Jacquemyn Xander, Dryer Rebbeca, Cordrey Kyla, Long Rita, Danford David A, Kutty Shelby, Barnes Benjamin T
The Blalock-Taussig-Thomas Pediatric and Congenital Heart Center, Department of Pediatrics, Johns Hopkins School of Medicine, Johns Hopkins University, Baltimore, Maryland, USA; Department of Cardiovascular Sciences, KU Leuven, Leuven, Belgium.
The Blalock-Taussig-Thomas Pediatric and Congenital Heart Center, Department of Pediatrics, Johns Hopkins School of Medicine, Johns Hopkins University, Baltimore, Maryland, USA.
JACC Adv. 2025 Jul;4(7):101885. doi: 10.1016/j.jacadv.2025.101885. Epub 2025 Jun 17.
Myocardial work (MW) predicts adverse outcomes in adults with hypertrophic cardiomyopathy (HCM), yet pediatric data are lacking.
The aim of the study was to describe longitudinal changes in MW and evaluate associations with adverse outcomes in a pediatric population.
A total of 74 patients with HCM (11.9 years [7.7-14.5], 50% males) were included. MW indices-global work index (GWI), global constructive work (GCW), global wasted work, and global work efficiency (GWE)-were measured and compared with a family history group (FH) (n = 72) (defined as having a first-degree relative with HCM, a second-degree relative with sudden cardiac death (SCD), or a pathogenic mutation without positive phenotype) and healthy controls (n = 50). The primary outcome was a composite endpoint encompassing all-cause mortality, SCD, aborted SCD, appropriate implantable cardioverter-defibrillator discharge, and sustained ventricular tachycardia.
MW indices differed significantly between groups at baseline. In HCM patients, GWI, GCW, and GWE were lower than in FH (pairwise P = 0.012, P < 0.001, and P = 0.001, respectively), while only GCW and GWE were significantly lower in HCM compared to healthy control (both pairwise P < 0.001). During follow-up (4.9 years [2.9-8.8]), patients with HCM showed significant decreases in GWI and GCW (P = 0.002 and P = 0.001), while global wasted work and GWE did not show significant changes (P = 0.665 and P = 0.126). In contrast, FH patients exhibited stable MW indices over time. Lower GWI and GCW were positively associated with the composite endpoint (both P < 0.001).
In pediatric HCM, MW declines over time and is linked to adverse outcomes but remains primarily a research tool, with no superior risk stratification compared to global longitudinal strain.
心肌做功（MW）可预测肥厚型心肌病（HCM）成年患者的不良结局，但儿科数据尚缺。
本研究旨在描述儿科人群中MW的纵向变化，并评估其与不良结局的关联。
共纳入74例HCM患者（年龄11.9岁[7.7 - 14.5岁]，50%为男性）。测量MW指标——整体做功指数（GWI）、整体建设性做功（GCW）、整体无用功和整体做功效率（GWE），并与家族史组（FH）（n = 72）（定义为有HCM一级亲属、心脏性猝死（SCD）二级亲属或有致病突变但无阳性表型）及健康对照组（n = 50）进行比较。主要结局是一个综合终点，包括全因死亡率、SCD、SCD未遂、合适的植入式心律转复除颤器放电及持续性室性心动过速。
基线时各组间MW指标差异显著。在HCM患者中，GWI、GCW和GWE低于FH组（两两比较P值分别为0.012、<0.001和0.001），而与健康对照组相比，仅HCM组的GCW和GWE显著降低（两者两两比较P值均<0.001）。随访期间（4.9年[2.9 - 8.8年]），HCM患者的GWI和GCW显著下降（P值分别为0.002和0.001），而整体无用功和GWE无显著变化（P值分别为0.665和0.126）。相比之下，FH组患者的MW指标随时间保持稳定。较低的GWI和GCW与综合终点呈正相关（两者P值均<0.001）。
在儿科HCM中，MW随时间下降且与不良结局相关，但目前主要仍是一种研究工具，与整体纵向应变相比，并无更好的风险分层作用。
