Myocardial Work in Children With Hypertrophic Cardiomyopathy

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.