Myocardial Work in Children With Hypertrophic Cardiomyopathy
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:...
Saved in:
| Main Authors: | , , , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Elsevier
2025-07-01
|
| Series: | JACC: Advances |
| Subjects: | |
| Online Access: | http://www.sciencedirect.com/science/article/pii/S2772963X25003059 |
| Tags: |
Add Tag
No Tags, Be the first to tag this record!
|
| _version_ | 1849744195269427200 |
|---|---|
| author | Xander Jacquemyn, BSc Rebbeca Dryer, MD Kyla Cordrey, MD Rita Long, RDCS David A. Danford, MD, MPH Shelby Kutty, MD, PhD, MHCM Benjamin T. Barnes, MD |
| author_facet | Xander Jacquemyn, BSc Rebbeca Dryer, MD Kyla Cordrey, MD Rita Long, RDCS David A. Danford, MD, MPH Shelby Kutty, MD, PhD, MHCM Benjamin T. Barnes, MD |
| author_sort | Xander Jacquemyn, BSc |
| collection | DOAJ |
| description | 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. |
| format | Article |
| id | doaj-art-29f70e0edf674e9db637ac418ead617e |
| institution | DOAJ |
| issn | 2772-963X |
| language | English |
| publishDate | 2025-07-01 |
| publisher | Elsevier |
| record_format | Article |
| series | JACC: Advances |
| spelling | doaj-art-29f70e0edf674e9db637ac418ead617e2025-08-20T03:21:46ZengElsevierJACC: Advances2772-963X2025-07-014710188510.1016/j.jacadv.2025.101885Myocardial Work in Children With Hypertrophic CardiomyopathyXander Jacquemyn, BSc0Rebbeca Dryer, MD1Kyla Cordrey, MD2Rita Long, RDCS3David A. Danford, MD, MPH4Shelby Kutty, MD, PhD, MHCM5Benjamin T. Barnes, MD6The 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; Address for correspondence: Xander Jacquemyn, Johns Hopkins Hospital, 1800 Orleans Street, Baltimore Maryland 21287, USA.The Blalock-Taussig-Thomas Pediatric and Congenital Heart Center, Department of Pediatrics, Johns Hopkins School of Medicine, Johns Hopkins University, Baltimore, Maryland, USAThe Blalock-Taussig-Thomas Pediatric and Congenital Heart Center, Department of Pediatrics, Johns Hopkins School of Medicine, Johns Hopkins University, Baltimore, Maryland, USAThe Blalock-Taussig-Thomas Pediatric and Congenital Heart Center, Department of Pediatrics, Johns Hopkins School of Medicine, Johns Hopkins University, Baltimore, Maryland, USAThe Blalock-Taussig-Thomas Pediatric and Congenital Heart Center, Department of Pediatrics, Johns Hopkins School of Medicine, Johns Hopkins University, Baltimore, Maryland, USAThe Blalock-Taussig-Thomas Pediatric and Congenital Heart Center, Department of Pediatrics, Johns Hopkins School of Medicine, Johns Hopkins University, Baltimore, Maryland, USAThe Blalock-Taussig-Thomas Pediatric and Congenital Heart Center, Department of Pediatrics, Johns Hopkins School of Medicine, Johns Hopkins University, Baltimore, Maryland, USABackground: 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.http://www.sciencedirect.com/science/article/pii/S2772963X25003059clinical outcomesechocardiographyglobal longitudinal strainhypertrophic cardiomyopathypediatric cardiologymyocardial work |
| spellingShingle | Xander Jacquemyn, BSc Rebbeca Dryer, MD Kyla Cordrey, MD Rita Long, RDCS David A. Danford, MD, MPH Shelby Kutty, MD, PhD, MHCM Benjamin T. Barnes, MD Myocardial Work in Children With Hypertrophic Cardiomyopathy JACC: Advances clinical outcomes echocardiography global longitudinal strain hypertrophic cardiomyopathy pediatric cardiology myocardial work |
| title | Myocardial Work in Children With Hypertrophic Cardiomyopathy |
| title_full | Myocardial Work in Children With Hypertrophic Cardiomyopathy |
| title_fullStr | Myocardial Work in Children With Hypertrophic Cardiomyopathy |
| title_full_unstemmed | Myocardial Work in Children With Hypertrophic Cardiomyopathy |
| title_short | Myocardial Work in Children With Hypertrophic Cardiomyopathy |
| title_sort | myocardial work in children with hypertrophic cardiomyopathy |
| topic | clinical outcomes echocardiography global longitudinal strain hypertrophic cardiomyopathy pediatric cardiology myocardial work |
| url | http://www.sciencedirect.com/science/article/pii/S2772963X25003059 |
| work_keys_str_mv | AT xanderjacquemynbsc myocardialworkinchildrenwithhypertrophiccardiomyopathy AT rebbecadryermd myocardialworkinchildrenwithhypertrophiccardiomyopathy AT kylacordreymd myocardialworkinchildrenwithhypertrophiccardiomyopathy AT ritalongrdcs myocardialworkinchildrenwithhypertrophiccardiomyopathy AT davidadanfordmdmph myocardialworkinchildrenwithhypertrophiccardiomyopathy AT shelbykuttymdphdmhcm myocardialworkinchildrenwithhypertrophiccardiomyopathy AT benjamintbarnesmd myocardialworkinchildrenwithhypertrophiccardiomyopathy |