Left atrial (LA) myopathy, characterized by LA enlargement and mechanical dysfunction, is associated with worse prognosis in hypertrophic cardiomyopathy (HCM) while the impact of sarcomere mutation on LA myopathy remains unclear. We aimed to assess the association between LA myopathy and sarcomere mutation and to explore the incremental utility of LA strain in mutation prediction.
METHODS: A total of 105 consecutive HCM patients (mean age 47.8 ± 11.9 years, 71% male) who underwent HCM-related gene screening and cardiac MRI were retrospectively enrolled. LA volume, ejection fraction and strain indices in reservoir, conduit, and booster-pump phases were investigated respectively.
RESULTS: Fifty mutation-positive patients showed higher LA maximal volume index (59.4 ± 28.2 vs 43.8 ± 18.1 mL/m2, p = 0.001), lower reservoir (21.3 ± 7.9 vs 26.2 ± 6.6%, p < 0.001), and booster-pump strain (12.1 ± 5.4 vs 17.1 ± 5.0%, p < 0.001) but similar conduit strain (9.2 ± 4.5 vs 9.1 ± 4.5%, p = 0.909) compared with mutation-negative patients. In multivariate logistic regression, LA booster-pump strain was associated with sarcomere mutation (odds ratio = 0.86, 95% confidence interval: 0.77-0.96, p = 0.010) independent of maximal wall thickness, late gadolinium enhancement, and LA volume. Furthermore, LA booster-pump strain showed incremental value for mutation prediction added to Mayo II score (AUC 0.798 vs 0.709, p = 0.024).
CONCLUSIONS: In HCM, mutation-positive patients suffered worse LA enlargement and worse reservoir and booster-pump functions. LA booster-pump strain was a strong factor for sarcomere mutation prediction added to Mayo II score.
CONCLUSIONS: The independent association between sarcomere mutation and left atrial mechanical dysfunction provide new insights into the pathogenesis of atrial myopathy and is helpful to understand the adverse prognosis regarding atrial fibrillation and stroke in mutation-positive patients.
CONCLUSIONS: • In patients with hypertrophic cardiomyopathy, left atrial (LA) reservoir and booster-pump function, but not conduit function, were significantly impaired in mutation-positive patients compared with mutation-negative patients. • LA booster-pump strain measured by MRI-derived feature tracking is feasible to predict sarcomere mutation with high incremental value added to Mayo II score.