Work

Myocardial Work in Children With Hypertrophic Cardiomyopathy

Year: 2025

Type: article

Abstract: BACKGROUND: Myocardial work (MW) predicts adverse outcomes in adults with hypertrophic cardiomyopathy (HCM), yet pediatric data are lacking. OBJECTIVES: The aim of the study was to describe longitudinal changes in MW and evaluate associations with adverse outcomes in a pediatric population. METHODS: 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. RESULTS: 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). CONCLUSIONS: 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.

Source: JACC Advances

DOI: 10.1016/j.jacadv.2025.101885