Department of Pediatric Cardiology, Baylor College of Medicine, Houston, Texas; Division of Cardiology, Department of Paediatrics, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada.
Division of Cardiology, Department of Paediatrics, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada.
J Am Soc Echocardiogr. 2024 May;37(5):508-517.e3. doi: 10.1016/j.echo.2023.11.025. Epub 2023 Dec 12.
Pediatric hypertrophic cardiomyopathy (HCM) is associated with adverse events. The contribution of diastolic dysfunction to adverse events is poorly understood. The aim of this study was to explore the association between diastolic phenotype and outcomes in pediatric patients with HCM.
Children <18 years of age with diagnosed with HCM were included. Diastolic function parameters were measured from the first echocardiogram at the time of diagnosis, including Doppler flow velocities, tissue Doppler velocities, and left atrial volume and function. Using principal-component analysis, key features in echocardiographic parameters were identified. The principal components were regressed to freedom from major adverse cardiac events (MACE), defined as implantable cardioverter-defibrillator insertion, myectomy, aborted sudden cardiac death, transplantation, need for mechanical circulatory support, and death.
Variables that estimate left ventricular filling pressures were highly collinear and associated with MACE (hazard ratio, 0.86; 95% CI, 0.75-1.00), though this was no longer significant after controlling for left ventricular thickness and genetic variation. Left atrial size parameters adjusted for body surface area were independently associated with outcomes in the covariate-adjusted model (hazard ratio, 0.69; 95% CI, 0.5-0.94). The covariate-adjusted model had an Akaike information criterion of 213, an adjusted R value of 0.78, and a concordance index of 0.82 for association with MACE.
Echocardiographic parameters of diastolic dysfunction were associated with MACE in this population study, in combination with the severity of left ventricular hypertrophy and genetic variation. Left atrial size parameters adjusted for body surface area were independently associated with adverse events. Additional study of diastolic function parameters adjusted for patient size could facilitate the prediction of adverse events in pediatric patients with HCM.
儿科肥厚型心肌病（HCM）与不良事件相关。舒张功能障碍对不良事件的影响知之甚少。本研究旨在探讨儿科 HCM 患者舒张表型与结局的关系。
纳入年龄<18 岁且诊断为 HCM 的患儿。在诊断时的首次超声心动图中测量舒张功能参数，包括多普勒血流速度、组织多普勒速度、左心房容积和功能。采用主成分分析识别超声心动图参数中的关键特征。将主成分回归至无重大不良心脏事件（MACE），定义为植入式心脏复律除颤器植入、心肌切除术、心源性猝死中止、移植、需要机械循环支持和死亡。
估计左心室充盈压的变量高度共线性，与 MACE 相关（风险比，0.86；95%CI，0.75-1.00），但在控制左心室厚度和遗传变异后，这不再具有统计学意义。经体表面积校正的左心房大小参数在协变量调整模型中与结局独立相关（风险比，0.69；95%CI，0.5-0.94）。协变量调整模型的 Akaike 信息准则为 213，调整 R 值为 0.78，与 MACE 相关的一致性指数为 0.82。
在这项人群研究中，舒张功能障碍的超声心动图参数与 MACE 相关，与左心室肥厚严重程度和遗传变异相结合。经体表面积校正的左心房大小参数与不良事件独立相关。进一步研究经患者体型校正的舒张功能参数可能有助于预测儿科 HCM 患者的不良事件。
