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Serial left ventricular wall measurements in Duchenne's muscular dystrophy
Journal of the American College of Cardiology
|July 1, 1983
Summary
Duchenne muscular dystrophy patients show normal initial left ventricular wall growth but later experience thinning, unlike healthy individuals. This thinning, linked to fibrosis, deviates from normal growth patterns, especially in older patients.
Area of Science:
- Cardiology
- Neurology
- Genetics
Background:
- Duchenne muscular dystrophy (DMD) is a progressive genetic disorder affecting muscle tissue.
- Cardiac involvement is a significant cause of morbidity and mortality in DMD patients.
- Understanding cardiac structural changes in DMD is crucial for patient management.
Purpose of the Study:
- To serially assess left ventricular (LV) wall thickness in DMD patients.
- To compare LV wall growth in DMD patients with normal subjects and a control group.
- To investigate the relationship between LV wall thinning and disease progression in DMD.
Main Methods:
- Serial echocardiography was used to measure LV wall thickness (end-systole and end-diastole) in 19 DMD patients over 12 months.
- LV wall thickness was compared to body surface area in DMD patients, normal subjects, and a control group with other neurologic disorders.
Main Results:
- Normal subjects exhibited linear LV wall thickness increase with body surface area.
- DMD patients initially showed linear wall growth, followed by significant thinning.
- Thinning onset was not age-dependent but more frequent in older patients; those who died showed marked deviation from normal growth.
Conclusions:
- Left ventricular wall thinning occurs in Duchenne muscular dystrophy, deviating from normal growth patterns.
- This thinning is likely due to myocardial fibrosis and myofibril loss.
- Cardiac structural changes in DMD warrant continued monitoring and investigation.