Kerstens, TP, van Everdingen, WM, Habets, J, van Dijk, APJ, Helbing, WA, Thijssen, DHJ and Udink ten Cate, FEA (2023) Left ventricular deformation and myocardial fibrosis in pediatric patients with Duchenne muscular dystrophy. International Journal of Cardiology, 388. ISSN 0167-5273

Preview

Text Left ventricular deformation and myocardial fibrosis in pediatric patients with Duchenne muscular dystrophy.pdf - Published Version Available under License Creative Commons Attribution Non-commercial No Derivatives. Download (2MB) | Preview

Abstract

Background: Left ventricular (LV) strain and rotation are emerging functional markers for early detection of LV dysfunction and have been associated with the burden of myocardial fibrosis in several disease states. This study examined the association between LV deformation (i.e., LV strain and rotation) and extent and location of LV myocardial fibrosis in pediatric patients with Duchenne muscular dystrophy (DMD). Methods and results: 34 pediatric patients with DMD underwent cardiovascular magnetic resonance (CMR) with late gadolinium enhancement (LGE) to assess LV myocardial fibrosis. Offline CMR feature-tracking analysis was used to assess global and segmental longitudinal and circumferential LV strain, and LV rotation. Patients with fibrosis (n = 18, 52.9%) were older than those without fibrosis (14 ± 3 years (yrs) vs 11 ± 2 yrs., p = 0.01). There was no significant difference in LV ejection fraction (LVEF) between subjects with and without fibrosis (54 ± 6% vs 56 ± 4%, p = 0.18). However, lower endocardial global circumferential strain (GCS), but not LV rotation, was associated with presence of fibrosis (adjusted Odds Ratio 1.25 [95% CI 1.01–1.56], p = 0.04). Both GCS and global longitudinal strain correlated with the extent of fibrosis (r =.52, p = 0.03 and r =.75, p < 0.01, respectively). Importantly, segmental strain did not seem to correspond to location of fibrosis. Conclusion: A lower global, but not segmental, strain is associated with presence and extent of LV myocardial fibrosis in pediatric DMD patients. Therefore, strain parameters might detect structural myocardial alterations, however currently more research is needed to evaluate its value (e.g., prognostic) in clinical practice.

Item Type:	Article
Uncontrolled Keywords:	Myocardium; Humans; Muscular Dystrophy, Duchenne; Cardiomyopathies; Fibrosis; Gadolinium; Contrast Media; Magnetic Resonance Imaging, Cine; Stroke Volume; Ventricular Function, Left; Child; Cardiomyopathy; Duchenne muscular dystrophy; Fibrosis; LV deformation; Rotation; Strain; Humans; Child; Contrast Media; Muscular Dystrophy, Duchenne; Magnetic Resonance Imaging, Cine; Gadolinium; Cardiomyopathies; Myocardium; Ventricular Function, Left; Stroke Volume; Fibrosis; 1102 Cardiorespiratory Medicine and Haematology; 1117 Public Health and Health Services; Cardiovascular System & Hematology
Subjects:	R Medicine > RA Public aspects of medicine > RA0421 Public health. Hygiene. Preventive Medicine R Medicine > RC Internal medicine > RC1200 Sports Medicine
Divisions:	Sport & Exercise Sciences
Publisher:	Elsevier
SWORD Depositor:	A Symplectic
Date Deposited:	07 Nov 2023 10:35
Last Modified:	07 Nov 2023 10:45
DOI or ID number:	10.1016/j.ijcard.2023.131162
URI:	https://researchonline.ljmu.ac.uk/id/eprint/21804

View Item