Spatial and Temporal Non-Uniform Changes in Left Ventricular Myocardial Strain in Dogs with Duchenne Muscular
Bijan Ghaleh1,2, Inès Barthélemy3, Lucien Sambin1
1Inserm U955-IMRB, Team 3, UPEC, Ecole Nationale Vétérinaire d'Alfort, 94700 Maisons-Alfort, France.
Journal of Cardiovascular Development and Disease
|May 26, 2023
Summary
Dystrophin-deficient cardiomyopathy in GRMD dogs shows early, non-uniform changes in left ventricular myocardial strain, even with normal global function. These alterations in circumferential and longitudinal strain offer insights into disease progression.
Area of Science:
- Cardiology
- Biomedical Engineering
- Animal Models
Background:
- Duchenne muscular dystrophy (DMD) patients have prolonged lifespans, increasing the importance of understanding dystrophin-deficient cardiomyopathy.
- Golden Retriever Muscular Dystrophy (GRMD) dogs serve as a relevant model for studying DMD-related cardiac conditions.
Purpose of the Study:
- To investigate the non-uniformity of myocardial strain within the left ventricle during the progression of cardiomyopathy in GRMD dogs.
- To analyze spatial and temporal alterations in myocardial strain using two-dimensional speckle tracking echocardiography.
Main Methods:
- Two-dimensional speckle tracking echocardiography was used to analyze circumferential strain (CS) and longitudinal strain (LS).
- Strain was assessed in the endocardial, middle, and epicardial layers of the left ventricle (LV) apex, middle-chamber, and base.
- Data were collected from GRMD (n=22) and healthy control dogs (n=7) aged 2 to 24 months.
Main Results:
- GRMD dogs showed reduced systolic CS in all three LV apex layers by 2 months, despite normal global systolic function.
- Spatial heterogeneity in CS progressed with age.
- A decrease in systolic LS was detected in all three LV wall layers by 2 months of age.
Conclusions:
- The study reveals spatial and temporal non-uniform alterations in LV myocardial strain in GRMD dogs.
- These findings provide new insights into the progression of dystrophin-deficient cardiomyopathy in this animal model.
- Early detection of strain abnormalities may aid in understanding and managing cardiac complications in DMD.
Keywords:
canine model of duchenne muscular dystrophydystrophin-deficient cardiomyopathyheterogeneitymyocardial strainspeckle-tracking echocardiography

