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Published on: April 10, 2019
Cardiac asynchrony in Duchenne muscular dystrophy
Abdallah Fayssoil1, Olivier Nardi, David Orlikowski
1Réanimation médicale, hôpital Raymond Poincaré (AP-HP), Université de Versailles SQY, 104 boulevard Raymond Poincaré, 92380, Garches, France, fayssoil2000@yahoo.fr.
Insights
Duchenne muscular dystrophy (DMD) patients often have left ventricular dysfunction. Systolic ventricular asynchrony is frequent, especially in those with reduced ejection fraction (EF).
Area of Science:
- Cardiology
- Genetics
- Neuromuscular Disorders
Background:
- Duchenne muscular dystrophy (DMD) is an inherited disorder affecting muscle, frequently leading to heart failure.
- The prevalence and characteristics of systolic dyssynchrony in DMD patients are not well-documented.
- Left ventricular (LV) dysfunction is a known complication, but its association with asynchrony requires further investigation.
Purpose of the Study:
- To evaluate the prevalence of left ventricular dysfunction in adult DMD patients.
- To assess the incidence of systolic asynchrony, both inter-ventricular and intra-ventricular, in this population.
- To explore the relationship between LV ejection fraction (EF) and the presence of systolic asynchrony.
Main Methods:
- Electrocardiography and echocardiography were performed on 48 adult DMD patients.
- Systolic asynchrony was assessed using echocardiography-Doppler and tissue Doppler imaging.
- Patients' ages ranged from 20 to 37 years.
Main Results:
- Left ventricular ejection fraction (EF) varied widely, with a median of 43%.
- Inter-ventricular asynchrony was observed in 11.9% of patients with EF < 35% and 2.6% with EF > 35%.
- Intra-ventricular asynchrony was present in 6% of patients with EF < 35%.
Conclusions:
- DMD patients exhibit a high prevalence of left ventricular dysfunction.
- Systolic ventricular asynchrony appears to be a common finding in DMD, particularly in those with significantly reduced LV function (EF < 35%).
- These findings highlight the importance of cardiac monitoring in DMD management.
Abstract:
Duchenne muscular dystrophy (DMD) is an inherited myogenic disorder due to mutations in the dystrophin gene on chromosome Xp21.1. Heart failure is a classical complication in this disease. Little data are available about systolic dyssynchrony in DMD. We sought to assess the prevalence of left ventricular dysfunction and systolic asynchrony in DMD patients using echocardiographic parameters. We performed electrocardiography and echocardiography for adult's patients with DMD. For systolic dyssynchrony assessment, echocardiography-Doppler was performed and completed by tissular Doppler imaging. 48 DMD were included in our study. Age ranged from 20 to 37 years. QRS duration >120 ms was present in 10 patients/48 and 1 patient disclosed a QRS duration >150 ms. Left ventricular (LV) ejection fraction (EF) ranged from 10 to 62 % with a median of 43 %. Inter-ventricular asynchrony was found in 11.9 % of patients with EF < 35 % and in 2.6 % of patients with EF > 35 %. Intra-ventricular asynchrony was present in 6 % of patients with EF < 35 %. We found a high prevalence of LV dysfunction in DMD. Systolic ventricular asynchrony seems frequent particularly in patients with EF < 35 %.
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